Aim and purpose

The clinical management of osteosarcoma differs significantly from that of chondrosarcoma;

Therefore it is extremely important to diagnose these two types of bone tumour accurately. In the absence of a specific marker, differential diagnosis by histochemistry is sometimes impossible, especially between chondroblastic osteosarcoma and conventional chondrosarcoma. The aim of the study was to find an useful diagnostic marker, simple to use for distinguishes chondroblastic osteosarcoma from conventional chondrosarcoma.

Purpose: The prognosis is dramatic in patients with malignant Schwannoma. These tumours appear to progress invariably irrespective of the complementary treatment given. In order to determine whether this clinical impression is founded, we reviewed retrospectively patients cared for conjointly by the Cochin Hospital and the Curie Institute since 1956.

Material and methods: This series included 32 patients (17 men and 15 women) including seven who had a Recklinghausen neurofibromatosis (three men and four women). The logrank test was used to compare the actuarial survival curves and determine prognostic factors and potential benefit of chemotherapy and radiotherapy protocols.

Results: Mean age at diagnosis was 42.1 years (11.8–78.9). Ten percent of the schwannomas were grade I, 25% grade II and 65% grade III. All patients except two underwent surgery. The resection was considered wide in three cases, marginal in 17, contaminated in seven and non-classifiable in three. There were 14 cases of local recurrence within a mean delay of 12 months (1–66 months). Twelve patients developed secondary local relapse within a mean delay of 36 months (0–169 months). Different neoadjuvant or adjuvant treatments were administered, including chemotherapy and radiotherapy. Twenty-four patients died with a median survival of 25 months. The survival curves showed that complementary treatments did not significantly improve survival. The only factor with prognostic value was the histological grade and the quality of the resection.

Discussion: Our findings are in general agreement with data in the literature demonstrating the lack of efficacy of complementary treatments in patients with malignant schwannomas. Inversely Recklinghausen disease did not appear to modify prognosis in our patients.

Purpose: This retrospective analysis was based on observations in 15 patients, seven men and eight women, mean age 48 years (19–72) treated between 1988 and 2000 at the Curie Institute. The tumour was located in limbs in eight patients (one humerus, two femurs, four tibias, one fibula), in the axial skeleton in five (four pelvi, one sacrum), and in the rib cage and the scapula in one each. Histology examination of the dedifferentiated component displayed fibrosacroma in six cases, HFM in two, rhabdomyosarcoma in two and leiomyosarcoma and osteosarcoma in one each. Six patients were given neoadjuvant and adjuvant chemotherapy of the osteosarcoma type and underwent conservative surgery of the affected limb in three out of four cases. Total histological necrosis was observed in three out of six cases. Six patients were given adjuvant treatment alone using an osteosarcoma protocol. Three unoperable patients were given palliative chemotherapy and radiotherapy.

Results: Nine patients died from their disease. The most frequent metastatic site was the lung; mean survival was 20 months. Six patients survived including five with no progression (1+, 5+, 6+, 7+, 12+ years). Three out of five had had preoperative chemotherapy with a complete histological response and two out of five had had osteosarcoma protocol adjuvant chemotherapy.

Conclusion: Dedifferentiated chondrosarcoma is generally considered to have very poor prognosis and should lead to an adapted therapeutic strategy. In our series, the osteosarcoma protocol provided complete histological response in three out of six patients. Five patients had prolonged survival, all had been given an osteosarcoma type chemotherapy protocol.

Purpose: We report oncological and functional outcome after ten pelvis reconstructions using the Puget technique.

Material and methods: Ten patients (six men and four women), mean age 50 years (37–71) with malignant bone tumours, generally a chondrosarcoma, were included in this series. The tumour involved zone II in five patients, zones II and III in the other five. Resection was followed by reconstruction using the superior portion of the homolateral femur and a cemented total hip arthroplasty. Mean resection was 13 cm (7–23). Mean operative time was 386 min and mean blood loss was 5490 ml. The resection was wide in seven patients, marginal in two and resection margins were contaminated in one. The patients were reviewed at three, six and twelve months then each year. The function score (Enneking) was recorded for all patients. Living patients also filled out a TESS quality of life questionnaire.

Results: At mean follow-up of 22 months (7–42), four patients were living and disease free and two patients were living with recurrent disease. Three patients had died from their disease and one from pulmonary embolism. Postoperative complications were: one dislocation, one phlebitis, one reflex dystrophy, one injury to the internal genital nerve, two infections and one necrosis of the scar tissue. Bone healing was obtained at a mean five months. The mean Enneking function score was 68% and the mean TESS score was 75%.

Discussion: The high proportion of deaths is related to the severe prognosis of these pelvic tumours. This reconstruction technique described by Puget, provides an interesting alternative to other reconstruction methods for the acetabular region. The autogenous graft combined with a standard total hip arthroplasty makes this a rather easy to perform and low-cost procedure.

Conclusion: Functional outcome has been, in our experience, better than with arthrodesis, acetabular prosthesis, or saddle prosthesis and massive allograft of the pelvis.

Purpose of the study: Chordoma is a malignant neoplasm believed to arise from notochord remnants. It accounts for approximately 3 to 4 p. 100 of primary bone tumors and is localized along the axial skeleton, 50 p. 100 being sacrococcygeal. Clinical, radiographical and histological findings have been well established since the first description by Ribbert in 1894. Sacral chordomas are however difficult to manage and remain a challenge for surgeons and radiotherapists alike. The purpose of this study was to evaluate the long-term results of surgical treatment and patterns of failure in patients treated for chordoma of the sacrum in our department.

Materials and methods: This retrospective study included 11 cases of sacral chordomas treated from 1973 to 1998. Patient age ranged from 36 to 77 years (mean 59 years). Six patients were female and five male. The initial treatment was surgery in all cases including intralesional removal in two cases, marginal resection in seven and complete en bloc resection in two.

Results: Median follow-up was 6 years (1 month to 14 years). Tumoral recurrences were observed in nine cases 5 months to 8 years after treatment. In two cases, recurrence was observed 8 years after radical sacrectomy. Treatment of recurrences was partial surgical removal with radiotherapy (40 to 70 Grays). Three patients developed metastases in lungs, liver and bone, respectively. Seven patients died, two from metastatic disease. The 5-year overall survival was 64 p. 100 but only 18 p. 100 of the patients survived 10 years. Average disease-free survival was 18 p. 100 at 5 years and 0 p. 100 at 10 years.

Discussion: Chordoma is a slow-growing tumor allowing survival for several years despite recurrent disease. However, only 10 to 20 p. 100 of the patients survive free of disease at 5 years. Recurrences are frequent (45 to 80 p. 100) and often multiple. Chordoma inevitably recurs and eventually leads to death after intralesional removal or marginal resection. Radical surgery should be attempted whenever technically feasible. When performed early, particularly for smaller lesions, it offers the best chance for cure. However, tumoral recurrence can occur postoperatively despite a macroscopically complete resection. Because radiation therapy seems to be more successful in controlling microscopic disease, it should be considered as a pre- or postoperative adjuvant to a macroscopically complete resection.