Treatment of Ewing's sarcoma in the cooperative Ewing's sarcoma study group

Multidisciplinary treatment approaches have greatly improved the prognosis of patients with Ewing's sarcoma. Four- or five-drug combination chemotherapy regimens are combined with radiation and/or surgery for local control. Currently, in the Cooperative Ewing's Sancoma Study (CESS) trials, approximately 60% of the protocol patients with Ewing's sarcoma show a relapse-free 10-year survival. For improvement of local control, preoperative radiotherapy and/or brachytherapy may be helpful. The early detection of blood and/or bone marrow contamination by molecular diagnosis can lead to a new clinical staging system. A myeloablative regimen consisting of simultaneous total-body irradiation and chemotherapy may improve the prognosis of patients with disseminated disease.     

Prognosis of Ewing's sarcoma of the head and neck

Five children with Ewing's sarcoma, an uncommon neoplasm originating in the facial bones, were treated with chemotherapy, surgery, and radiotherapy. Younger children have poor prognoses. Children with tumors originating in the mandible appear to have better prognoses. Hematogenic metastases were the most frequent and those originating from maxillary tumors showed much faster growth and were resistant to the chemotherapy used.     

Local and systemic control in Ewing's sarcoma of the femur treated with chemotherapy,and locally by radiotherapy and/or surgery

The role of radiotherapy and/or surgery in the local treatment of Ewing's sarcoma has still to be determined. The outcome of Ewing's sarcoma may differ according to its location and a selection bias towards surgery limits the ability to compare methods of local treatment. We have carried out a retrospective review of 91 consecutive patients treated for non-metastatic Ewing's sarcoma of the femur. They received chemotherapy according to four different protocols. The primary lesion was treated by surgery alone (54 patients), surgery and radiotherapy (13) and radiotherapy alone (23). One was treated by chemotherapy alone. At a median follow-up of ten years, 48 patients (53%) remain free from disease, 39 (43%) have relapsed, two (2%) have died from chemotherapeutic toxicity and two (2%) have developed a radio-induced second tumour. The probability of survival without local recurrence was significantly (p = 0.01) higher in patients who were treated by surgery with or without radiotherapy (88%) than for patients who received radiotherapy alone (59%). The five- and ten-year overall survival rates were 64% and 57%, respectively. Patients who were treated by surgery, with or without radiotherapy, had a five- and ten-year overall survival of 64%. Patients who received only radiotherapy had a five- and ten-year survival of 57% and 44%, respectively. Our results indicate that in patients with Ewing's sarcoma of the femur, better local control is achieved by surgical treatment (with or without radiotherapy) compared with the use of radiotherapy alone. Further studies are needed to verify the impact of this strategy on overall survival.     

Fractures of long bones previously treated for Ewing's sarcoma

Patients with Ewing's sarcoma of a long bone who survive for two years from the time of diagnosis and have been treated with irradiation and chemotherapy have a significant risk of fracture of the involved segment of bone. In our experience, this risk is especially high when the humerus or femur is involved. Healing of these fractures is not normal, and our data suggest that early or even prophylactic internal fixation and bone-grafting may be indicated.     

Ewing's sarcoma in the retroperitoneum

The patient was a female aged 18. Hematuria was discovered at an examination two years previously at which time the patient was complaining of right lumbar pain. The patient visited our hospital with a chief complaint of right abdominal tumor in June, 1988. A retroperitoneal tumor, 77 X 45 mm in size, was observed by CT and ultrasonic examination, and the right kidney was in hydronephrosis. An operation was performed in September, 1988, based on a diagnosis of retroperitoneal tumor and hydronephrosis. The tumor was seen to have involved the right ureter. Subsequently the tumor was surgically removed; right total nephroureterectomy and partial cystectomy were performed at that time. The removed tumor was 90 X 70 mm in size and 120 g in weight; it was histopathologically diagnosed as extraskeletal Ewing sarcoma. A systemic examination was carried out postoperatively, but no abnormalities were observed. As of September, 1990, the patient had had no recurrence and was being kept observation on an outpatient basis. There have been a total of 210 case reports regarding extraskeletal Ewing sarcoma published worldwide, including our case and 26 cases in Japan, so far as we know. However those of a lesion of retroperitoneal origin are extremely rare, numbering only 11 cases worldwide and 2 Japan. Herein we report our case of extraskeletal Ewing sarcoma with reference to the other 209 cases reported in the literature to date.     

Ewing's sarcoma of the mandible

Cure rates of Ewing's sarcoma have improved from 10% to 75% with the use of a combination of local therapy and aggressive multi-drug chemotherapy, to decrease both the incidence of local disease recurrence and the development of pulmonary and skeletal metastases. In Ewing's sarcoma of the mandible, complete surgical resection is preferred to irradiation for local control in those instances when surgery can be performed with minimal loss of function and disfigurement.     

Ewing's sarcoma of the pelvis

Summary Two hundred and thirty-nine patients with Ewing's sarcoma were treated at our institution between 1972 and 1987: 42 of these had lesions in the pelvis, 29 were in the wing of the ilium or involved the sacroiliac joint (type I), 5 were periacetabular (type II), and 8 were in the anterior pelvic arch (type III). Radiotherapy alone was used for the primary lesion in 11 cases, adjuvant chemotherapy in 20 and a neoadjuvant protocol in 22. The overall disease-free survival at a mean follow up of 34 months was 19%. There was no difference in survival related to age or the site in the pelvis, none in disease-free survival with adjuvant and neoadjuvant chemotherapy, or in the incidence of local recurrence and metastases in these two groups. Similarly, there was no difference in disease-free survival between operative treatment, with or without radiotherapy, and radiotherapy alone. There was a slight trend towards better local control of the disease in the former group compared to the latter, although this difference was not statistically significant. Our conclusion is that treatment needs to be planned for each individual patient.

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Résumé De 1972 à 1987, 239 patients atteints de sarcome d'Ewing ont été traités à l'Institut Orthopédique Rizzoli. Parmi eux 42 avaient des lésions pelviennes et ont été pris en compte dans cette étude. Vingt-neuf lésions étaient localisées à l'aile iliaque ou atteignaient l'articulation sacro-iliaque (type I), cinq lésions étaient periacétabulaires (type II) et huit étaient localisées à l'arc pelvien antérieur (type III). Dans 11 cas la lésion primitive à été traitée par résection seule ou par résection associée à la radiothérapie. Dans 31 cas on a traité la lésion primitive seulement par radiothérapie. La chimiothérapie adjuvante à été utilisée chez 20 patients alors qu'un protocole «néoadjuvant» a été suivi chez 22 d'entre eux. La survie sans rechute avec un recul moyen de 34 mois a été de 19%. Aucune différence de survie concernant l'âge ou la localisation pelvienne n'a été observée. En comparant les résultats de survie sans rechute aucune différence n'a été notée entre les patients traités par chimiothérapie adjuvante ou «néoadjuvante». Il n'a pas été noté non plus de différences de fréquence des récidives locales ou des métastases entre les 2 groupes. La survie sans rechute a été la même pour les patients dont la lésion primitive, irradiée ou non, a été traitée chirurgicalement (2/11=18,8%) ou simplement par irradiation (6/31=19,4%). La maladie a été un peu mieux contrôlée localement chez les patients traités par la chirurgie: 27,3% de récidives locales au lieu de 38,7% chez les malades irradiés seulement. Cette différence n'est cependant pas statistiquement significative. Les résultats de cette étude nous induisent à penser que le traitement du sarcome d'Ewing du bassin doit être adapté à chaque cas.

     

The immunogistochemical aspects of the angiogenesis by Ewing's sarcoma

Results of observation were analyzed in 68 patients with Ewing's sarcoma. Reagents made by DAKO (France) such as CD-31, endothelial cell, clone JC 70A, isotype: Lg GI, kappa 02/1 ml were used in the study. It was estimated, that CD31 expression activity in endothelial elements was inversely proportional and reliably correlated with malignant process by the Ewing's sarcoma. Indices of lymphoid infiltration in pathological focus were inversely proportional to the expression of CD-31 positive cells. Statistically reliable inversely proportional relationship was revealed between CD-31 expression of endothelial cells and spontaneous tissue necrotization in the Ewing's sarcoma. Reliable direct correlation was ascertained between tumor angiogenesis and amount of CD-31.     

Extraosseous Ewing's sarcoma of the kidney

Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (EES/PNET) of the kidney is an uncommon entity and usually arises from the retroperitoneum and paraspinal region. We report a case of renal EES/PNET and discuss its clinical features, histological findings and management.     

Extra-osseous Ewing's sarcoma of the thumb

The hand is an uncommon location for Ewing's tumour of the bone. This paper details the presentation, pathology and management of an extra-osseous Ewing's sarcoma of the thumb in an adult woman. The management entailed a combination of neo-adjuvant chemotherapy and surgical excision, followed by reconstruction with an interposition bone graft from the subcutaneous surface of the ulna.     

Ewing's sarcoma of the hand: a case report

Ewing's sarcoma of the hand is rare. This tumor was first described in 1921, since then, only a few cases with hand involvement have been reported. We report a case of Ewing's sarcoma of the left hand, presenting as a swelling of the hand gradually enlarging over six months. Plain radiographs showed marked osteolysis of the second, third and fourth metacarpals. Histological confirmation was made following biopsy. Despite amputation and post-operative chemotherapy, death occurred two months later due marrow aplasia.     

Silver-coated megaprostheses in the proximal femur in patients with sarcoma

European Journal of Orthopaedic Surgery & Traumatology - Proximal femur replacements in patients with sarcoma are associated with high rates of infection. This study is the largest one...     

Ewing's sarcoma of the hand: localization and treatment

Ewing's sarcoma of the hand is rare. Only 20 cases of Ewing's sarcoma of the hand have been reported in the literature. Although Ewing's sarcoma of the hand is rare and highly malignant, modern combinations of treatment are possibly curative due to the tumors surgical accessibility and apparent restricted involvement of only tubular bone. This case study illustrates currently accepted combination therapy consisting of chemotherapy, radiation therapy, and surgical tumor reduction. In spite of the advances of adjuvant therapy, surgical removal of the tumor still remains the most effective treatment.