Haemangioendothelioma of bone is a rare intermediate grade malignancy. Because of its rareness there is a lack of information in the literature about the well established treatment strategies depending on series with large numbers. The outcome of wide resection with postoperative external irradiation would be presented. 4 patients (2 females, 2 males) with a mean age of 40.5 (26–52) with solitary haemangioendothelioma of bone admitted with local pain on the affected bone and limited restriction of function. Anatomical sites were scapula, calcaneum, midshaft of radius and metaphysodiaphyseal region of femur. Plain X-ray, CT, MRI, Tc 99 tecnetium wholebody bone scan investigations were applied. All lesions were hot on bone scan and lytic irregular permeative lesions T1 hypo, T2 hyper with gadolinium enhancement were present. Open biopsy resulted with the diagnosis of intermediate haemangioendothelioma of bone. Wide resection of tubular bones and intercalary lyophilised allograft recostruction with IM rod and cerclage wire and total calcaneum resection and allograft replacement with talar arthrodesis, total scapulectomy subsequent autoclaved bone reimplantation were the surgical procedures applied. Mean follow-up was 96 months (40–132). Three patients except scapula case received 50 Gy external irradiation. No patient developed local recurrence in the follow up. Regarding complications calcaneum patient developed skin necrosis after the irradiation which led to removal of the allograft but eventually healed. Scapula patient had late infection treated by antibiotics. All patients had satisfactory function. Intercalary allografts united in 6 months time. Calcaneum patient developed multiple small lung metastasis 1 year after the operation and treated by adriamycin based chemotherapy and interpherone. The lung lesions showed slight regression but the patient is alive since 112 months with no further relapse. Wide excision with subsequent irradiation and wide excision of total scapula resulted with no local recurrence in our small group of patients with this rare malignancy. Irradiation provided relatively less soft tissue sacrification and a sufficient local tumour control without risking the patient to an impending amputation in the occurence of local recurrence

We have reviewed 20 cases of parosteal osteosarcoma treated by wide local resection and prosthetic replacement and followed up for six to 17 years. Limb function was excellent in 85%. One patient with grade III histological disease developed pulmonary metastases. Four patients had local recurrences, which were related to repeated preliminary biopsies, inappropriate siting of biopsy and vascular encroachment by the tumour. After this mode of treatment, the outcome was not related to medullary invasion by the tumour

In countries where Confucianism is popular, it is extremely difþcult to get allograft. Twenty seven cases of limb salvage with recycled autogenous bone were performed after wide resection of malignant tumors. Recycling was done in 9 cases with pasteurization and in 18 cases with irradiation. Pasteurization was done in 60¡-30minutes with thermostatic saline bath. Irradiation was performed in sterile plastic jar þlled with saline. 25 Gy radiation was given þrst anteroposteriorly and then another 25Gy posteroanteriorly. Internal þxation was done either with plate and/or intramedullay nail, and in 13 cases, intramedullary packing of bone cement was added. Among 9 pasteurized cases, 5 were intercalary diaphyseal resection, 2 whole bone resection of metatarsals, and 2 prosthesis-pasteurized bone composite arthroplasty. Among 18 irradiated cases, 12 were osteoarticular, and 6 intercalary resections. Follow-up period was 16 to 112 months (mean: 46 months). There was no recycled bone-related local recurrence. Time for union varied greatly.(4 to 14 months). There was no statistical difference in union time between pasteurized and irradiated bone (Wilcoxon rank test). Complications were 9 delayed or non-union, 3 fractures of recycled bone, and 2 cases of separation-resorption of growth plate. Irradiation seems much better than pasteurization because it could preserve mechanical property of articular cartilage and soft tissues such as tendon and capsule. We recommend intraoperative-extracorporeal irradiation as a good substitute for osteoarticular allograft because soft tissue attachment could be saved. Intramedullary packing of bone cement was proved as a good method to enhance the stability of þxation and to prevent fracture

Aims. The standard of wide tumour-like resection for chronic osteomyelitis (COM) has been challenged recently by adequate debridement. This paper reviews the evolution of surgical debridement for long bone COM, and presents the outcome of adequate debridement in a tertiary bone infection unit. Methods. We analyzed the retrospective record review from 2014 to 2020 of patients with long bone COM. All were managed by multidisciplinary infection team (MDT) protocol. Adequate debridement was employed for all cases, and no case of wide resection was included. Results. A total of 53 patients (54 bones) with median age of 45.5 years (interquartile range 31 to 55) and mean follow-up of 29 months (12 to 59) were included. In all, ten bones were Cierny-Mader type I, 39 were type III, and five were type IV. All patients were treated with single-staged management, except for one (planned two-stage stabilization). Positive microbial cultures grew in 75%. Overall, 46 cases (85%) had resolution of COM after index procedure, and 49 (90.7%) had resolution on last follow-up. Four patients (7%) underwent second surgical procedure and six patients (11%) had complications. Conclusion. We challenge the need for wide tumour-like resection in all cases of COM. Through detailed preoperative evaluation and planning with MDT approach, adequate debridement and local delivery of high concentration of antibiotic appears to provide comparable outcomes versus radical debridement. Cite this article: Bone Jt Open 2023;4(8):643–651

In 45 patients we assessed the functional results and complications for three different reconstructive procedures after resection of primary tumors of the proximal humerus. An osteoarticular allograft was used in 11, a clavicula pro humero operation in 15 and a tumour prosthesis in 19. The glenoid was resected with the proximal humerus in 25 patients. The axillary nerve was resected in 42 patients.

The complication rate was lowest after reconstruction with a tumour prosthesis. The clavicula pro humero operation resulted in the most revisions. Cumulative survival rates for all the reconstructive procedures were similar.

At follow-up at two years the functional results for the three reconstructive procedures were the same with a mean functional rating of 79% (Musculoskeletal Tumor Society). Excision of the glenoid had no influence on the functional result.

Our findings indicate that the use of a tumour prosthesis is the most reliable limb-salvage procedure for the proximal humerus. The clavicula pro humero is an appropriate procedure if a prosthesis cannot be used.

Resection of malignant bony tumours of the pelvis creates large bone and soft-tissue defects, and is frequently associated with complications such as wound dehiscence and deep infection. We present the results of six patients in whom a rectus abdominis myocutaneous (RAM) flap was used following resection of a malignant tumour of the pelvis. Bony reconstruction was performed using a constrained hip tumour prosthesis in three patients, vascularised fibular graft in two and frozen autograft in one. At a mean follow-up of 63 months (16 to 115), no patients had a problem with the wound.

Immediate reconstruction using a RAM flap may be used after resection of a malignant tumour of the pelvis to provide an adequate volume of tissue to eliminate the dead space, cover the exposed bone or implants with well-vascularised soft tissue and to reduce the risk of complications.

Cite this article: Bone Joint J 2014;96-B:270–3.

We investigated whether the presence of a pathological fracture increased the risk of local recurrence in patients with a giant cell tumour (GCT) of bone. We also assessed if curettage is still an appropriate form of treatment in the presence of a pathological fracture. We conducted a comprehensive review and meta-analysis of papers which reported outcomes in patients with a GCT with and without a pathological fracture at presentation. We computed the odds ratio (OR) of local recurrence in those with and without a pathological fracture.

We selected 19 eligible papers for final analysis. This included 3215 patients, of whom 580 (18.0%) had a pathological fracture. The pooled OR for local recurrence between patients with and without a pathological fracture was 1.05 (95% confidence interval (CI) 0.66 to 1.67, p = 0.854). Amongst the subgroup of patients who were treated with curettage, the pooled OR for local recurrence was 1.23 (95% CI 0.75 to 2.01, p = 0.417).

A post hoc sample size calculation showed adequate power for both comparisons.

There is no difference in local recurrence rates between patients who have a GCT of bone with and without a pathological fracture at the time of presentation. The presence of a pathological fracture should not preclude the decision to perform curettage as carefully selected patients who undergo curettage can have similar outcomes in terms of local recurrence to those without such a fracture.

Cite this article: Bone Joint J 2015;97-B:1566–71.

We assessed three children who had limb salvage surgery for sarcomata of the proximal fibula. Their ages ranged from 6 to 13 years. MRI showed involvement of the anterior, lateral and deep posterior compartments. Incisional biopsy showed two osteoblastic osteosarcomata and one Ewing’s sarcoma. Preoperative angiogram confirmed that the tibialis posterior artery was the main blood supply to the foot.

The surgical technique was as described by Malawer (1984): via an anterolateral incision, the anterior, lateral and deep posterior compartments were excised en bloc with the proximal fibula. The peroneal artery was sacrificed in all three patients, and the common peroneal nerve and tibialis anterior artery in two. In the patient with Ewing’s sarcoma, the tibialis anterior muscle, artery and nerve did not require excision. What remained were the tibialis posterior nerve and artery, gastrocnemius, flexor digitorum longus and most of soleus. The lateral gastrocnemius was rotated forward to fill the defect. The collateral ligament of the knee was reconstructed by suturing the lateral collateral ligament and biceps femoris to the proximal tibia.

All three patients had neo-adjuvant chemotherapy. Tumour necrosis ranged from 90% to 100%.

At follow-up at 2, 4 and 9 years, there was no recurrence. The Musculoskeletal Tumour Society evaluation was used and all patients scored more than 80% and were happy with the cosmetic result. Although two of the three patients required an orthotic because of weak dorsiflexion, the functional result was good.

A “hands-on” composite gives a similar functional result as a custom-made prosthesis and has a much better function than alternative techniques. Less expensive and more flexible than custom-made prostheses, it can be used even when no part of the iliac wing remains. The use of cement permits the adjunction of antibiotics needed for these complicated cases.

After peri-acetabular resection for bone sarcoma, a reconstructive procedure is necessary to stabilize the hip, avoid limb discrepancy, and permit full weight bearing. This procedure needs to be easy to perform because resection of the area is time and blood consuming. This leads to the use of a “hands-on” composite prosthesis.

Our reconstructive procedure uses a titanium cup with a long screw that is fixed in the remaining bone (sacrum or spine). When the cup is firmly fixed to the bone, the gap between the cup and bone is filled with cement loaded with antibiotics, and the polyethylene component is cemented on the innominate prosthesis. The femoral component of a usual hip total prosthesis is then implanted.

Since 1990 we have used this reconstructive procedure in 50 patients, 27 with bone sarcomas involving the acetabulum (11 chondrosarcomas, 9 Ewing’s sarcomas and 7 other sarcomas) and 23 for metastatic disease. Thirty of these patients were already metastatic when operated. The average duration of the reconstructive procedure was 45 minutes. Walking started from the fourth to tenth day after operation, but full weight bearing was usually authorised after six weeks.

Postoperative complications were frequent. Seven deep infections occurred, four required ablation of the prosthesis, and one would benefit from a saddle prosthesis. 33% of the patients had postoperative dislocation of the hip prosthesis and 13 patients had to be reoperated. Only two loosenings have been observed – one after deep infection and one after local recurrence in the sacral bone. Oncologic results: With a mean follow-up of five years, 28 patients died of disease and one from an unrelated disease. Four others with disease are still living. Seven local recurrences were observed (four in chondrosarcomas with a contaminated resection). The difficulty in obtaining wide margins explains the high rate of local recurrence (14 %). For patients with localised disease, the five-year overall survival rate is 75% and the five-year disease-free survival rate is 60%.

According to the Society for Musculoskeletal Oncology criteria, orthopaedic results were excellent in 7 patients, good in 30, fair in 6, and bad in 6. The mean functional score of 46 patients who still have their prostheses is 83% with usually no pain, excellent acceptance, length discrepancy of less than 1 cm, average flexion of 100 degrees, and unlimited walking without support.

We conclude that the rapidity and flexibility of this procedure are the positive aspects of this reconstructive technique. However, perfect positioning of the prosthesis remains difficult in a very large peri-acetabular resection. A computed guide is of great help to specify safe margins and prosthesis positioning. Longer follow-up is needed to ensure that the rate of late loosening will not be too high.

A “hands-on” composite gives a similar functional result as a custom-made prosthesis and has a much better function than alternative techniques. Less expensive and more flexible than custom-made prostheses, it can be used even when no part of the iliac wing remains. The use of cement permits the adjunction of antibiotics needed for these complicated cases.

After periacetabular resection for bone sarcoma, a reconstructive procedure is necessary to stabilize the hip, avoid limb discrepancy, and permit full weight bearing. This procedure needs to be easy to perform because resection of the area is time and blood consuming. This leads to the use of a “hands-on” composite prosthesis.

Our reconstructive procedure uses a titanium cup with a long screw that is fixed in the remaining bone (sacrum or spine). When the cup is firmly fixed to the bone, the gap between the cup and bone is filled with cement loaded with antibiotics, and the polyethylene component is cemented on the innominate prosthesis. The femoral component of a usual hip total prosthesis is then implanted.

Since 1990 we have used this reconstructive procedure in 50 patients, 27 with bone sarcomas involving the acetabulum (11 chondrosarcomas, 9 Ewing’s sarcomas and 7 other sarcomas) and 23 for metastatic disease. Thirty of these patients were already metastatic when operated. The average duration of the reconstructive procedure was 45 minutes. Walking started from the fourth to tenth day after operation, but full weight bearing was usually authorised after six weeks.

Postoperative complications were frequent. Seven deep infections occurred, four required ablation of the prosthesis, and one would benefit from a saddle prosthesis. 33% of the patients had postoperative dislocation of the hip prosthesis and 13 patients had to be reoperated. Only two loosenings have been observed – one after deep infection and one after local recurrence in the sacral bone. Oncologic results: With a mean follow-up of five years, 28 patients died of disease and one from an unrelated disease. Four others with disease are still living. Seven local recurrences were observed (four in chondrosarcomas with a contaminated resection). The difficulty in obtaining wide margins explains the high rate of local recurrence (14 %). For patients with localised disease, the five-year overall survival rate is 75% and the five-year disease-free survival rate is 60%.

According to the Society for Musculoskeletal Oncology criteria, orthopaedic results were excellent in 7 patients, good in 30, fair in 6, and bad in 6. The mean functional score of 46 patients who still have their prostheses is 83% with usually no pain, excellent acceptance, length discrepancy of less than 1 cm, average flexion of 100 degrees, and unlimited walking without support.

We conclude that the rapidity and flexibility of this procedure are the positive aspects of this reconstructive technique. However, perfect positioning of the prosthesis remains difficult in a very large periacetabular resection. A computed guide is of great help to specify safe margins and prosthesis positioning. Longer follow-up is needed to ensure that the rate of late loosening will not be too high.

Aims. The risk of postoperative complications after resection of soft-tissue sarcoma in the medial thigh is higher than in other locations. This study investigated whether a vessel sealing system (VSS) could help reduce the risk of postoperative complications after wide resection of soft-tissue sarcoma in the medial thigh. Methods. Of 285 patients who underwent wide resection for soft-tissue sarcoma between 2014 and 2021 at our institution, 78 patients with tumours in the medial thigh were extracted from our database. Information on clinicopathological characteristics, preoperative treatment, surgical treatment (use of VSS, blood loss volume, operating time), and postoperative course (complications, postoperative haemoglobin changes, total drainage volume, and drainage and hospitalization durations) were obtained from medical records. We statistically compared clinical outcomes between patients whose surgery did or did not use VSS (VSS and non-VSS groups, respectively). Results. There were 24 patients in the VSS group and 54 in the non-VSS group. There were no significant differences between the two groups in terms of clinicopathological background. The total drainage volume in the VSS group was significantly less than that in the non-VSS group (1,176 ml vs 3,114 ml; p = 0.018). Moreover, the drainage and hospitalization durations were significantly shorter in the VSS group compared to the non-VSS group (p = 0.017 and p = 0.024, respectively). Conclusion. Our results suggest that use of VSS can help reduce the risk of postoperative complications after wide resection of soft-tissue sarcoma in the medial thigh. Cite this article: Bone Jt Open 2023;4(6):442–446

Paediatric bone sarcomas around the knee are often amenable to either endoprosthetic reconstruction or rotationplasty. Cosmesis and durability dramatically distinguish these two options, although patient-reported functional satisfaction has been similar among survivors. However, the impact on oncological and surgical outcomes for these approaches has not been directly compared. We retrospectively reviewed all wide resections for bone sarcoma of the distal femur or proximal tibia that were reconstructed either with an endoprosthesis or by rotationplasty at our institution between June 2004 and December 2014 with a minimum two year follow-up. Pertinent demographic information, surgical and oncological outcomes were reviewed. Survival analysis was performed using the Kaplan-Meier method with statistical significance set at p<0.05. Thirty eight patients with primary sarcomas around the knee underwent wide resection and either endoprosthetic reconstruction (n=19) or rotationplasty (n=19). Groups were comparable in terms of demographic parameters and systemic tumour burden at presentation. We found that selection of endoprosthetic reconstruction versus rotationplasty did not impact overall survival for the entire patient cohort but was significant in subgroup analysis. Two-year overall survival was 86.7% and 85.6% in the endoprosthesis and rotationplasty groups, respectively (p=0.33). When only patients with greater than 90% chemotherapy-induced necrosis were considered, overall survival was significantly better in the rotationplasty versus endoprosthesis groups (100% vs. 72.9% at two years, p=0.013). Similarly, while event-free survival was not affected by reconstruction method (60.2% vs. 73.3% at two years for endoprosthesis vs rotationplasty, p=0.27), there was a trend towards lower local recurrence in rotationplasty patients (p=0.07). When surgical outcomes were considered, a higher complication rate was seen in patients that received an endoprosthesis compared to those who underwent rotationplasty. Including all reasons for re-operation, 78.9% (n=15) of the endoprosthesis patients required a minimum of one additional surgery compared with only 26.3% (n=5) among rotationplasty patients (p=0.003). The most common reasons for re-operation in endoprosthesis patients were wound breakdown/infection (n=6), limb length discrepancy (n=6) and periprosthetic fracture (n=2). Excluding limb length equalisation procedures, the average time to re-operation in this patient population was 5.6 months (range 1 week to 23 months). Similarly, the most common reason for a secondary procedure in rotationplasty patients was wound breakdown/infection, although only two patients experienced this complication. Average time to re-operation in this group was 23.8 months (range 5 to 49 months). Endoprosthetic reconstruction and rotationplasty are both viable limb-salvage options following wide resection of high-grade bony sarcomas located around the knee in the paediatric population. Endoprosthetic reconstruction is associated with a higher complication rate and may negatively impact local recurrence. Study of a larger number of patients is needed to determine whether the reconstructive choice affects survival

Primary bone tumors are rare, complex and highly heterogeneous. Its diagnostic and treatment are a challenge for the multidisciplinary team. Developments on tumor biomarkers, immunohistochemistry, histology, molecular, bioinformatics, and genetics are fundamental for an early diagnosis and identification of prognostic factors. The personalized medicine allows an effective patient tailored treatment. The bone biopsy is essential for diagnosis. Treatment may include systemic therapy and local therapy. Frequently, a limb salvage surgery includes wide resection and reconstruction with endoprosthesis, biological or composites. The risk for local recurrence and distant metastases depends on the primary tumor and treatment response. Cancer patients are living longer and bone metastases are increasing. Bone is the third most frequently location for distant lesions. Bone metastases are associated to pain, pathological fractures, functional impairment, and neurological deficits. It impacts survival and patient quality of life. The treatment of metastatic disease is a challenge due to its complexity and heterogeneity, vascularization, reduced size and limited access. It requires a multidisciplinary treatment and depending on different factors it is palliative or curative-like treatment. For multiple bone metastases it is important to relief pain and increases function in order to provide the best quality of life and expect to prolong survival. Advances in nanotechnology, bioinformatics, and genomics, will increase biomarkers for early detection, prognosis, and targeted treatment effectiveness. We are taking the leap forward in precision medicine and personalized care

Introduction. Wide, tumor-like resection for chronic osteomyelitis (COM), a standard practice previously, has been challenged recently with adequate, local debridement. This paper reviews the evolution of surgical debridement for long bone osteomyelitis, and presents the outcome of adequate debridement in a tertiary bone infection unit. Materials & Methods. Retrospective review of records from 2014 to 2020 of patients with long bone osteomyelitis. All records were searched electronically and imaging reviewed. All patients were managed by Multidisciplinary Infection Team protocol. Results. 53 patients (54 bones) with median age of 45.5 years (IQR 31 to 55) and mean follow-up of 29 months (12 – 59) were identified. According to Cierny-Mader classification, ten bones were type I, 39 were type III, and five were type IV; via the BACH classification of long bone osteomyelitis 21 were uncomplicated, 32 were complex, and one had limited options. All patients were treated with single-staged management with one planned second stage stabilization. Seventy-five percent grew positive microbial cultures. Forty-six (85%) cases had resolution of COM after index procedure and 51 (94%) had resolution at last follow up. Four (7%) patients underwent second surgical procedure and six (11%) patients had complications. Conclusions. We report high COM resolution rate through detailed pre operative evaluation and planning with multidisciplinary team approach. We challenge the need for wide tumor-like resection and the need for regenerative procedures in all cases of COM. Adequate debridement and local delivery of high concentration of antibiotic appears to provide comparable outcomes

Wide resection, with or without adjuvant therapy, is the mainstay of treatment for soft tissue sarcoma of the extremities. The surgical treatment of soft tissue sarcoma can portend a prolonged course of recovery from a functional perspective. However, data to inform the expected course of recovery following sarcoma surgery is lacking. The purpose of this study was to identify time to maximal functional improvement following sarcoma resection and to identify factors that delay the expected course of recovery. A retrospective chart review was performed of all patients undergoing surgical treatment of a soft tissue sarcoma of the extremities between January 1st, 1985 and November 15, 2020 with a minimum of 1 follow up. The primary outcome measure was time to maximal functional improvement, defined as failure to demonstrate improvement on two consecutive follow up appointments, as defined by the functional outcome measures of Toronto Extremity Salvage Score (TESS) and Musculoskeletal Tumor Society (MSTS) Score or by achieving 90% of maximum outcome score. We identified 1188 patients who underwent surgical resection of a soft tissue sarcoma of the extremities. Patients typically achieved a return to their baseline level of function by 1 year and achieved “maximal” functional recovery by 2 year's time postoperatively. Patient and tumor factors that were associated with worse functional outcome scores and a delayed return to maximal functional improvement included older age (p=0.007), female sex (p-0.004), larger tumor size (p < 0 .001), deep tumor location (p < 0 .001), pelvic location (p < 0 .001), higher tumor grade (p < 0 .001). Treatment factors that were associated with worse functional outcome scores and a delayed return to maximal functional improvement included use of radiation therapy (p < 0 .001), perioperative complications (p < 0 .001), positive margin status (p < 0 .001) and return of disease, locally or systemically (p < 0 .001). Most patients will recover their baseline function by 1 year and achieve “maximal” recovery by 2 years’ time following surgical resection for soft tissue sarcoma of the extremities. Several patient, tumor and treatment factors should be used to counsel patients as to a delayed course of recovery

Introduction. Aneurysmal bone cysts commonly found in lower limbs are locally aggressive masses that can lead to bony erosion, instability and fractures. This has major implications in the lower limbs especially in paediatric patients, with potential growth disturbance and deformity. In this case series we describe radical aneurysmal bone cyst resection and lower limb reconstruction using cable transport and syndesmosis preservation. Materials & Methods. Case 1 - A 12-year-old boy presented with a two-week history of atraumatic right ankle pain. An X-ray demonstrated a distal tibia metaphyseal cyst confirmed on biopsy as an aneurysmal bone cyst. The cyst expanded on interval X-rays from 5.5cm to 8.5cm in 9 weeks. A wide-margin en-bloc resection was performed leaving a 13.8cm tibial defect. A cable transport hexapod frame and a proximal tibial osteotomy was performed, with syndesmosis screw fixation. The transport phase lasted 11 months. While in frame, the boy sustained a distal femur fracture from a fall. The femur and the docking site were plated at the same sitting and frame removed. At one-year post-frame removal he is pain-free, with full ankle dorsiflexion but plantarflexion limited to 25 degrees. He has begun graduated return to sport. Results. Case 2 - A 12-year-old girl was referred with a three-month history of lateral left ankle swelling. X-ray demonstrated an aneurysmal bone cyst in the distal fibula metaphysis. The cyst grew from 4.2 × 2.3cm to 5.2 × 3.32cm in 2 months. A distal fibula resection (6.2cm) with syndesmosis fixation and hexapod cable transport frame were undertaken. The frame was in situ for 13 weeks and during this time she required an additional osteotomy for premature consolidation and had one pin site infection. After 13 weeks a second syndesmosis screw was placed, frame removed, and a cast applied. 3 months later she had fibular plating, BMAC and autologous iliac crest bone graft for slow union. At 3 years post-operative she has no evidence of recurrence, is pain-free and has no functional limitation. Conclusions. We describe two cases of ankle syndesmosis preservation using cable transport for juxta-articular aneurysmal bone cysts. This allows wide resection to prevent recurrence while also preserving primary ankle stability and leg length in children. Both children had a minor complication, but both had an excellent final outcome. Cable bone transport and prophylactic syndesmosis stabilization allows treatment of challenging juxta-articular aneurysmal bone cysts about the ankle. These techniques are especially useful in large bone defects

Introduction. Fibrous dysplasia is a pathological condition, where normal medullary bone is replaced by fibrous tissue and small, woven specules of bone. Fibrous dysplasia can occur in epiphysis, metaphysis or diaphysis. Occationally, biopsy is necessary to establish the diagnosis. We present a review of operative treatment using the Ilizarov technique. The management of tibial fibrous dysplasia in children are curettage or subperiosteal resection to extra periosteal wide resection followed by bone transport. Materials and Methods. A total of 18 patients were treated between 2010 – 2020; 12 patients came with pain and 6 with pain and deformity. All patients were treated by Ilizarov technique. Age ranges from 4–14 years. 12 patients by enbloc excision and bone transportation and 6 patients were treated by osteotomy at the true apex of the deformity by introducing the k/wires in the medullary cavity with stable fixation by Ilizarov device. The longest duration for bone transport was 16 weeks (14–20 weeks) for application, after deformity correction was 20 weeks. We have never used any kind of bone grafts. Results. All the 18 patients were treated successfully by Ilizarov compression distraction device. The patients with localized tibial pathology with deformity had the shortest period on the Ilizarov apparatus, 14 weeks. Conclusions. Preservation and bone regeneration by distraction histogenesis constitutes a highly conservative limb saving surgery. Patients with bone defects of <10 cm, a great deal of preserved healthy tissue and good prognosis are good candidates for these methods

Purpose: Giant cell tumor (GCT) of bone is a rare, usually benign, primary skeletal lesion. The disease’s clinical course may be complicated by local recurrence subsequent to surgical treatment or the development of benign pulmonary metastases. Intra-lesional curettage is the standard treatment of primary GCT of bone. However, the value of intralesional procedures in recurrent GCT has not been well established. Method: Forty-six patients with recurrent GCT of long bones treated between 1983 and 2005 were followed retrospectively. Minimum follow-up was three years; mean follow-up was 11.1 (±4.8) years. Results: Wide resections were performed in 18 patients. Intralesional, joint preserving procedures were performed in 28 patients. Subsequent recurrence occurred in nine patients (20%). Wide resection was performed if joint salvage was not achievable due to expansion of the tumor. Reconstructions following wide resection included arthroplasty (n=4), osteoarticular allograft (n=3), APC (n=1) and fibular autograft reconstruction of the wrist (n=3). Amputations were performed in two patients. Patients undergoing wide resections for local recurrence had a significantly smaller risk of subsequent recurrence as compared to patients treated with intra-lesional surgery (6% versus 32%, hazard ratio: 0.28, p< 0.05). In patients treated with intralesional surgery, application of polymethylmethacrylate (PMMA) in addition to local phenol treatment significantly reduced the risk of subsequent recurrence (PMMA + phenol: 7% vs. Phenol: 25%, hazard ratio: 0.23, p< 0.05). Soft tissue expansion was not associated with an increased risk of subsequent recurrence. At follow-up, all patients with subsequent recurrence were without local disease after additional intralesional surgery (n=3) or wide resection (n=5). Metachronous benign pulmonary metastases evolved in five cases. There was no correlation between the development of pulmonary metastases and the type of treatment of recurrent disease found. Conclusion: In recurrent disease of GCT of long bones and the possibility to salvage the adjacent joint intra-lesional surgery is the treatment of choice independent of whether soft tissue expansion is present. Intra-lesional surgery does not increase the risk of development benign pulmonary metastases. In cases with extensive tumor formation and without the possibility to preserve the adjacent joint wide resection has a high chance for long-term recurrence free disease

Aims: Chordoma is an uncommon malignant tumour with developing the remnants of notochord and usually manifesting itself in patients in their forties and fifties, aggressive local tumour in some cases resulting metastatic progression and might its histological picture show malignancy in long-lived patients. The objective are the presentation of the surgical technique of wide resection in the sacrococcygeal region, discuss the technical aspects of body wall reconstruction and presentation the long-term results of the surgical treatment of sacrococcygeal chordoma in our department. Methods: Although we have treated 48 patients surgically between 1992 and 2005, this lecture includes 37 patients with at least 2 years follow-up. We applied wide resection in all cases and if it was necessary, reconstruction of the body wall was performed by insertingmetal wire frame and artificial fascia layer. Results: We performed 48 wide resections, in 28 cases combined with reconstruction. Most of the patients have had local pain and 26 patients have had preoperative neurological symptoms. It was attained significant local pain relief in 90 percent of the patients and in 77 percent of the patients improvement of the neurological symptoms. Due to radiologically proved local recurrence of the tumour in twelve cases we had to perform second, and in two cases third surgery. Conclusion: The wide resection of sacrococcygeal chordoma following with reconstruction of the body wall is a well designed and safe surgical procedure which results long survival in combining in certain cases with radiotherapy

We retrospectively studied 14 patients with proximal and diaphyseal tumours and disappearing bone (Gorham’s) disease of the humerus treated with wide resection and reconstruction using an allograft-resurfacing composite (ARC). There were ten women and four men, with a mean age of 35 years (8 to 69). At a mean follow-up of 25 months (10 to 89), two patients had a fracture of the allograft. In one of these it was revised with a similar ARC and in the other with an intercalary prosthesis. A further patient had an infection and a fracture of the allograft that was revised with a megaprosthesis. In all patients with an ARC, healing of the ARC-host bone interface was observed. One patient had failure of the locking mechanism of the total elbow replacement. The mean post-operative Musculoskeletal Tumor Society score for the upper extremity was 77% (46.7% to 86.7%), which represents good and excellent results; one patient had a poor result (46.7%). In the short term ARC effectively relieves pain and restores shoulder function in patients with wide resection of the proximal humerus. Fracture and infection remain significant complications

Aims. The aim of this study was to investigate the local recurrence rate at an extended follow-up in patients following navigated resection of primary pelvic and sacral tumours. Patients and Methods. This prospective cohort study comprised 23 consecutive patients (nine female, 14 male) who underwent resection of a primary pelvic or sacral tumour, using computer navigation, between 2010 and 2012. The mean age of the patients at the time of presentation was 51 years (10 to 77). The rates of local recurrence and mortality were calculated using the Kaplan–Meier method. Results. Bone resection margins were all clear and there were no bony recurrences. At a mean follow-up for all patients of 59 months (12 to 93), eight patients (34.8%) developed soft-tissue local recurrence, with a cumulative rate of local recurrence at six-years of 35.1% (95% confidence interval (CI) 19.3 to 58.1). The cumulative all-cause rate of mortality at six-years was 26.1% (95% CI 12.7 to 49.1). Conclusion. Despite the positive early experience with navigated-assisted resection, local recurrence rates remain high. With increasing knowledge of the size of soft-tissue margins required to reduce local recurrence and the close proximity of native structures in the pelvis, we advise against compromising resection to preserve function, and encourage surgeons to reduce local recurrence by prioritizing wide resection margins of the tumour. Cite this article: Bone Joint J 2019;101-B:484–490

Background. There is doubt regarding resection compared to curettage for pelvic metastases. Previous studies have reported that curettage is associated with decreased survival compared with wide resection, and have justified a radical surgical approach to achieve pain palliation and tumor control. Aim. To evaluate the role of wide en bloc resection compared to curettage/marginal resection for patients with pelvic metastases. The rationale was that wide resection does not improve survival even in patients with solitary pelvic metastases. Method. Between 1985 and 2009, 21 patients (6 women, 15 men; age, 34–76 years) were treated for pelvic metastases. Histology included thyroid carcinoma in 5 patients, bladder carcinoma in 4 patients, renal and endometrium in 2 cases each and colon, ovarium, cerebral and lung carcinoma in 1 case each; the primary tumor was undiagnosed in 4 patients. Three patients had sacral and 1 patient had sacroiliac joint metastasis. According to Enneking's classification of the anatomical site involved, 5 patients had type I, 1 patient had type II, 6 patients had type III, 1 patient had type I and II, and 4 patients had type II and III pelvic metastasis. Metastatic disease was localized in the pelvic ring in 15 patients and multifocal in 6 patients. Eight patients had surgical treatment only; 13 patients had surgical treatment in addition to radiation therapy (2 patients), chemotherapy (1 patient), embolization (3 patients), or combined adjuvant treatments (7 patients). 21 patients with pelvic metastases were treated with wide resection (12 patients) and curettage/marginal resection (9 patients) and adjuvants. Sixteen patients had solitary pelvic metastases. Reconstruction of the hip joint was performed in three patients. Results. At a mean of 27.6 months (range, 2–152 months), the overall survival to death and local recurrence was 15% at 66 months and 47% at 26 months, respectively. Survival to death of patients treated with wide en bloc resection was 18% at 46 months compared to 62% at 12months of patients treated with curettage/marginal resection; no difference in survival to death between wide en bloc resection and curettage/marginal resection was observed (p=0.570). Survival to local recurrence of patients treated with wide en bloc resection was 67% at 24 months compared to 26% at 24 months of patients treated with curettage/marginal resection; this was also not statistically significant (p=0.0683). One patient treated with wide en bloc resection for a solitary pelvic bone metastasis had a postoperative complication. Conclusion. This series showed that neither the combination of surgical and adjuvant treatments nor the type of surgical resection were statistically significant parameters for local recurrence. We found no difference in survival to death or local recurrence 1 with wide en bloc resection compared to curettage or marginal resection, even in patients with solitary pelvic metastases

Presentation of two cases of pelvic periacetabular sarcoma, which were treated with wide resection of the tumor, pelvic reconstruction and lower limb salvage. Two patients, one male 23 y.o. with chondrosarcoma and one female 75 y.o. with chondroblastic osteosarcoma, were treated in our clinic. Both tumors were stage II according to Enneking’s classification. Both tumors were treated with Enneking type II internal hemipelvectomy due to their periacetabular localization. After wide resection of tumors, pelvic deficit was reconstructed with allograft, which was internally fixated, and total hip replacement with constrained prosthesis. Clinical evaluation showed absence of pain and satisfactory function of the limb. Imaging evaluation with x-ray, 3D-scan kai MRI showed satisfactory position and condition of allograft and internal fixation without evidence of loosening. Non weight bearing mobilization commenced 3 weeks postoperatively. Internal hemipelvectomy requires precise preoperative planning and surgical knowledge because it is technically demanding due to complex structure of the pelvis, the great number of muscular attachments and the presence of important vessels, nerves and pelvic viscera. Wide pelvic resection and reconstruction with allograft for periacetabular sarcomas is a challenging procedure, which offers the opportunity of limb salvage associated with functional outcome

Purpose of the study: Resection of a malignant primary tumour of the proximal humerus implies sacrifice of a large part of the humeral shaft and the periarticular muscles. Reconstruction can be difficult and raises the problem of preserving function. Recent work has demonstrated the pertinence of combining a glenohumeral prosthesis with an allograft. Several complications are nevertheless reported: non-union, allograft resorption, loosening. We report three cases of malignant primary tumours requiring wide resection of the humerus which were treated by reconstruction with a shoulder arthrodesis applying the induced membrane technique. Material and methods: Three patients (mean age 15 years) presented a malignant primary tumour of the proximal humerus (Ewing sarcoma or osteosarcoma) which was locally extensive but not metastatic. Resection implied resection of 16 cm of the humerus (mean). The same procedure was used for the three patients: first phase: wide resection of the tumour and neighbouring soft tissues which removed the majority of the proximal end of the humerus and the glenohumeral joint, then insertion of a cement spacer; second phase: reconstruction with a shoulder arthrodesis using cancellous grafts positioned inside the induced membrane. Stabilisation was ensured by insertion of a non-vascularised fibula inside the membrane and with a plate fixation on the scapular spine. Results: Mean follow-up is five years. There has been no local recurrence and no distant spread. The arthrodeses and the reconstructions healed without reoperation within six to eight months. The functional outcomes were not different from those obtained with shoulder arthrodesis with a mean elevation of 90°. Discussion: There are many advantages of reconstruction with shoulder arthrodesis using the induced membrane technique: possible wide initial resection, more satisfactory carcinological resection, the periarticular muscles are not pertinent after arthrodesis; there is no need for prosthetic elements or an allograft exposing to later complications; the reconstruction time is a simple procedure; elevation remains satisfactory. Conclusion: This technique should be included in the surgical armamentarium just like vascularised transfers, allografts and massive prostheses. The indication should be reserved for extensive resection

Aims. The aim of this study was to identify factors that determine outcomes of treatment for patients with chondroblastic osteosarcomas (COS) of the limbs and pelvis. Patients and Methods. The authors carried out a retrospective review of prospectively collected data from 256 patients diagnosed between 1979 and 2015. Of the 256 patients diagnosed with COS of the pelvis and the limbs, 147 patients (57%) were male and 109 patients (43%) were female. The mean age at presentation was 20 years (0 to 90). Results. In all, 82% of the patients had a poor response to chemotherapy, which was associated with the presence of a predominantly chondroblastic component (more than 50% of tumour volume). The incidence of local recurrence was 15%. Synchronous or metachronous metastasis was diagnosed in 60% of patients. Overall survival was 51% and 42% after five and ten years, respectively. Limb localization and wide surgical margins were associated with a lower risk of local recurrence after multivariable analysis, while the response to chemotherapy was not. Local recurrence, advanced patient age, pelvic tumours, and large volume negatively influenced survival. Resection of pulmonary metastases was associated with a survival benefit in the limited number of patients in whom this was undertaken. Conclusion. COS demonstrates a poor response to chemotherapy and a high incidence of metastases. Wide resection is associated with improved local control and overall survival, while excision of pulmonary metastases is associated with improved survival in selected patients. Cite this article: Bone Joint J 2019;101-B:739–744

Aim. Giant cell tumour (GCT) of bone is a benign but locally aggressive tumour. Although topical adjuvants have been used in the past, local recurrence following intralesional excision of GCT of bone continues to remain a problem. The use of bisphosphonates as an anti-osteoclastic agent in the management of osteolytic bone metastases is well accepted. Therefore our study aims to retrospectively demonstrate whether the administration of bisphosphonate as an adjuvant can control aggressive local recurrence of GCT and prevent wide resections of bones or amputations. Method. A retrospective study was performed between 2004 and 2010. 6 patients were diagnosed with aggressive local recurrence of appendicular GCT. All patients were treated for the primary tumour by surgical curettage and cryoablation followed by cementation or biological reconstruction. In 5 patients the tumour was located in the distal radius and in one in the first metacarpal bone. All recurrences were in the bone with large soft-tissue extension. After histological diagnosis – by CT core needle biopsy – the patients were treated by intravenous bisphosphonate, followed by clinical & radiological assessments. Results. Average follow-up of 42 months, ranging from 12 to 72 consecutive months. All patients showed good response to bisphosphonate treatment: lesions become calcified gradually as shown in x-rays & CT scans, reduction in size of soft tissue components, patient reported relief of pain & improvement of the affected limb. All treated patients did not report any untoward effects. Conclusion. In the current study bisphosphonate treatment is found to be an effective treatment for local control of aggressive local recurrence of GCT of the extremities and can therefore be a good alternative to wide resections of bone and complicated reconstructions. Functional results are shown to be promising as well. The study results need further investigation & a larger scale of patients

Introduction and Objectives: Synovial sarcoma is an infrequent mesenchymal neoplasia (between 8–10% of soft tissue sarcomas) that can originate in the joint capsule, bursa and tendon sheaths. Materials and Methods: We studied 52 cases of synovial sarcomas reviewed between 1983 and 2006, with a mean follow-up of 91.4 months (24–204 months). The mean age of the patients was 38.4 years of age (range 13–86). The most frequent location was the knee and the popliteal area. In 25 cases a wide resection was performed and in 17 cases amputation was carried out. The most frequently used treatment protocol was preoperative chemotherapy plus postoperative chemo and radiotherapy. In one of the recurrence cases isolated perfusion of the limb was performed due to a recurrence of synovial sarcoma in a hand. Results: One or more recurrences during follow-up were seen in 24 patients. There were 2 cases (3.8%) of skin and cervical spine metastasis and 20 cases of lung metastasis. Eleven patients died (19%). The survival rate at 5 years was 61%. Discussion and Conclusions: Synovial sarcoma is the third most frequent soft tissue sarcoma. It is an aggressive entity, with a high recurrence rate and considerable mortality. When it is not possible to achieve safe margins the ideal treatment is a wide resection or amputation. Neoadjuvant or adjuvant therapies have an important role in treatment. Treatment by a multidisciplinary team is indispensable to improve survival rates

It is well known that wide resection and reconstruction with modular or composite prostheses is the treatment of choice in high chondrosarcoma of metasepiphyseal bone. Nevertheless there is a debate concerning the treatment of low grade chondrosarcoma, a locally aggressive tumour, similar also histologically to benign lesion. Two different therapeutic options are reported in these lesions: wide resection and intralesion curettage. Between 1995 and 2003 the Authors analysed a series of 37 cases of low grade chondrosarcoma of long bone treated with curettage and local adjuvant, like liquid nitrogen and acrylic cement, if necessary associated with synthesis. The least follow-up was two years. The authors observed 3 local recurrences within the first 12 months from the surgical treatment; and, in every case, an increased grade of malignity was observed at histology. The Authors confirmed that the aggressive intralesional treatment with the use of the local adjuvant like liquid nitrogen and cement, is a valid therapeutic possibility in these lesions, but they confirm that it’s necessary an accurate preoperative diagnosis with also open biopsy for an efficacy treatment

Undifferentiated pleomorphic sarcoma/NOS (former pleomorphic and storiform MFH) of the extremities is a common malignant soft tissue tumor in adults. The objective of this study is to determine prognostic factors for the outcome after surgical treatment with respect to the recent developments in classification. From 1996 to 2004, 140 undifferentiated pleomorphic sarcomas/NOS were identified out of 1200 soft tissue sarcomas of the extremities that were treated at our institution and recorded in a prospective database. Overall survival (OS) and isolated local recurrence (ILR) were determined by Kaplan-Meier analysis. All tumors were retrospectively analyzed regarding prognostic factors of the disease, including patient’s background (primary or recurrent), histological grade (G2/G3), adjuvant chemotherapy and radiotherapy, size (T1-2) and depth of the tumor, and surgical margins (R0, R1, R2). In 123 patients, a wide resection was performed (limb sparing surgery). In 9 patients, an amputation was necessary. The overall 5-year survival rate was 72% (median follow-up: 52 months). There was a significant difference between the group presenting with primary tumors (5y survival: 84%, p< 0.05) and recurrent tumors (5y survival: 62%, p< 0.05). Isolated local recurrence occurred in 36 patients. In terms of OS and ILR, primary or recurrence, negative surgical margins, size and grading had a highly significant influence, whereas the site of surgery and adjuvant chemotherapy, adjuvant radiation and tumor depth did not. Prognosis for patients with undifferentiated pleomorphic sarcoma of the extremities depends predominantly on adequate wide resection of the primary tumor

Introduction. Local recurrence of Giant cell tumours of bone (GCT) is considered the main complication of surgical treatment (50%). Intra-lesional curettage with adjuvants like phenol or polymethylmethacrylate (PMMA) is recommended as initial treatment, decreasing the risk of recurrence. However, risk factors for local recurrence in skeletal GCT have not yet been firmly established and a golden standard for treatment remains controversial. Aim of this study is identification of risk factors for recurrence in GCT, specifically after intra-lesional curettage with or without adjuvants. Methods. In a retrospective single-institution study 191 patients treated for GCT between 1964 and 2009 were included. Mean follow-up was 111 months (range 12-415). The recurrence-free survival and hazards for different treatment strategies and various patient and tumour characteristics were determined. Results. Overall risk of recurrence was 36.1% (n=66, 95% CI: 28.3-42.1). Recurrence rate after wide resection was 20%, after curettage with adjuvants 33% and after curettage alone 77%. Hogendoorn-grade III (Hazard Ratio: 5.7, p⋋0.001), localisation in axial skeleton (HR: 3.7, p⋋0,001), primary treatment in a non-specialised centre (HR: 2.8, p⋋0.001) and extension into soft tissue (HR: 2.0, p=0.02) were significant risk factors for local recurrence. Curettage with adjuvants proved superior to curettage alone (p⋋0.004 p=0.07, HR: 0.54), but the application of both PMMA and phenol did not present a significantly better outcome than PMMA alone (HR: 1.07, p=0.9). Discussion. Of all possible risk factors only soft tissue extension and localisation in distal radius significantly influenced the risk of local recurrence for all treatments. We found that high-grade tumours and localisation in the axial skeleton were additional risk factors for local recurrence after intra-lesional surgery. Although wide resection increases morbidity, it can be the therapy of choice in high risk patients. Intra-lesional therapy can be advised for low risk patients using curettage adjuvants and PMMA

Although the recurrence rate of giant cell tumors of bone (GCTB) is relatively high exact data on treatment options for the recurrent cases is lacking. The possible surgical procedures range from repeated intralesional curettage to wide resection. 214 patients with histologically certified GCTB have been treated at the authors department from 1980 to 2007. 67 patients with at least one local recurrence were included in this study. The mean follow-up was 77.3 months. The data was evaluated according the re-recurrence rate with regard to the surgical procedure for the recurrence. The mean time until the first local recurrence was 22.0 months; the mean number of recurrences per patient was 1.4. The recurrence occurred in 69.7 % (46 out of 66 patients) within the first two years. If after intralesional procedures (curettage or intralesional resection) no adjunct was used the re-recurrence rate was 58.8 % (10 out of 17 patients) and decreased to 21.7 % (5 out of 23 patients) if a combination of all adjuncts (PMMA + burring) was used. The likelihood of re-recurrence was reduced by the factor 5.508 which was clearly significant (p = 0.016). In case of wide resection no re-recurrence occurred. Seven patients (10.5 %) developed pulmonary metastases. Fourteen patients (20.9 %) finally received an endoprosthesis; 12 due to tumor recurrence, 2 due to secondary arthritis. Recurrent GCTB can be treated by further curettage with additional burring and cementing with an acceptable re-recurrence rate of 21.7 %. The rate of patients finally needing an endoprosthesis is 20.9 %. Due to the high rate of pulmonary metastases recurrent GCTB may be considered as a severe disease

Wide resection of bone tumour has become an accepted treatment in the limb salvage surgery. The reconstruction of the residual defect following wide resection is a major problem. Author had reviewed the results of five reconstruction methods. Osteoarticular graft is suitable for proximal tibial reconstruction and endoprosthesis for distal femoral reconstruction. Autograft is rarely used to reconstruct the large residual defect. Cement can reconstruct the larger defect, but it is not a suitable procedure on long term basis. Health technology has been defined by WHO as the set of techniques, drugs, equipment and procedure used by health care professionals in delivering medical care to individuals and the system within which such care is delivered. Health technology assessment includes analyses of safety, efficacy and effectiveness, cost and cost effectiveness, infrastructure factors, social impact and fit, needs and capabilities of local health care delivery system. The reimplanatation of resected autoclaved tumour bone graft is technically a simple, financially a cost saving and a biological solution for this difficult problem. This method of reconstruction fulfills all criteria of health technology. It is the suitable method of reconstruction in limb salvage surgery for all countries, but most suitable for the developing and poor countries where the resources for other methods are not available due to financial, technical or socio-cultural reasons

Synovial sarcoma (SS) is rare but increasingly diagnosed and associated with poor prognosis. Primary surgical resection with wide margins and adjuvant radiation-therapy is considered gold standard in treatment of primary SS. Although (Neo)adjuvant chemo- and radiationtherapy are used in the primary treatment of SS, they are not advocated outside a clinical trial setting. In patients with primary SS and pulmonary metastases, (neo)adjuvant chemotherapy is often added to the treatment protocol but it’s effect on overall survival seems limited. Between 1985 and 2004 33 patients with primary SS were treated in our clinic. Seventeen patients were diagnosed with pulmonary metastases at presentation (9) or during postoperative follow-up (8). Wide resection or focally marginal resection followed by radiotherapy was used as primary treatment for all patients. All primary metastasized patients were treated with adjuvant multi-agent chemotherapy including Isofosfamide. Average survival in this group was 32 months (5 year OS 50%), compared to 60 months in the late metastasized patient-group (2 and 5 year OS 50 and 11%). Wide resection was not related to improved overall survival when compared to marginal margins and additional radiation therapy. In the early metastasized group combined chemo-radiaton therapy provided no significant improvement in overall survival over adjuvant chemotherapy or radiation therapy alone. However additional chemotherapy in the late metastasized group was slightly associated with increased overall survival (5 year OS 0% vs 66%). Treatment of early pulmonary metastasized SS remains highly dependent of the individual preference of patient and physician. In contrast to the reported prolonged disease free/overall survival of Enneking stage IIA and IIB SS patients, aggressive surgical and chemo-radiation therapy has not yet been associated with improvement of disease free/overall survival in stage III disease. Patients presenting with late pulmonary metastasis might benefit from adjuvant multi-agent chemotherapy treatment

Sternal resection is commonly performed for primary and metastatic chest wall tumours involving the sternum or the ribs near the sternum and, in case of wide resections, it is necessary to restore the stability of the chest wall. We analyze our experience with emphasis on surgical management and survival. From 2001 to 2007, 16 patients underwent surgical resection of the sternum for malignant lesions: 10 (62.5%) primary lesions (chondorsarcoma n=8; osteosarcoma n=2) and 6 (37.5%) secondary lesions (4 local recurrence from breast cancer and 2 metastases). We performed 12 partial resections (resected area from 65 to 20 %), 2 subtotal resections (about 90% of total area) and 1 total resection. Chest wall stability was obtained by prosthetic material, rigid and non rigid, and muscolar flaps. As non rigid material we used a polytetrafluoroethylene patch (Gore-tex Dual Mesh Plus) while replacement after total sternectomy was performed using a new rigid system of mould-able titanium connecting bars and rib clips (Strasbourg Thoracic Osteosyntheses System, Medxpert, GMbH). Prosthetic material was combined in 3 cases with a latissimus dorsi muscolar flap, in 1 case with a vertical rectus abdominis muscolar flap, in 12 with a pectoralis major flap. There was no perioperative mortality or significant morbidity. All patients were extubated within 24 hours after operation. At a mean follow-up of 44.1 months (range 82–14), 5 years actuarial survival for primary tumours was 85%, while 3 years actuarial survival after resection of secondary tumours was 39% (median 20,5 months). In case of primary lesion wide resection with tumour-free margins is necessary to minimize local recurrence and to contribute to long-term survival; reconstruction with a rigid system composed of mould-able titanium bars and rib clips allows to plan extensive demolition minimizing the risk of chest wall instability. In metastatic disease surgery can provide good palliation, although survival is poor

Epitheloid haemangioendothelioma is a rare tumour of vascular origin. It is characterised by the appearance of epitheloid endothelial cells and occurs typically in soft-tissue, skin, and liver. Less frequently it is found in bone. The tumour is more often located in the long bones of the lower extremities, and the pelvis than in the upper extremities, vertebral column, and flat bones. The lesion nearly affects all age groups and there is a male predilection. Case 1: A 71-year old woman had pain in the area of her right hip after a downfall. X-ray showed a lucency of the cortical substance of the right femur. Scintigraphy showed a cortical lesion, oedema of the bone-marrow and an involvement of soft-tissue. Carcinoembryonic antigen, CD 31, and CD 8 were positive. An open biopsy verified an epitheloid haemangioendothelioma. Staging was negative. A wide resection of the proximal femur and reconstruction with a tumour-prosthesis were performed. Four months later the patient had osteolytic metastases of os ilium, os pubis, acetabulum and in the fifth lumbar vertebra. The patient died 8 months after the wide resection of the tumour because of myocardial infarction. Case 2: An epitheloid haemangioendothelioma of the liver was diagnosed in a 21-year old male patient. Twelve years after the primary tumour the patient had osteolyses of the first cervical vertebra, manubrium sterni, and ribs. An open biopsy verified the metastatic spread. Local radiotherapy was performed. Furthermore the patient developed a destruction of processus spinosus and a pathologic fracture of first thoracic vertebra. The patient died of metastatic disease 2 years later or 14 years after the initial diagnosis. Epitheloid haemangioendothelioma of bone is a rare tumour and the diagnosis is quite difficult. Metastatic rate is about 20–30% and mortality about 10–20%. As presented in our cases bone involvement could either be attributed to primary haemangioendotheliomas of bone or to metastases of non-osseous forms. As in our cases it has been reported, that predicting prognosis is difcult, however nuclear atypia, mitotic activity, spindling of cells, and necrosis have been reported as negative prognostic factors

Limb salvage involving wide resection and reconstruction is now well established for managing musculoskeletal sarcomas. However, involvement of major nerves and vessels with a large volume of muscle and skin may result in a useless limb, contributing to depression and a low quality of life. We have been studying alternative treatments for musculoskeletal sarcoma since 1990, and have recently established a regime using photodynamic surgery with cells labelled with acridine orange, photodynamic therapy with cells treated similarly and radiodynamic treatment using the effect of X-rays on such cells. These techniques have been used after marginal or intralesional resection of tumours since 1999 and have enabled maintenance of excellent limb function in patients with sarcomas

Background. Giant cell tumours of bone (GCT) are benign bone tumours with a locally aggressive character. Local recurrence is considered the main complication of surgical treatment and is described in up to 50% of patients. Intralesional curettage with the use of adjuvants like phenol or polymethylmetacrylate (PMMA) is recommended as initial treatment, significantly decreasing the risk of recurrence. However, risk factors for local recurrence in skeletal GCT have not yet been firmly established and a golden standard for local therapy remains controversial. Objective. The identification of risk factors predisposing for an increased risk of local recurrence. In addition, different surgical techniques are compared to identify the optimal surgical approach for the identified risk factors. Methods. In a retrospective study all 215 patients with bone GCT treated between 1964 and 2009 in one centre were included, of which 193 were suitable for analysis. All patients had minimal follow-up of 12 months (mean 115; range 12–445). Using a Kaplan Meier survival analysis recurrence free survival rates were calculated. Cox-regression was used to determine the influence of different types of therapy, the use of adjuvants, and various patient and tumour characteristics. Results. The mean local recurrence rate for all patients was 35.2% (n=68, 95%CI: 28.3–42.1). Recurrence rate after wide resection was 0.17 (n=6, 95%CI: 0.04–0.29), after curettage with adjuvants 0.32 (n=42, 95%CI 0.24–0.41) and after curettage alone 0.74 (n=20, 95%CI: 0.57–0.91, p < 0.001). Soft tissue extension (Hazard Ratio: 3.8, p < 0.001), localisation in radius and ulna (HR: 2.6, p=0.013), and surgical experience (HR: 2.2, p=0.022) were identified as significant general risk factors for local recurrence. For intralesional resection, Campanacci grade III (HR: 3.9, p=0.019) and location in axial skeleton (HR: 3.3, p=0.016) additionally significantly increased this risk. Comparing treatments our data showed that curettage followed by adjuvants was superior to curettage alone (p < 0.004), and the application of both phenol and PMMA did not present a significantly better outcome than curettage and PMMA alone (HR: 1.07, p=0.881). Conclusion. Of all possible risk factors only soft tissue extension, localisation in radius and ulna and non-radical resections significantly influenced the risk of local recurrence for all treatments. In addition, we found that high-grade tumours and localisation in the axial skeleton were additional risk factors for local recurrence after intralesional surgery. Although wide resection increases patient morbidity, it can be the therapy of choice in high risk patients. Intralesional therapy can be advised for low recurrence risk patients using curettage and PMMA only, whereas our study could not confirm the predicted effect of phenol as an additional adjuvant

Goals: Sarcomatous degeneration of giant cell tumours (GCT) occurs rarely. It occurs in less than 1% of the cases, and most of them are GCT previously treated with radiotherapy. The goal of this presentation is to review the CGT cases treated at our unit that have evolved towards malignization. Methods: Retrospective study of 96 GCT treated at our Hospital between 1983 and 2005. 5 presented sarcomatous degeneration in their evolution. These were the cases of 3 men and 2 women with a mean age of malignization of 42 years (32 years – 54 years). The median follow-up period was 155 months (5 months – 209 months). 3 cases affected the distal femur, one case affected distal radius and one case affected proximal humerus, with a slight tendency to the right hemibody. The primary treatment for GCT in these patients was curettage and bone graft. Only one case had received previous radiotherapy. In the same period of time we had two cases of lung dissemination of CGT with typical histology, without previous malignization of tumour. Results: Malignization takes place, on average, at the 1.8th recurrence (1.3). Histologically, we find 3 osteosarcomas and 2 indifferentiated tumours. Three patients developed distant dissemination; 2 patients died due to lung metastases, with a mean time between the first surgery and the sarcomatous degeneration of 90 months (40 monts – 183 months) and a mean time between malignization and mestastases of 22.3 months (9 months – 34 months) The treatment, once the malignization was diagnosed, consisted in wide resection and substitution with mega-arthroplasty in cases of distal femur and osteoarticular graft at the shoulder. 2 cases required amputation of the affected limb due to irresecable recurrence in soft tissues. Conclusions: There is no predictive criteria of which type of primary typical CGT will evolve into sarcoma. The malignization always has as a result high grade sarcomas, with a high tendency to hematogenous dissemination. When lung metastases appear the survival prognosis is a number of months. We must suspect malignization of a benign CGT when one of the relapses shows a very rapid growth with radiologic aggressive characteristics; in these cases we prefer wide resection of the tumour instead of curettage and thus we prevent the possible sarcomatous degeneration

Introduction. Rotational or axial alignment is an important concept in total knee surgery. Malrotation of the femoral component can lead to patellofemoral maltracking, pain and stiffness. In reconstruction surgery of the knee, achievement of correct rotation is even more difficult because of the lack of anatomical landmarks. The linea aspera is often the only remaining landmark, but its reliability is questionable. Goal of research. Can custom-made 3D-guides help with rotational alignment of the knee after a wide resection of the distal femur?. Material and methods. Custom-made 3D-guides were designed from CT-scans, with the help of the commercially available Mimics software (Materialise NV, Leuven, Belgium) and SolidWorks (SolidWorks Corp., MA, USA). Anterior was defined as 90° relative to the PCL, with the center of the best-fitting inner cylinder, inside the femoral diaphysis, as rotation point. Firstly, the accuracy of the 3D-guides was tested. Twelve 3D-guides, on different heights, were made for 3 cadaveric femora. Anterior was marked with a pin and the position was evaluated with CT-scan. Secondly, to mimic surgery, seven reconstruction prostheses were placed in 4 cadavers, using the 3D-guide to indicate anterior and cutting surface. Resection height was aimed at 13cm. The position of the prostheses was also evaluated using CT-scan. Results. First test: The pins deviated on average 0.65° (SE: 0.75°) from anterior. Eighty-three percent deviated less then 1° from anterior, and only 2 pins deviated more than 1° (1.5° and 2.6°). The resection height indicated by the 3D-guide was on average 2.4mm (SE: 0.7mm) to high. Second test: The 7 reconstruction prostheses deviated on average 3.1° (SE: 2,18°) from anterior, with 4 prostheses deviating more than 1°. The 2 prostheses in endorotation were placed more lateral then was planned, while the 2 in exorotation were placed more medial. Deviation in the coronal and sagittal plane was respectively 1.56° (SE: 1.64°) and 1.84° (SE: 1.04). The mean height was 12.9cm. Discussion. The 3D-guides were accurate in indicating a previously established ‘anterior’ point on the femur and the resection height, but when used to position the femoral component during surgery they inadequately controlled rotation. The 3D-guides did not take into account that centering of the prosthesis could be a problem. When the prosthesis was place more medial or more lateral than anticipated the rotation point of the component was changed and when then aligned with the previously indicated anterior mark, it was placed respectively in exorotation and endorotation. Future research. Will aim to develop custom-made 3D-guides that also guide centering of the femoral component. Repercussion on function and kinematics of improved axial alignment will be evaluated with knee simulator testing and a control group

Giant cell tumour is the most common aggressive benign tumour of the musculoskeletal system and has a high rate of local recurrence. When it occurs in proximity to the hip, reconstruction of the joint is a challenge. Options for reconstruction after wide resection include the use of a megaprosthesis or an allograft-prosthesis composite. We performed a clinical and radiological study to evaluate the functional results of a proximal femoral allograft-prosthesis composite in the treatment of proximal femoral giant cell tumour after wide resection. This was an observational study, between 2006 and 2012, of 18 patients with a mean age of 32 years (28 to 42) and a mean follow-up of 54 months (18 to 79). We achieved excellent outcomes using Harris Hip Score in 13 patients and a good outcome in five. All allografts united. There were no complications such as infection, failure, fracture or resorption of the graft, or recurrent tumour. Resection and reconstruction of giant cell tumours with proximal femoral allograft–prosthesis composite is a better option than using a prosthesis considering preservation of bone stock and excellent restoration of function. A good result requires demanding bone banking techniques, effective measures to prevent infection and stability at the allograft-host junction. . Cite this article: Bone Joint J 2014; 96-B:1106–10

Six patients underwent wide segmental resection and limb salvage surgery for primary or metastatic bone tumors involving the diaphysis of the femur, the tibia and the humerus using a modular intramedullary diaphyseal segmental defect fixation system. There were 4 men and 2 women with a mean age of 62 years (range, 40 to 77 years). Histological diagnosis included adamantinoma, dedifferentiated synovial sarcoma attached to the tibia, multiple myeloma, and metastatic renal cell carcinoma, myeloid carcinoma of the thyroid gland and metastatic adenocarcinoma of the stomach. The mean follow-up was 16 months (range, 11 to 24 months). At the latest examination, 5 patients were free of local or distant disease; one patient had deceased with distant disease, without evidence of local recurrence. Revision surgery was necessary in one patient because of mechanical loosening of the proximal fixation of the prosthesis. The mean increase of the Enneking rating from the pre to the postoperative status was 87.82%. The intramedullary diaphyseal segmental defect fixation system used herein is associated with a satisfactory functional and oncological outcome after wide resection of diaphyseal bone tumors

Between 1996 and 2003, 16 patients (nine female, seven male) were treated for a primary bone sarcoma of the femur by wide local excision of the tumour, extracorporeal irradiation and re-implantation. An additional vascularised fibular graft was used in 13 patients (81%). All patients were free from disease when reviewed at a minimum of two years postoperatively (mean 49.7 months (24 to 96). There were no cases of infection. Primary union was achieved after a median of nine months (interquartile range 7 to 11). Five host-donor junctions (16%) united only after a second procedure. Primary union recurred faster at metaphyseal junctions (94% (15) at a median of 7.5 months (interquartile range 4 to 12)) than at diaphyseal junctions (75% (12) at a median of 11.1 months (interquartile range 5 to 18)). Post-operatively, the median Musculoskeletal Tumour Society score was 85% (interquartile range 75 to 96) and the median Toronto Extremity Salvage score 94% (interquartile range 82 to 99). The Mankin score gave a good or excellent result in 14 patients (88%). The range of movement of the knee was significantly worse when the extracorporeally irradiated autografts were fixed by plates rather than by nails (p = 0.035). A total of 16 (62%) of the junctions of the vascularised fibular grafts underwent hypertrophy, indicating union and loading. Extracorporeal irradiation autografting with supplementary vascularised fibular grafting is a promising biological alternative for intercalary reconstruction after wide resection of malignant bone tumours of the femur

Objective: Modular tumour prostheses are often chosen for the reconstruction of osseous or joint defects following wide tumour resection in limb salvage procedures. In this retrospective trial we were looking for the clinical use in accordance to long-term-follow up especially on aseptic loosening of stem, wear of polyethylene, implant related complications and clinical and functional results. Methods: From 1996 to 2008 we performed in our clinic in 69 cases a modular distal femur replacement (MUTARS) after wide bone or soft tissue tumour resection. In our outpatient clinic we have assessed the clinical follow-up as clinical examination (Enneking-score) and standardized radiological follow-up for 5 years, then once per year. In the focus of interest were aseptic loosening of the stems, wear of polyethylene, and mechanical problems as implant failure. Results: In long-term-follow-up 6 polyethylene locks had to be changed into PEEK locks (8,6%9). PEEK-lock complications were not seen in this series. In 5 cases late infection of the prosthesis occured. In another 5 cases aseptic loosening of the prosthesis was diagnosed, fractures of the stems were not seen. We conclude that in tumour patients with major osseous reconstruction after wide resection a certain loss ob function cannot be avoided, but the rate of complications in the long-term-follow-up after implantation of modular tumour prosthesis is acceptable

Osteosarcoma arising on the periosteal aspect of bone comprises a biologically heterogeneous group of neoplasm. Parosteal osteosarcoma is a low-grade malignant tumour originates at the surface of bone comprising 3–6% of all osteosarcomas and 2% of primary osseous neoplasms. It is most common in young and middle-aged adults and occurs most frequently on the posterior aspect of the distal femur or tibia. The radiologic appearance is often characterized by a large, lobulated, ossific mass in a juxtacortical position. Cortical thickening without aggressive periosteal reaction can be present. Typically the medullary canal is uninvolved. Wide surgical resection and reconstruction is the treatment of choice. The overall prognosis for patients with this lesion depends on the stage of the tumor at presentation. The prognosis for a Parosteal osteosarcoma is generally excellent. We present an uncommon case of elderly women 70 year-old with a surface osteosarcoma in right femur concomitant with disseminated Breast Carcinoma. The radiological findings showed a juxtacortical mass on the anteromedial aspect of the junction between the mid-third and the distal third of the right femur with areas of new bone formation mimicking Periostal osteosarcoma. We observed a typical Parosteal osteosarcoma when the biopsy was performed. The elected treatment was a wide resection with PTR MUTARS and adjuvant chemotherapy controlling local and systemic diseases. In conclusion, due to the treatment for the juxtacortical osteosarcomas varies with the diagnosis, an accurate evaluation and appropriate management must be executed to have the best outcomes

Condromixoid sarcoma is a rare tumor (about 2,3% of soft tissue sarcomas in one of the series published) occurring mainly in muscular part of extremities. The reconstruction after block resection of tumor lesions of dorsal column invading the thorax almost always represents a great challenge to the surgical team. The case presented reports an infrequent location of this rare tumor what highlights it in an oncologic point of view. From the surgical point of view the surgical steps of wide tumor resection are described and of the reconstruction of the dorsal column and the involved thoracicregion (adjacent to vital structures) what resulted in an asymptomatic correction. The authors present a case of a 47 years old patient operated to a volumous dorsal condromixoid sarcoma, practically asymptomatic, with invasion and compression of the neurological space and thoracic cavity. After biopsy, a wide resection of the tumor was made, using a double surgical approach (anterior and posterior), with resection of posterior part of vertebras D6–D9 and part of the 7th, 8th and 9th costal arches. The reconstruction consisted in correction of thoracic wall with prosthesis and stabilization of column with pedicular instrumentation from D5 to D11. The post-operatory recover had no complications and in clinically the patient is asymptomatic. Only the elevated level of suspicion conducted the realization of biopsy in an apparent innocent lesion. The Condromixoid sarcoma occurs rarely in the nervous axis, what created some difficulties in the histological diagnosis. The dimensions of the tumor mass and its localization were object of great discussion and of detailed surgical planning. After a massive surgical resection, the clinical result after 2 years of follow-up is excellent (patient asymptomatic). The almost inevitable oncological decision of surgery in a malignant tumor with medullar cord compression was the only effective way of treatment

Myxoid or Myxoid Round Cell liposarcoma (MLS) is a mesenchymal malignancy with adipocyte differentiation accounting for 15–20% of liposarcomas and 5% of all adult soft tissue sarcomas (STS). Like other STS, treatment of MLS is generally by wide surgical resection in conjunction with radiotherapy and this approach is associated with low rates of local recurrence [1]. However, most MLS are located between muscles so wide local excision(WLE) can be quite morbid with adverse functional results. MLS are known to be extremely sensitive to radiotherapy which has led to development of treatment protocols utilizing neoadjuvant radiation. Given the radiosensitivity of MLS, we hypothesize that resection with marginal margins (1mm) does not result in higher rates of local recurrence or disease-free survival if performed following pre-operative radiotherapy. We identified all patients with localized MLS who underwent preoperative radiation and surgical resection between January 2000 and January 2018 from a prospectively collected sarcoma database. We calculated the ellipsoid tumour volume(ETV=h x w x d x ()) at diagnosis and after radiation, and documented the necrosis percentage of the tumour and margin status following resection based on histological analysis [2]. Marginal resection was identified by the operative report describing no or only very minimal resection of muscle surrounding the tumour, and a negative surgical margin 0.1cm/1mm as defined histologically. In comparison, WLE was defined if the operative note described an attempt to remove the tumour with a cuff of surrounding muscle, and the final histology reported a negative margin 0.1cm/1mm which included skeletal muscle. The third group for analysis included patients with positive resection margins. There were 91 patients with MLS of mean age 48 years (range 18–88) with 69% male and 31% female. The mean pre-radiotherapy ETV was 507cm. 3. (16.1cm. 3. to 6961cm. 3. ) and decreased to 361cm. 3. (8.7cm. 3. to 5695cm. 3. ) following radiation, with an average percentage reduction in ETV of −45%(+129%to-99%). Mean patient follow-up was 7.3 years (0.2–18) with no differences between margin groups(p=0.284). Only 10(11%) patients had positive margins, of which 43(47%) underwent marginal resection and 38(42%) WLE. Overall the mean necrosis as measured histologically following preoperative radiation was 69% (range 0–100%) with no differences between the 3 resection groups(p-0.151). Similarly, there was no significant difference in reduction in mean ETV between the three resection groups(p=0.311), there was no significant difference between the three treatment groups in 5-year LR-free survival (p=0.469), metastasis-free survival (p=0.841) or overall survival (p=0.873). Most patients with MLS experienced significant reduction in tumour size and necrosis following preoperative radiation. We found no differences in the risk of local recurrence or survival based on the type of surgical approach for patients with MLS. Based on these results, we conclude that MLS can be safely removed by marginal resection following preoperative radiotherapy with high rates of local tumour control. Even following a positive margin resection, local recurrence remains uncommon after preoperative radiation. A marginal surgical approach for patients with MLS reduces the need for major muscle resection and is associated with improved functional outcomes with less complications

Dermatofibrosarcoma protuberans (DFSP) is a rare, monoclonal dermal neoplasm. DFSP is known to be locally aggressive and infiltrative, but with a very low systemic recurrence risk. It is reported to be associated with high local recurrence rates following surgical excision. Positive or marginal resection margins can lead to a high risk of local recurrence. The objective was to determine the oncologic outcome for DFSP treated at our institution. We reviewed our prospectively collected database for all DFSP treated at our unit between 1990 and 2016. Patients were included whether or not they had excision prior to referral (“whoops” procedure). Those with fibrosarcomatous degeneration at presentation to our unit or less than 1 year of follow-up were excluded. The goal of surgery was a negative margin with a minimum margin of 2 cm where possible. Patients were followed up after surgery to monitor complications, recurrence, transformation and/or metastasis. 139 patients with a mean age of 42.7 (SD=14.1) were included. Mean follow-up was 56 months.101 patients had prior “whoops” surgery before referral. 14 patients were also treated with radiotherapy (13 preoperatively, 1 postoperatively). Following surgery, 6 patients had positive margins, 4 underwent radiation treatment while the other 2 had no further treatment. One patient who presented to our unit with a local recurrence developed a further local recurrence, which demonstrated fibrosarcomatous degeneration at the time of resection (1/139, 0.7%). 1 other patient developed a lesion at another site. The recurrence rate in our DFSP cohort is significantly lower than previous reports. Wide margin resection following oncologic principles can result in a very low recurrence rate. After the initial recovery phase, these patients do not require ongoing, frequent follow-up. Future studies should look at if closer margins can also produce similar treatment outcome

Aims

The present study investigated receptor activator of nuclear factor kappa-Β ligand (RANKL), osteoprotegerin (OPG), and Runt-related transcription factor 2 (RUNX2) gene expressions in giant cell tumour of bone (GCTB) patients in relationship with tumour recurrence. We also aimed to investigate the influence of CpG methylation on the transcriptional levels of RANKL and OPG.

Aims

The preoperative grading of chondrosarcomas of bone that accurately predicts surgical management is difficult for surgeons, radiologists, and pathologists. There are often discrepancies in grade between the initial biopsy and the final histology. Recent advances in the use of imaging methods have shown promise in the ability to predict the final grade. The most important clinical distinction is between grade 1 chondrosarcomas, which are amenable to curettage, and resection-grade chondrosarcomas (grade 2 and 3) which require en bloc resection. The aim of this study was to evaluate the use of a Radiological Aggressiveness Score (RAS) to predict the grade of primary chondrosarcomas in long bones and thus to guide management.

Aims

Low-grade central osteosarcoma (LGCOS), a rare type of osteosarcoma, often has misleading radiological and pathological features that overlap with those of other bone tumours, thereby complicating diagnosis and treatment. We aimed to analyze the clinical, radiological, and pathological features of patients with LGCOS, with a focus on diagnosis, treatment, and outcomes.