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.