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.

Purpose of the study: Epithelioid hemangioendothelioma is considered to be a low-grade malignant sarcoma. Surgical treatment is indicated. The multifocal form of the disease is always located on the right side, involving either a limb or the half-body. Based on a case report and a review of the literature, we discuss the nature of this tumor which may or may not be malignant and examine possible indications for non-mutilating surgery.

Case report: A 17-year-old female consulted in 1996 for lytic lesions involving the right lower limb. Most of the lesions were located on the lower portion of the femoral metaphysic and the upper tibia. The lesions appeared as lytic defects with minimal fracture-like lines on the rims. There was also a defect in the lower tibia shaft and in the foot. All lesions involved the right lower limb. Biopsy material obtained in July 1996 confirmed the diagnosis of grade I epithelioid hemangioendothelioma. Search for skeletal and visceral extension was negative. The classical treatment described in the literature is disarticulation of the hip. Considering the static presentation of the lesions, a conservative treatment was attempted. On July 17, 1996, the patient underwent surgery for curettage, liquid nitrogen cyrotherapy, and cement filling of the tibial and lower femoral defects where there was a risk of fracture. The other lesions were not treated. In 1999, the lesion located in the toe became painful and was treated by cement filling. The patient was discharged with simple surveillance. In 2005, i.e. 102 months after cement filling, this young patient was continuing her university education. No new lesion had developed. All lesions remained stable or regressed, including those which had not been treated.

Discussion: Multifocal epithelioid hemangioendothelioma is generally considered as a sarcoma. Careful examination of the literature (less than 50 cases published) shows many discordant elements. First, the lesions are always hemimelic on the right side, never described on the left, an unusual situation for a malignant process. In addition, the clinical courses described to not lead to death by metastasis. The only progression reported involves possible growth of known lesions. The malignant nature of this disease is thus questionable, even though the histological aspect is exactly the same as monofocal epithelioid hemangioendothelioma which can have a much more aggressive, clearly malignant, course.

Conclusion: Surgeons must not propose first-intention amputation for multifocal epithelioid hemangioendothelioma. Because of the favorable outcome observed in our patient warrants, the malignant nature of this condition is questionable.

Purpose of the study: Prognosis is generally considered poor for patients with an iliac bone localization of Ewing sarcoma because the deep tumor is often large with initial metastases. This study demonstrates that the predictive value of these factors is related to treatment and that early en bloc resection can modify the prognosis.

Material and method: We have treated 62 cases of Ewing tumor of the iliac bone since 1976, 36 males and 25 females, mean age 16.5 years (range 4 – 47). Thirteen patients presented primary metastases. Mean tumor size, measured by digital imaging, was 729 cm3. Adapted chemotherapy was given in all cases. Local treatment included exclusive radiotherapy in 20 patients, radiochemotherapy in 15 and exclusive en bloc extratumoral resection in 27.

Results: At 15 years mean follow-up, overall relapse-free survival at ten years was 38%. This rate was 43% among patients without metastasis and 18% for those with initial metastasis (the three other patients underwent surgical resection of a primary focus and a bone metastasis). For patients with localized disease, prognosis was essentially determined by type and timing of local treatment. Surgical resection did not appear to have a significant effect on prognosis for patients operated on after three months; for these patients, only those with total histological response survived. Conversely, patient who underwent surgery before three months with en bloc resection and chemotherapy with at least five drugs had a relapse-free survival of 80% at ten years.

Conclusion: The prognosis of Ewing sarcoma is seriously dependent on the therapeutic modality, even when the localization is known to have a poor prognosis such as the iliac bone. Early en bloc extratumoral resection (before three months) greatly improved the prognosis of patients without metastasis, even for those with a very large tumor. Conversely, prognosis remained very poor for patients given exclusive radiotherapy or operated on late.

Purpose of the study: Infection is the most severe orthopedic complication observed after conservative surgery. The purpose of this study was to ascertain the incidence and causes of such infection and analyze progress achieved over the last ten years.

Material and methods: From 1983 to 2004, surgical procedures were performed in more than 600 patients with bone sarcomas; 520 underwent reconstruction with a prosthesis and/or massive allow graft and were followed for at least six months. Age ranged from 4.5 to 82 years. Deep infections occurred in 47 patients requiring one or several revisions. Three other cases of infection, in patients initially given in other institutions, were included in the series. The study population thus included 50 deep infections after massive reconstruction. Forty-five of these patients had received chemotherapy and 20 radiotherapy. All patients were given adapted antibiotic therapy. Four patients required emergency amputation, and cleaning was attempted in 26. When the infection persisted, or when the infection became chronic, implanted material was removed systematically with insertion of an antibiotic-loaded spacer (gentamycin alon before 1990 then gentamycin+vancomycin). Reimplantation was attempted three to six weeks later when the laboratory results were satisfactory and the muscular and cutaneous situation was sufficient.

Results: Mean follow-up after infection was 8.5 years. At last follow-up, amputation had been necessary in 21 of the 50 patients. The limb was intact in the 29 others but the prosthesis could be reimplanted in only 27 after an average of 2.4 operations. The statistical analysis demonstrated that radiotherapy is a factor of poor prognosis (14 amputations in 20 radiotherapy patients versus 7 amputations in 30 patients without radiation) and that adjunction of vancomycin into the spacer cement has a beneficial effect (15 amputations in 23 patients without vancomycin versus 6 amputations in 27 patients with vancomycin.

Conclusion: Infection of a massive prosthesis is the most serious orthopedic complication because limb survival is compromised. Preventive treatment is crucial: radiotherapy should be avoided and duration of aplasia limited by the use of hematopoietic growth factors. Curative treatment can be achieved with early removal of implanted material, surgical cover with a muscle flap, and adjunction of vancomycin to the spacer cement. The role of prolonged systemic antibiotics remains controversial.