Introduction: Location on iliac bone account for 20% to 30% of sarcomas. Gold standard of local treatment is wide resection but till now few papers tried to evaluate the long term results of reconstructive procedures when chemotherapy and/or radiotherapy are used.

Patients: 44 patients (25 males and 19 females aged 9 to 66 years) with bone sarcoma of innominate bone in Zone 1, 2 or 4 (without involvement of acetabulum) were treated and/or followed up by the same team in 23 years. Histology was: chondrosarcoma (28), Ewing (13), osteosarcoma (2), MH (1)

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).

Results: With a median follow-up of 15 years (minimal 2- maximal 22). 11 patients died from disease after local recurrence (6) and/or metastases (7). One disease free survivor has been lost for follow after 3 years,1 patient is alive with disease. The 31 others are disease free survivors.

Prognostic value: in our patients the prognosis was directly correlated with the histological grading (low grade chondrosarcoma have a 85% DFS) and for high grade tumours with the efficacy of the chemotherapy protocol. For primary metastatic patients, when chemotherapy is suboptimal or margins contaminated, the prognosis is dismal. With our most effective protocols and free margins, metastatic lesions did not affect the disease free survival of our patients.

Orthopaedic results: weight bearing was immediate in all cases. We observed 3 deep infections (2 compelled to make resection of the cement) and 2 late mobilisations of cement. In all other patients, the reconstructive procedure gave a good and stable functional result even in very long follow up.

Conclusion: Acrylic reconstruction is an easy and reliable reconstructive procedure after en bloc resection of iliac bone for malignant tumours in zone 1, 2 or 4. It is more reliable than bone graft when chemotherapy or radiotherapy are necessary.

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.

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.

Conclusion: preoperative chemotherapy is feasible despite low age of the patients, can allow conservative surgery and avoid late metastases.

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.

Results: in 10 of 11 patients with peri-scapular fibromatosis, the passive mobility of the scapulo thoracic spacewas severely impaired (less than 20°). In non tumoral pathology of shoulder, the passive mobility of the shoulder is frequently impaired but the stiffness hangs only on scapulo humeral articulation. In metastases, sarcoma and soft tissue tumour (except fibromatosis) the passive mobility of the scapulo-humeral joint is usually preserved and the mobility of the scapulothoracic space is always normal even in very huge tumours.

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.

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.