MALIGNANT VASCULAR TUMORS IN YOUNG-PATIENTS

BACKGROUND. To the authors' knowledge there are few published series o f malignant vascular tumors in patients age less than or equal to 21 y ears. METHODS. The authors retrospectively documented the clinical pre sentation, pathology, treatment, and outcome of patients age less than or equal to 21 years with malignant vascular tumors treated between 1 970-1995 at Memorial Sloan-Kettering Cancer Center. The histologic sec tions were rereviewed to confirm the diagnosis. RESULTS. Four patients were identified with angiosarcoma and two with malignant hemangioendo thelioma. Five patients were female and one was male; the median age a t diagnosis was 11.8 years (range, 8 months-21 years). The tumor invol ved the skin in one patient, soft tissue in one patient, bone in two p atients, and internal organs in two patients. One patient had associat ed diffuse angiomatosis and another had the Klippel-Trenaunay-Weber sy ndrome. This patient received prior radiation therapy to the primary s ite with the subsequent development of a vascular sarcoma. None of the patients had distant metastases at diagnosis. Resection was attempted in five patients and completed in four. Chemotherapy alone was given to three patients whereas radiation therapy alone and radiation plus c hemotherapy were administered to one patient each. The median follow-u p was 4.9 years (range, 1 month-12 years). There were three deaths fro m progressive disease and two deaths from locoregional recurrences. Re excision and radiotherapy controlled one local recurrence. Another pat ient developed recurrence to regional lymph nodes and further dissemin ation. The primary tumor in all three survivors was excised completely whereas two of the three patients who died of progressive disease und erwent an incomplete excision or biopsy alone. CONCLUSIONS. Malignant vascular tumors are rare in the first two decades of life and when the y do occur are very aggressive. Complete resection is curative for pat ients with localized lesions. Cancer 1998;83:1634-9. (C) 1998 American Cancer Society.