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

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

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

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.