Preoperative screening of patients included standard X rays, CT and bone technetium scan in all cases and MRI in 15 cases. Diagnosis was made by open biopsy except for 4 cases of chondrosarcoma for these preoperative screening was sufficient (and diagnosis confirmed by postoperative histological examination). Following limb salvage using reconstruction of pelvis was performed with methyl metacrylate without prosthesis Titanium screws were inserted in remaining bone before moulding of acrylic cement (2 to 3 packs of antibiotic loaded cement).
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.
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.
Congenital fibrosarcoma (CFS) is a rare tumor most often affecting extremities of babies. Considering age, surgery of primary is preferred. Nevertheless amputation rate remains high. Preoperative chemotherapy (CT) role must be emphasised. We present 3 cases receiving preoperative CT. Patients and methods in 1985, we treated a 3 months old girl for CFS of the thigh. To avoid amputation, preoperative CT (3 Ifosfamide- Vincristine- Actinomycine D) was performed leading to complete radiological and histological response. She benefited of conservative surgery She is in first complete remission 23 years later. In September 1999, a 3 ½ y old boy with recurrent l buttock CFS operated elsewhere twice (6 months old, 2 years old), received preoperative chemotherapy with good clinical and radiological response. “En-bloc” extra tumoral resection was performed. Histology showed viable tumoral cells. We completed treatment by chemotherapy. In 01/ 2003 bilateral pulmonary metastases occurred leading to surgery and chemotherapy. In 09/ 2003 a new local recurrence appeared treated by surgery and postoperative chemotherapy. From this time, he received Alpha interferon. He is in complete remission for 6 years. In 12/2005, a 14 y old girl, with local recurrence of CFS, treated elsewhere at the age of 5 months by partial surgery and chemotherapy (remained in remission for 13 years)was admitted. Since this time, she recurred locally despite resections and multiple lines of chemotherapy, but without metastasising. She was amputated in 2008.
The stiffness of the shoulder can result of many illness. Nevertheless, we observed a severe stiffness of the scapulo thoracic space only in fibromatosis. To precise the real diagnostic value of this symptom, we examined patients with different diseases of shoulder (tumoral and non tumoral). The passive mobility of the shoulder of 11 patients with peri-scapular fibromatosis was compared to the mobility of those in 50 patients with non tumoral diseases of shoulder (arthritis and rotator cuff pathology), 50 peri-scapular soft tissues tumours, and 100 patients with primitive or secondary malignancies of humerus or scapula.
After treatment of fibromatosis, 9/11 patients are in complete remission and the mobility of their scapulo thoracic space restored. 2 patients are in stable disease and one suffers of a residual stiffness of the scapulothoracic space. We conclude that the frozen scapulo thoracic is a specific symptom of peri scapulo thoracic fibromatosis. The restoration of the mobility of the scapulo thoracic after cure of the desmoid tumour confirms its specific role and represents a good marker of the tumoral evolution.
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.