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

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

The modified Glasgow Prognostic Score (mGPS) is a validated prognostic indicator in various carcinomas as demonstrated by several meta-analyses. The mGPS includes pre-operative CRP and albumin values to calculate a score from 0–2 that correlates with overall outcome. Scores of 2 are associated with a poorer outcome. Our aim was to assess if the mGPS is reliable as a prognostic indicator for soft tissue sarcoma (STS) patients. All patients with a STS diagnosed during years 2010–2014 were identified using our prospectively collected MSK oncology database. We performed a retrospective case note review examining demographics, preoperative blood results and outcomes (no recurrence, local recurrence, metastatic disease and death). 94 patients were included. 56% were female and 53% were over 50 years. 91% of tumours were high grade (Trojani 2/3) and 73% were >5cm. 45 patients had an mGPS score of 0, 16 were mGPS 1 and 33 were mGPS 2. On univariate analysis, an mGPS of 0 or 2 was statically significant with regards to outcome (p=0.012 and p=0.005 respectively). We have demonstrated that pre-treatment mGPS is an important factor in predicting oncological outcome. A score of 0 relates to an improved prognosis whilst a score of 2 relates to an increased risk of developing metastases and death. mGPS as a prognostic indicator was not affected by either the tumour size or grade. We believe that a pre-operative mGPS should be calculated to help predict oncological outcome and in turn influence management. Further work is being undertaken with a larger cohort

INTRODUCTION. Limb salvage surgery is a common treatment for patients who suffer from bone tumors. In the case of pelvic tumors this creates a challenge for the surgeon and the treatment remains controversial because the oncologic complications like local recurrence, dissemination and orthopaedic ones, like infection, haemorrhage, and mechanical problems of reconstructions Tumors affecting the acetabulum are a challenge for the surgeon because of the impact in the function of the extremity. There are many reconstruction techniques described in the literature like prosthesis, allograft systems, arthrodesis, etc…, but still there is not a gold standard due to the poor functional results at long term follow up, and the associated complications of all techniques. In this study we show the experience in our center on pelvic reconstructions after tumors affecting the acetabulum area (zone II). MATERIAL AND METHODS. We surgically treated 81 pelvic tumors from 1997 to 2009 following the Enneking and Dunham calssification attending to the localization of the tumor: Zone I 38 (iliac bone)Zone II 25 (acetabulum)Zone III 18 (pelvic branches)In zone II tumors we performed pelvic reconstruction in eight cases, with different type of prosthesis. In 5 cases we performed saddle prosthesis (group A) and in 3 cases we performed Coned-Stanmore Implants type prosthesis with sacro-iliac anchorage. The mean follow up of the serie was 3,5 years (1–6 years). In group A the mean follow up was 5 years and in group A and in group B the mean follow up was 1 year due to the recent implantation in our center of Coned type prosthesis for pelvic reconstruction. We evaluated our results with these two types of prosthesis. RESULTS. Oncologic: group A we had a local recurrence of 25% and 25% of the patients died. group B we had no cases of local or systemic recurrence and we didn't registered any death. Functional: In group A the patients showed local pain and difficulty to walk probably due to the change of the center of rotation of the hip and instability of the saddle prosthesis. In group B all patient's followed physical therapy programs without problems and were able to walk with crutches ten months after surgery. Complications:group A we had a 25% of perioperative infection and a 25% of dislocation of the prosthesis. In group B we did not have any of these complications. CONCLUSIONS. Saddle prosthesis mantain the length of the extremity and allow weight bearing but they do not give a good stability. Even if we only have one year follow up with this Coned prosthesis with sacro iliac anchorage we achieved much better functional results and a lower rate of complications

AIM. To present our experience in patients treated under primary diagnosis giant cell tumor of bone at Department Orthopaedic Surgery Zagreb University School of Medicine in a 15-year period from 1995 to 2009. METHODS. We performed a retrospective study of all patients treated in our Department because of giant cell tumor of bone (GCT) from 1995 to 2009. The mean age of our patients was 29,9 years (range: 14 to 70 years). Sex distribution showed prevalence in female (F:M=23:12=66%:34%). All together, 39 patients were operated under primary diagnosis of GCT. Four patients were lost in follow-up. In total, 35 patients were included in study. Diagnosis of GCT was made according to clinical, imaging and histological findings, and distributed by Campanacci's classification. RESULTS. Not including diagnostic biopsy, 84 operations were performed on 35 patients. Fourteen patients (40%) had GCT grade 1, fourteen (40%) had GCT grade 2, and seven (20%) had GCT grade 3. From the first symptoms to diagnosis there was an average duration of 7 months (range: 0 to 24 months), where the main symptoms were pain and swelling of affected bone and/or joint. GCT was localized in distal femur (n=12, 34%), proximal tibia (n=10, 29%), distal tibia (n=4, 11%), distal radius (n=3, 9%), and other locations (n=6, 17%). Patients with less aggressive GCT (grades 1 and 2) were treated with marginal excision: excochleation and reconstruction with bone transplant (n=12, 34%). In patients with locally more aggressive tumor (grades 2 and 3), “en bloc” resection and reconstruction with tumor endoprosthesis or bone transplant was performed (n=22, 63%). Due to localization of tumor, one patient was treated with radiation (3%). Complications were recorded in 12 patients (34%), and are shown as total number and percentage of all complications. Complications were the most common in knee region, proximal tibia (n=4, 33%) and distal femur (n=3, 25%). Also, the complications occured more frequently after “en bloc” resection (n=7, 58%). GCT classified as gradus 2 had most complications (n=5, 42%) till GCT classified as gradus 3 had least (n=3, 25% of complications, 9% of all). We recorded and treated local recurrence of tumor (n=6, 50%), infection (n=2, 17%), and mehanical complications of endoprosthesis (n=2, 17%). Due to local recurrences, in 2 patients underlying osteosarcoma was revealed, and they were treated with amputation. CONCLUSION. Each patient with GCT should be treated individually. Regardless non-malignant attribute, local behaviour of tumor determines treatment approach according to treatment principles of malignant tumor of bone. Number of complications in our patients is relatively high, recorded in one third of our patients, which matches the literature in announced studies

Endoprosthetic replacement of the proximal femur may be required to treat primary bone tumours or destructive metastases either with impending or established pathological fracture. Modular prostheses are available off the shelf and can be adapted to most reconstructive situations for this purpose. We have assessed the clinical and functional outcome of using the METS (Stanmore Implants Worldwide) modular tumour prosthesis to reconstruct the proximal femur in 100 consecutive patients between 2001 and 2006. We compared the results with the published series for patients managed with modular and custom-made endoprosthetic replacements for the same conditions. There were 52 males and 48 females with a mean age of 56.3 years (16 to 84) and a mean follow-up of 24.6 months (0 to 60). In 65 patients the procedure was undertaken for metastases, in 25 for a primary bone tumour, and in ten for other malignant conditions. A total of 46 patients presented with a pathological fracture, and 19 presented with failed fixation of a previous pathological fracture. The overall patient survival was 63.6% at one year and 23.1% at five years, and was significantly better for patients with a primary bone tumour than for those with metastatic tumour (82.3% vs 53.3%, respectively at one year (p = 0.003)). There were six early dislocations of which five could be treated by closed reduction. No patient needed revision surgery for dislocation. Revision surgery was required by six (6%) patients, five for pain caused by acetabular wear and one for tumour progression. Amputation was needed in four patients for local recurrence or infection. The estimated five-year implant survival with revision as the endpoint was 90.7%. The mean Toronto Extremity Salvage score was 61% (51% to 95%). The implant survival and complications resulting from the use of the modular system were comparable to the published series of both custom-made and other modular proximal femoral implants. We conclude that at intermediate follow-up the modular tumour prosthesis for proximal femur replacement provides versatility, a low incidence of implant-related complications and acceptable function for patients with metastatic tumours, pathological fractures and failed fixation of the proximal femur. It also functions as well as a custom-made endoprosthetic replacement

A systematic literature review of distraction osteogenesis (DO) for the primary reconstruction of bone defects following resection of primary malignant tumours of long bones (PMTLB) is presented. Fewer than 50 cases were identified. Most reports relate to benign tumours or secondary reconstructive procedures. The outcomes of our own series of 7 patients is also presented (4 tibiae, 3 femora). All patients had isolated bone lesions without metastases and were assessed through the hospital sarcoma board. Mean follow-up was 59 months (17–144). Mean age was 42 years. Final histologic diagnoses were 3 chondrosarcoma, 2 malignant fibrous histiocytoma, 1 adamantinoma and 1 malignant intraosseous nerve sheath tumour. Mean bone defect after resection was 13.1cm (10–17) and bone transport was the reconstruction method in all. There was one local recurrence of tumour six months post-resection, necessitating amputation. Mean frame index for remaining cases was 30.9 days/cm (15.7–41.6). Complications included pin infection, docking site non-union, premature corticotomy union, soft-tissue infection and minor varus deformity. Six cases remain tumour-free with united, well-aligned bones and good long-term function. We conclude DO provides an effective biologic reconstruction option in select cases of PMTLB

Introduction. Although various reports analyzed “en-bloc” excision of sacral tumors, there are still technical problems to improve protection of nerve roots, preserve surrounding structures and reduce intraoperative bleeding, maintaining the oncologic result. We present a new technique for sacral resection, with short term preliminary results, derived with modification from Osaka technique. Methods. Seven patients were resected for their sacrococcygeal chordoma with the followed described technique. Two patients had previous surgery elsewhere. The sacrum is exposed by a posterior midline incision and complete soft-tissue dissection. Lateral osteotomies were performed through the sacral foramina using a threadwire saw and Kerrison rongeurs, to avoid sacral roots damage. After proximal osteotomy, the sacrum is laterally elevated and mobilized to allow dissection of presacral structures. Mean surgical time was 5 hours (range: 3 to 8). Mean blood loss was 3640 ml. Results. Level of resection was S1 in 2 pts, S2 in 4 pts, S3 in 1. Margins were wide in 6 patients and marginal in one. At a mean follow-up of 2 years, six patients were disease-free, one had a local recurrence. No complications were showed. Conclusion. This technique allows wide margins with roots preservation and reduction of complications and operative time. Indications for posterior approach only can be extended to resection proximal to S3, when there is minimal pelvic invasion and none or partial involvement of sacroiliac joints. However, the long term benefits of this technique need to be evaluated

Introduction. Telangiectatic osteosarcoma (TOS) is a rare subtype of osteosarcoma. We review our experience to characterize its prevalence, treatment, relapse and survivorship at long term follow-up. Methods. Eighty-seven patients aged from 4 to 60 years (mean 20 years), were treated from 1985 to 2008. Lesions affected the femur (38), humerus (20), tibia (19), fibula (4), pelvis (3), foot (2) and radius (1). Eight patients had metastatic disease at diagnosis. Seventy-eight patients were treated with neoadjuvant chemotherapy with three or more drugs according to different protocols, nine had surgery as first treatment. Limb salvage surgery was performed in 71 cases, amputation in 14 and rotationplasty in one. One patient died before surgery. Prognostic factors were evaluated with Kaplan-Meier analysis. Results. At a mean follow-up of 8 years, overall survival was 81%, 65% and 65% at 2, 5 and 10 years respectively. Fifty-two patients were disease-free, three were alive with disease, twenty-nine died with disease and three died of other causes. Thirteen local recurrences were observed. Twenty-three patients developed lung (20) or bone (3) metastases. Pathologic fracture did not significantly influence survivorship. Prognostic influence of age of the patients was evaluated at three different cut-off (15, 20 and 25 years-old): younger patients had better survivorship, without statistical significance. Induced necrosis according to Huvos’ classification was significant at both univariate and multivariate regression Cox analysis (p=0.0001). Conclusion. TOS does not have a poor prognosis as previously reported in the literature. A high percentage of patients can be cured with neoadjuvant chemotherapy and surgery. In most patients, limb sparing surgery is possible and safe

Purpose. To investigate the prognostic effect of surgical margins in soft tissue sarcoma on Local Recurrence (LRFS), Metastasis (MFS) and Disease Free Survival (DFS). Patients and Methods. This is a retrospective, single center study of 105 consecutive patients operated with curative intent. Quality of surgery was rated according to the International Union Against Cancer classification (R0/R1) and a modification of this classification (R0M/R1M) to take into account growth pattern and skip metastases in margins less than 1mm. Univariate and multivariate analysis was done to identify potential risk factors. Kaplan-Mayer estimated cumulative incidence for LRFS, MFS and DFS were calculated. Survival curves were compared using Log rank tests. Results. Estimated LRFS was 0.64 [0.52;0.76] at 5 years following R1 surgery, 0.9 [0.85;0.95] following R0 (p=0.023), 0.64 [0.519;0.751] following R1M surgery and 0.92 [0.87;0.96] following R0M (p=0.01). The R status was associated with DFS (p=0.028), but not MFS (p=0.156). The RM status was associated with both outcomes (p=0.001 and p=0.007). Multivariate analysis showed an independent association with LRFS for RM status (HR 6.77 [1.78–25.7], p=0.005), with DFS for RM status (HR 2.83 [1.47–5.43], p=0.001) and Grade (HR=3.17 [1.38–7.27], p=0.003) and with MFS for Grade (HR=3.96 [1.50–10.5], p=0.006). Conclusions. In Soft Tissue Sarcoma, surgical margins are the strongest prognostic factor for LRFS. In this study, taking growth pattern and skip metastases into account for margins less than 1mm increased prognostic significance of surgical margins for LRFS, DFS and MFS

Background. Modular endoprostheses today represent a standard treatment option in the management of musculoskeletal tumors of the lower extremities. Long-term results of these reconstructions, however, are often limited by the course of the underlying disease. We therefore report our experiences in cancer patients with megaprostheses of the lower limb after a minimum of 15 years. Materials and Methods. 62 patients, 34 men and 28 women, with a mean age of 26 years (median, 20; range, 6–83) were included in this investigation with a mean follow-up of 230 months (median, 228; range, 180–342). Endoprosthetic reconstructions of the proximal femur (11), the distal femur (28), the total femur (2) or the proximal tibia (21) were indicated for osteosarcoma (43), chondrosarcoma (5), malignant fibrous histiocytoma (3) or other tumors (11). All patients have received either a KMFTR (22) or a HMRS (40) modular prosthesis; 23 patients had a muscle flap, 14 had a fibular transposition osteotomy and 4 have received an artificial LARS ligament for soft-tissue reconstruction. Results. 7 patients (11.3%) died throughout the follow-up period, but none succumbed to primary disease. One patient (1.6%) developed a local recurrence after 31 months that was resected. Overall, 56 patients (90.3%) underwent revision of their prosthesis; 50 (80.7%) had multiple revisions up to a maximum of 12 operations (mean, 3 per patient). The median overall prosthetic survival to first revision was 40 months; the corresponding 5-, 10- and 15-year survival rates were 35.5%, 14.5% and 12.9%, respectively. 3 patients (5.4%) had an infection, 8 (14.3%) had a soft-tissue related failure, 30 (53.6%) had a mechanical or structural failure and 15 (26.8%) had an aseptic loosening. The 15-year survival rates of these respective endpoints were 87.1% for infection, 79.0% for soft-tissue related failure, 32.3% for mechanical or structural failure and 56.5% for aseptic loosening. 59 patients (95.2%) have retained their prosthesis; 2 patients (3.2%) underwent secondary amputation due to an irresolvable complication, another one (1.6%) for a second malignancy. Conclusion. Modular prosthetic reconstructions of the lower extremities have a high revision rate in the long-term, primarily due to mechanical failures. Given that patients survive their malignant disease the rate of secondary implant removal, however, tends to be low, providing satisfactory function and body integrity. Further advances in implant design, soft tissue management and infection prophylaxis are required to reduce revision rates

Sacral tumours are rare and can present difficult diagnostic and therapeutic challenges even at an early diagnosis. Surgical resection margins have a reported prognostic role in local recurrence and improved survival. Successful management is achieved within a specialist multidisciplinary service and involves combination chemotherapy, radiotherapy and surgery. We present our experience of patients with sacral tumours referred to our unit, who underwent total and subtotal sacrectomy procedures. Materials and Methods. Between 1995 and 2010, we identified twenty-six patients who underwent a total or subtotal sacrectomy operation. Patients were referred from around the United Kingdom to our services. We reviewed all case notes, operative records, radiological investigations and histopathology, resection margins, post operative complications, functional outcomes and we recorded long-term survival outcomes. Patients who were discharged to local services for continued follow up or further oncological treatment were identified and information was obtained from their general practitioner or oncologist. We reviewed the literature available on total sacrectomy case series, functional outcomes and soft tissue reconstruction. Results. We reviewed 26 patients, 16 male and 10 female, with a mean age at presentation of 53.4 years (range 11–80 years). Duration of symptoms ranged from 2 weeks to 6 years; lower back pain and sciatica were amongst the most common presenting features. Histological diagnoses included chordoma, Ewing's, malignant peripheral nerve sheath tumour, chondromyxoid fibroma, spindle cell sarcoma, synovial sarcoma, chondrosarcoma. A combined approach was used in two-thirds of patients and most of these patients had a soft tissue reconstruction with pedicled vertical rectus myocutaneous flap. Complications were categorised into major and minor and subdivided into wound, bladder and bowel symptoms. Wound complications and need for further intervention were more common amongst the patient group who did not have simultaneous soft tissue reconstruction at operation. All patients had a degree of bladder dysfunction in the early postoperative period. We present survivorship curves including recurrence and development of metastases. Conclusion. Total sacrectomy procedures carry a high risk of associated morbidity but can improve survival amongst specific groups of patients. They present challenges in diagnosis and management, but must be referred to a specialist service, that will instigate appropriate investigations and treatment regimes within a multidisciplinary setting. The expansion of services from other specialties required for the postoperative and ongoing rehabilitation plays an important role in overall management and appropriate pathways to coordinate these services are necessary

Introduction. Primary malign tumors and solitary metastatic lesions of the thoracic and thoracolumbar spine are indications for radical en bloc resections. Extracompartimental tumor infiltration makes the achievement of adequate oncological resection more difficult and requires an extension of the resection margins. We present a retrospective clinical study of patients that underwent chest wall resection in combination with vertebrectomy due to sarcomas and solitary metastases for assessing the clinical outcome especially focusing on onco-surgical results. Method. From 01/2002 to 01/2009 20 patients (female/male: 8/12; mean age: 52 (range of age: 27–76yrs)) underwent a combined en bloc resection of chest wall and vertebrectomy for solitary primary spinal sarcoma and metastatic lesions. The median follow-up was 20,5 (3–80) months. Histological analysis revealed 17 primary tumors and 3 solitary metastatic lesions. In the group of primary tumors 10 sarcomas, 1 giant cell tumor, 2 PNET, 1 histiocytoma, 1 aggressiv fibrous dysplasia, 1 pancoast tumor and 1 plasmocytoma were histologically documented. We included 1 rectal carcinoma, 1 breast cancer metastases and 1 renal cell carcinoma. All patients underwent a chestwall resection en bloc with multilevel (1/2/3/4 segments: n=4/6/6/4) hemi (n=7) or total vertebrectomy (n=13) with subsequent defect reconstruction. Reconstruction of the spinal defect following total resections was accomplished by combined dorsal stabilization and carbon cage interposition. The chest wall defects were closed with a goretex ® -patch. One patient also received a musculocutaneus latissimus dorsi flap. Results. The surgical margins were R0 in 19 (wide in 14, marginal in 5) and one R1 resection. Marginal/R1 resections were due to extracompartimental sarcoma invasion (spinal canal) and dural involvement. In these patients postoperative radiotherapy was performed. Surgical complications requiring revision occurred in 1 patient due to injury of the ductus thoracicus and persisting chylothorax. Temporary subileus or mild pneumonia appeared in 3 patients. No superficial/deep infection or neurological deficits (except those related to oncologically required dissection of thoracic nerve roots) were observed. At follow up 2 patients died due to the disease after 7,5 months. Local recurrences were seen in 3 patients at median 24 months (13–43). Pulmonary metastases necessitating polychemotherapy were seen in 7 patients after median 17 months (7–44). Conclusion. Despite the only midterm follow up, the combined en bloc resection of chest wall and multilevel en bloc spondylectomy/hemivertebrectomy is a challenging but safe and effective technique in order to achieve adequate margins and local control in selected with spinal sarcomas extending to the dorsolateral chest wall

Background. Extendable proximal femoral replacements(PFR) are used in children with bone tumours in proximity to the proximal femoral physis, previously treated by hip disarticulation. Long-axis growth is preserved, allowing limb salvage. Since 1986, survival outcomes after limb salvage and amputation have been known to be equal. Method. Retrospective review of all patients <16years undergoing extendable PFR at Royal National Orthopaedic Hospital (UK) between 04/1996 and 01/2006, recording complications, failures, procedures undertaken and patient outcomes. Results. 8 patients (mean age 8.9±3 years) underwent extendable PFR for Ewing's Sarcoma(5), Osteosarcoma(1), Chondrosarcoma(1) and rhabdomyosarcoma(1). 2 primary PFRs failed (infection of unknown source & local recurrence, both at 26months); 2 required revision for full extension (1 became infected at revision, requiring 2 stage revision). 3 patients had the original prosthesis in situ at last follow-up (mean 7.2;range 3–10.5years). 1 patient had no implant complications, but died (neutropaenic sepsis) 63 days after implant insertion. 2 were treated for recurrence but disease free at last review. 5 were continuously disease free. 5 patients were lengthened a mean 3.7cm; 2 were not lengthened.1 had incomplete data. 5 patients suffered subluxation/dislocation (mean 15.6months), 3 recurrently. Each underwent a mean 1.6 open & 1.4 closed procedures for the displaced joint. 3 patients had 4 open reductions and acetabuloplasties and 2 patients were converted to THR, with 3 major complications: 2 sciatic nerve palsies and 1 (THR) infection. The 5th patient was due for acetabuloplasty but had hip disarticulation for recurrence. Acetabular erosion occurred in 3; 2 were revised to THR (3.5 & 6.8years). 3 patients suffered peri-prosthetic supracondylar fracture (treated conservatively). 5 patients were revised to THR (mean 5.9years): 2 for dislocation, 2 for acetabular erosion & 1 for infection. 1 underwent amputation and another died. Only 1 surviving implant was not converted to THR: this patient had progressive acetabular erosion at 10.5 years & will eventually require THR. The amputee had poor hip function prior to disarticulation but went on to become an international Paralympic sportsman and had very good function 11.4 years post-disarticulation. 3 patients had fixed hip adduction deformity. 1 was isolated and treated with adductor tenotomy, whilst 2 were associated with knee flexion deformity (one required in-patient physiotherapy; the other prosthetic shortening). Conclusions. Extendable PFR permits limb salvage with psychological & functional benefits, but complications are common and some are specific to PFR. Surgery for these may result in further complications. Patients should be warned of the high conversion rate to THR. All the above should be borne in mind when selecting patients. As illustrated above, functional outcome is sometimes better with amputation