Purpose of the study: Nearly all published series of Ewing sarcoma present the present of bone metastasis as a factor of very poor prognosis. Reviewing our experience, we noted that the prognosis is not as bad as expected in these patients if surgical resection of all known foci can be achieved. Case reports: Case n° 1 was a 16-year-old girl who presented a Ewing sarcoma involving the left iliopubic ramus. No other foci could be identified on the plain x-rays, scintigraphy and bone computed tomography. Preopeartive magnetic resonance imaging revealed a metastatic focus in the neck of the homolateral femur. The two foci were resected after preoperative chemotherapy: resection of the left hemi-pelvis and resection of the upper potion of the femur with replacement with a pelvic prosthesis and and massive prosthesis for the proximal femur. Eight years later, the patient has remained in complete primary remission, consulting for orthopedic gait problems related to prosthetic loosening. Case n° 2 was a 13-year-old boy who presented an Ewing sarcoma of the upper tibial metaphysic. Preoperative magnetic resonance imaging revealed three other metastatic localizations in the homolateral femur. Bifocal resection of the tibia and the femur was performed with implantation of an active growth prosthesis. Chemotherapy was continued. Seven years later, the patient remains in primary complete remission. Lengthening the prosthesis has enabled equivalent growth for the two limbs. The patient has a normal life style excepting contact sports which are prohibited. Case n° 3 was a 17-year-old boy who presented a voluminous Ewing sarcoma of the right pelvis. Search for extension revealed a unique metastasis in the fourth lumbar vertebra. The patient was given preoperative chemotherapy before resection of the pelvic tumor then two months later resection of the vertebral metastasis. The patient died 4.5 years later from a traffic accident. He had remained in complete remission. Discussion and conclusion: These three cases of complete long-term primary remission of patients with primary bone metastases show that like other bone sarcomas, eradication of all recognized bone metastases is essential for the prognosis of Ewing sarcoma.
Desmoid tumour is an histological benign tumour. Nevertheless, peri-scapular relapses can decrease the function and intra thoracic progression threaten life. To prevent these complications, damaging treatment (radiotherapy, amputation) are sometimes proposed. To precise the optimal indications of treatment, we reviewed our cases. Patients: from 1984 to 2008, we treated 11 patients with peri-scapula fibromatosis (mean age 42 (13–58)). Only 4 patients were seen at first hand, 7 for relapses (3 of them after radiotherapy). Treatment was adapted to each patient, in function of age, history of illness, and risks of spontaneous evolution. En bloc extratumoral resection was performed each time, when it didn’t expose to heavy functional risk (8). The other patients were treated by contaminated resection, but never invaliding. 4 patients received pre or/and post-operative chemotherapy. 1 received Interferon alpha, and 7 tamoxifen. Results: with a median follow-up 15 years 3 months, 7 patients suffered of recurrence. No patient died from disease (thoracic complications) or therapeutic complication9 patients are in complete remission and 2 in stable disease. Following radiotherapy, local relapses (7 cases) and repeated surgery, functional sequellaes are numerous: 2 circumflex nerve palsies, 3 articular stiffness. Major functional sequellaes came from radiotherapy (limb discrepancy, lung and thorax deformity, skin and muscle atrophy. Conclusion: in this non predictable illness, therapeutic indications should individually balance risks of spontaneous evolution and of complications of treatment. Besides surgery, needed in fast all cases, but often insufficient, it must be considered the value of interferon, tamoxifen and/or chemotherapy. The most important concept is the necessity to treatment avoiding late sequellaes and particularly radiotherapy or mutilating surgery.
