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

Metastatic osteosarcoma is seen in 10-20% of patients at initial presentation with the lung the most common site of metastasis. Historically, prognosis has been poor. We studied trends in survival in our small developed nation and aimed to identify correlations between the survival rate and three factors: newer chemotherapy, advances in radiological imaging and a more aggressive approach adopted by cardiothoracic surgeons for lung metastases. Our national bone tumour registry was used to identify patients at the age of 18 or under, who presented with metastatic disease at initial diagnosis between 1933 and 2006. There were 30 patients identified. Kaplan-Meier analysis was used to determine survival rates and univariate analysis was performed using the Cox regression proportional hazards model. Median survival has improved over the last 50 years; highlighted by the ‘Kotz’ eras demonstrating incremental improvement with more effective chemotherapy agents (p=0.004), and a current 5-year survival of 16%. Aggressive primary and metastatic surgery also show improving trends in survival. Three patients have survived beyond five years. The introduction of computerised tomography scanning has led to an increase in the prevalence of metastases at initial diagnosis. Metastatic osteosarcoma remains with a very poor prognostic factor, however, aggressive management has been shown to prolong survival

Introduction. Dedifferentiated chondrosarcoma (D.C.) has a very poor prognosis. The efficacy of chemotherapy is still debated. Aim of this study was to evaluate the survival of patients with D.C. and to evaluate possible prognostic factors. Methods. Between 1990 and 2006, 109 patients were treated for D.C.: 55 males and 54 females, mean age of 59.6 years. In 81 cases tumor was located in the extremities and in 28 cases in the trunk. The most frequent dedifferentiation was in osteosarcoma (53.2%) followed by spindle cell sarcoma (21%), malignant fibrous histiocytoma (13.8%), fibrosarcoma (6.4%). All patients received surgery and mostly, limb salvage with tumor resection and implantation of a megaprosthesis or allograft (65 patients). Chemotherapy was given to 43 patients. Results. 16 patients (15.5%) were Ned at a mean followup of 10 yrs, 8 patients Ned1 after treatment of relapse, 1 patient AWD at 4.7 yrs, 77 patients DWD at a mean time of 1.6 yrs, 1 patient dead for other causes and 6 patients lost to followup were excluded. The overall survival of patients was 15%. There was significant difference in survival between patientens with D.C. of the trunk and those with D.C. of the extremities (p=0.0156). There was no significant difference in survival with chemoterapy and surgery or with surgery only (p=0.1115). Conclusion. The prognosis for patients with D.C. remains dismal. Surgery with wide margins is the principal treatment for this condition. There was no statistical evidence of any beneficial effect from chemotherapy

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

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.