Malignant vascular tumors in young patients

• Published in: Cancer Vol. 83; no. 8; pp. 1634 - 1639
• Main Authors: Valle, Pablo Lezama‐del, Gerald, William L., Tsai, Josephine, Meyers, Paul, Quaglia, Michael P. La
• Format: Journal Article
• Language: English
• Published: New York John Wiley & Sons, Inc 15.10.1998; Wiley-Liss
• Subjects: Adolescent; Adult; Angiomatosis - complications; angiosarcoma; Antineoplastic Agents - therapeutic use; Biological and medical sciences; Blood and lymphatic vessels; Bone Neoplasms - pathology; Cardiology. Vascular system; Cause of Death; Chemotherapy, Adjuvant; Child; Child, Preschool; Disease Progression; Female; Follow-Up Studies; hemangioendothelioma; Hemangioendothelioma - drug therapy; Hemangioendothelioma - pathology; Hemangioendothelioma - radiotherapy; Hemangioendothelioma - secondary; Hemangioendothelioma - surgery; Hemangiosarcoma - drug therapy; Hemangiosarcoma - pathology; Hemangiosarcoma - radiotherapy; Hemangiosarcoma - secondary; Hemangiosarcoma - surgery; Humans; Infant; Klippel-Trenaunay-Weber Syndrome - complications; Lymphatic Metastasis; Male; Medical sciences; Miscellaneous; Neoplasm Recurrence, Local - pathology; pediatric; Radiotherapy, Adjuvant; Retrospective Studies; Skin Neoplasms - pathology; Soft Tissue Neoplasms - pathology; treatment; vascular neoplasms; Human; Hemangioendothelioma; Prognosis; Sarcoma; Cardiovascular disease; Malignant tumor; Angiosarcoma; Vascular disease; Case study; Pathology; Treatment; Adolescent; Child
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/(SICI)1097-0142(19981015)83:8<1634::AID-CNCR20>3.0.CO;2-S

BACKGROUND To the authors' knowledge there are few published series of malignant vascular tumors in patients age ≤ 21 years. METHODS The authors retrospectively documented the clinical presentation, pathology, treatment, and outcome of patients age ≤ 21 years with malignant vascular tumors treated between 1970‐1995 at Memorial Sloan‐Kettering Cancer Center. The histologic sections were rereviewed to confirm the diagnosis. RESULTS Four patients were identified with angiosarcoma and two with malignant hemangioendothelioma. Five patients were female and one was male; the median age at diagnosis was 11.8 years (range, 8 months‐21 years). The tumor involved the skin in one patient, soft tissue in one patient, bone in two patients, and internal organs in two patients. One patient had associated diffuse angiomatosis and another had the Klippel‐Trénaunay‐Weber syndrome. This patient received prior radiation therapy to the primary site 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 chemotherapy were administered to one patient each. The median follow‐up was 4.9 years (range, 1 month‐12 years). There were three deaths from progressive disease and two deaths from locoregional recurrences. Reexcision and radiotherapy controlled one local recurrence. Another patient developed recurrence to regional lymph nodes and further dissemination. The primary tumor in all three survivors was excised completely whereas two of the three patients who died of progressive disease underwent an incomplete excision or biopsy alone. CONCLUSIONS Malignant vascular tumors are rare in the first two decades of life and when they do occur are very aggressive. Complete resection is curative for patients with localized lesions. Cancer 1998;83:1634‐1639. © 1998 American Cancer Society. Malignant tumors of vascular tissues are extremely rare. Surgical excision is effective for patients with localized disease.