Purpose: To determine the mode of presentation of soft tissue sarcomas to a tertiary centre and the factors associated with a delay in diagnosis and referral.

Methods and Results Between 2000 and 2002, we identified 120 patients with primary soft tissue sarcomas histologically verified at our institution. We reviewed the case histories, referral letters and the histology reports in all cases. The mean age of the study group was 54.5 (range 10 to 91 years). Pathological diagnoses included 32 liposarcomas, 20 malignant fibrous histiocytomas, 19 synovial sarcomas, 12 leiomyosarcomas, 9 fibrosarcomas, 7 spindle cell sarcomas, 4 pleomorphic sarcomas, 3 identified only as high grade soft tissue sarcomas, 3 epithelioid sarcomas, 2 extraosseous Ewing’s sarcomas, 2 malignant round cell tumours and one each of neurofibroma, malignant peripheral nerve sheath tumour, angiosarcoma, alveolar cell sarcoma, extraosseous chondrosarcoma, extraosseous osteosarcoma and clear cell sarcoma of tendon sheath.

Presenting features included a discrete lump or diffuse swelling in 115 patients (95.8%). Pain was not a feature in 69 patients. 3 patients (2.5%) presented with pain alone and 2 patients (1.7%) with varicosities. 116 patients (96.7%) were referred to our unit as a possible soft tissue sarcoma. 4 patients were referred as suspected benign lesions requiring specialist assessment owing to size and location. 46 patients (38.3%) ignored their swelling, resulting in a delay to presentation to their local units. In only 13 patients was pain a feature. These were not considered to be true delayed referrals. 24 patients (20%) experienced a delayed referral to our unit. An alternative diagnosis was suggested in 7 patients by preliminary imaging investigations. A ‘Whoops procedure’ was performed in 14 patients. 3 patients were initially misdiagnosed as muscular injuries, with consequent conservative management resulting in delay.

Conclusion: Soft tissue sarcomas are relatively rare when considered alongside benign soft-tissue lesions. Pain is an infrequent feature, which may result in patients undervaluing their significance and delay their initial presentation to a doctor. Delayed referral is relatively common, although may not always be avoidable. Clinicians should maintain a high level of suspicion when dealing with soft-tissue lesions, with referral to a specialist centre whenever concern exists as to the nature of the lesion.

Purpose: The purpose of this study was to evaluate the results of custom-made endoprosthetic reconstruction for both tumourous and non-tumourous conditions around the elbow joint.

Methods and Results: 28 consecutive cases of endoprosthetic elbow reconstruction, performed between 1989 and 2003, were identified using the unit database. There were 12 males and 16 females, with a mean age of 48.9 years, (range 14–84). There were 16 cases of malignant tumour (10 primary, 6 secondary), 3 cases of benign tumour (one each of giant cell tumour, osteoblastoma and pigmented villonodular synovitis) and 10 non-oncological cases, (5 cases of fracture, 3 failed total elbow replacements and 1 infected synostosis). A distal humeral prosthesis was used in 16 patients, distal humeral and proximal ulna in 10 patients, proximal ulna with a humeral component in one patient and proximal radial replacement in one patient. Clinical and radiographic review of all available patients, including a functional assessment with The Toronto Extremity Salvage (TES) score was undertaken. The average follow-up was 54.9 months (range 5–144). 5 patients with malignancy died of their disease. One patient required amputation for local recurrence at 5 months. There were two revisions at 4 and 7 years for aseptic loosening of the humeral component. Six patients developed deep prosthetic infection at an average of 21 months (range 6–31), necessitating two-stage revision, of which one patient required subsequent excision arthroplasty. There were no cases of permanent nerve palsy. 18 patients (64.3%) were available for functional follow-up. Average flexion deformity was 33 degrees (7–80) and average flexion was 85 degrees (30–133). The mean TES score was 67.8 (range 36.6–96.6). The mean TES score following two-stage revision for infection, (available in 4/5 patients) was 73.9 (range 54.8–91.6).

Conclusion: Custom-made endoprosthetic reconstruction around the elbow joint is effective in a wide range of pathologies, allowing in most cases a reasonable level of function, even following two-stage revision for infection.

Purpose: Dedifferentiated chordomas are a rare and aggressive variant of chordoma. They usually occur in recurrences or following radiotherapy. We describe the rare occurrence of three cases arising de novo within conventional chordoma.

Methods and Results: 29 cases of histopathologically verified chordoma were identified from our unit database. From these, we identified three cases of primary dedifferentiated chordoma. The study group included three men, with an average age of 58.3 years (range 57–61). Presenting features were comparable and included lower back pain and rectal discomfort, with a mass palpable per rectum. A needle biopsy was undertaken in one patient, which demonstrated evidence of a pleomorphic spindle cell tumour without evidence of chordoma. Surgical management involved subtotal sacrectomy, with complete excision achieved in two cases. Histopathological examination confirmed dedifferentiated chordoma with varying amounts of sarcomatous elements in each case. Post-operative radiotherapy was administered in two patients and chemotherapy in one patient. Two patients died at 7 and 10 months following presentation with pulmonary metastases. The third patient remains well and alive at two years follow-up. This patient had a complete excision with post-operative radiotherapy, however histopathological examination revealed only a small focus of the dedifferentiated component.

Conclusions: Dedifferentiated chordoma is a fatal tumour, with metastases and death the likely outcome. Most cases in the literature and the two cases reported in this series, uphold this ominous prognosis. However this may not always hold true. Small areas of dedifferentiation within the chordoma tumour may carry a more favourable prognosis.