A total of 60 consecutive patients with localized Ewing's sarcoma of bone who were entered into the Cooperative Ewing's Sarcoma Study of the German Society of Pediatric Oncology from January 1981 until April 1985 were evaluable for tumor volume at diagnosis. The tumor volume was calculated from plain X-rays and CT scans as ellipsoidal or cylindrical depending on the tumor configuration and presence or absence of a soft tissue component. The 3-year disease-free survival rate according to Kaplan-Meier life table analysis was 78% for tumors with a volume less than 100 ml compared to 17% for tumors greater than or equal to 100 ml volume. These results were independent of the site of the tumor, though larger tumors were primarily located in central and proximal extremity sites. Maximal tumor extension was less precise than tumor volume in predicting prognosis. The ratio of tumor volume to body surface area, body length, or body weight did not increase the ability to separate prognostic groups compared to tumor volume. The better prognosis for patients following radical surgery seems to be in part due to a biased distribution of tumor volumes within local therapy groups, since more patients with smaller tumors had surgery for local control.

The clinical presentation of the disease and the results of treatment in 42 patients with malignant peripheral neuroectodermal tumors (MPNT) entered into the Cooperative Ewing's, soft tissue, and neuroblastoma trials of the German Society of Pediatric Oncology were retrospectively analyzed. Within the Ewing's sarcoma trial, patients with chest wall lesions were particularly analysed for MPNT features. The period of observation ranged from 15 to 86 months; the median relapse-free time was 24 months. There were 28 male and 14 female patients, the median age of patients was 15 years (range, 9 months-23 years). Thirty-two patients had l o c a l i i disease (MO), and ten patients presented with primary metastases (Ml). The predominant location of the tumors was the thoracopulmonary region, followed by the extremities, the abdominal/pelvic, and head and neck region. Thirty-one of 42 tumors invohed the adjacent bone. The disease-free survival according to Kaplan-Meier life-table analysis was 56% f 11% for Stage MO patients at 3 years. Nine of ten patients with M1 disease showed progression of their disease. Most patients had combined modality treatment with surgery, chemotherapy and radiation therapy. Best results were obtained with extensive surgery. Radiation doses ranged from 20 to 60 Gy and could not be correlated with the outcome of the disease. Most recurrences occurred at the site of the primary tumor. In patients with primary chemotherapy after biopsy-proven diagnosis, the responsiveness of this disease to chemotherapy could be demonstrated. Combination chemotherapy containing anthracyclines and high doses of alkylating agents appeared superior. Cancer 61:349-357, 1988. ALIGNANT PERIPHERAL neuroectodermal tumors M (MPNT) form a distinct entity from other malignant small round cell tumors such as neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma, and lymphoma and were first distinguished by their light and electron microscopic features. Their neuroectodermal origin was then confirmed by immunohistochemical rnethod~.~" Cytogenetic studies demonstrated a special relation of MPNT to Ewing's sarcoma.'-1° The MPNT arise in soft tissue and bone.

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