Angiosarcomas arising in the viscera and soft tissue of children and young adults: a clinicopathologic study of 15 cases.

Journal Article

Angiosarcomas are rare tumors that predominantly affect adult and elderly patients and pursue an aggressive clinical course with high mortality. Although angiosarcomas are well described in a variety of clinical settings, they have been incompletely characterized. We identified 15 high-grade angiosarcomas arising from the viscera and soft tissue of patients 21 years old and younger from institutional and consultation files. Both clinical (patient age, tumor site, tumor size, tumor focality) and histologic features including growth pattern (vasoformative vs. solid), nuclear grade (high vs. low), mitotic rate (mitotic figures/10 high-power fields), necrosis (present vs. absent), and cell shape (epithelioid vs. nonepithelioid) were assessed. Tumors arose in both sexes (8 males; 7 females); age at diagnosis ranged from 3 months to 19 years (mean, 10 y, 5 mo; median, 11 y). Tumors were located in the mediastinum (7), visceral organs (2 in liver, 1 in spleen), breast (2), mesentery (1), pelvis (1), and deep soft tissue of upper extremity (1). Tumor size was documented for 8 patients (range 3.5 to 13 cm; mean 8.1 cm). Eight cases showed epithelioid morphology and 7 cases were primarily spindled. Of 8 cases assessed for podoplanin expression by immunohistochemistry, 7 were negative and 1 was positive. Clinical follow-up was obtained for all patients: 10 (67%) died of disease (range, 27 mo to 11 y; mean, 6 y 8 mo) and 4 patients were alive without disease (range, 27 to 132 mo; mean, 79 mo). Although extremely rare, angiosarcomas do affect children and young adults and this diagnosis should be considered in atypical vascular tumors occurring in the mediastinum and those with brisk mitotic activity and/or necrosis.

Full Text

Duke Authors

Cited Authors

  • Deyrup, AT; Miettinen, M; North, PE; Khoury, JD; Tighiouart, M; Spunt, SL; Parham, D; Weiss, SW; Shehata, BM

Published Date

  • February 2009

Published In

Volume / Issue

  • 33 / 2

Start / End Page

  • 264 - 269

PubMed ID

  • 18987547

Electronic International Standard Serial Number (EISSN)

  • 1532-0979

International Standard Serial Number (ISSN)

  • 0147-5185

Digital Object Identifier (DOI)

  • 10.1097/pas.0b013e3181875a5f

Language

  • eng