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.
The GPO Cooperative Ewing's Sarcoma Study (CESS 81 with 10 months four-drug combination chemotherapy (vincristine, actinomycin D, cyclophosphamide, and adriamycin = VACA) and local control with surgery and/or radiation, following week 18, resulted in a Kaplan-Meier estimated disease-free survival of 51% after 6 1/2 years (51/93 patients disease-free). Tumor volume and histological response to primary chemotherapy were identified as most significant prognostic factors. As a consequence, the CESS 86 regimen was stratified according to risk of relapse. Standard risk patients (extremity tumors less than 100 ml tumor volume) were continued on VACA chemotherapy. In high risk patients (extremity tumors greater than 100 ml tumor volume, central tumors), cyclophosphamide in conventional dose (1200 mg/m2/course) was replaced by high doses of ifosfamide (6 g/m2/course) with mesna uroprotection (VAIA). Local control was obtained following week 9. Patients with radiation were randomised for conventional fractionation or accelerated split-course hyperfractionation. The study was piloted from February to December 1985: 27/37 patients were disease-free on October 1, 1987. The ongoing trial was started on January 1, 1986. On October 1, 1987. 63/66 patients were disease-free. In patients with large primaries, according to Kaplan-Meier life-table analysis, the disease-free survival was significantly better in patients receiving VAIA chemotherapy, compared to the previous VACA regimen. The toxicity of both combination chemotherapy regimens was comparable.
Using 31P-MR spectroscopy spectra with good signal-to-noise ratio were obtained in five different types of tumours (Ewing's sarcoma, osteosarcoma, malignant melanoma, metastases from a squamous cell carcinoma, parotid adenoma). Surface coils were used. Short- and long-term follow-up after chemotherapy was possible in some cases. In the short-term follow-up, changes in the phosphocreatine and inorganic phosphate resonances could be observed within minutes after the start of the infusion. In the longer follow-ups, changes in phosphodiester and phosphomonoester resonances were observed within two days. There were no significant changes in tissue pH during treatment, but increased pH values were observed in all tumours.
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