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Pediatric Cutaneous Angiosarcomas: A Clinicopathologic Study of 10 Cases

Deyrup, Andrea T. MD, PhD*; Miettinen, Markku MD; North, Paula E. MD, PhD; Khoury, Joseph D. MD§; Tighiouart, Mourad PhD; Spunt, Sheri L. MD¶,♯; Parham, David M. MD**; Shehata, Bahig M. MD††; Weiss, Sharon W. MD††

The American Journal of Surgical Pathology: January 2011 - Volume 35 - Issue 1 - p 70–75
doi: 10.1097/PAS.0b013e3181ffd9d5
Original Articles

Cutaneous angiosarcomas are rare tumors, which predominantly arise in the sun-exposed skin of the head and neck of adult and elderly patients. Rarely, these tumors can be seen in children. We identified cutaneous angiosarcomas in 10 children and assessed clinical (patient age, tumor site, tumor size, and tumor focality) and histologic features including growth pattern (vasoformative vs. solid), mitotic rate (mitotic figures per 10 high power field), necrosis (present vs. absent), and cell shape (epithelioid vs. nonepithelioid). Tumors predominated in the lower extremities (6 of 10) of female patients (2 male and 8 female); age at diagnosis ranged from 1.5 months to 15 years. Four patients had preexisting conditions: congenital hemihypertrophy of the contralateral limb, the Aicardi syndrome, congenital lymphedema, and congenital hemangioma treated with radiation therapy. Tumors were located in the lower extremity (6), flank (1), elbow (1), and buccal mucosa (1), and ranged in size from 0.6 to 6.5 cm. Eight cases showed predominantly epithelioid morphology, 1 case showed mixed epithelioid and spindled morphology and 1 case was entirely spindled. Mitotic activity ranged from 1 to 55 mitotic figures per 10 high power field. Necrosis was seen in 5 cases. Clinical follow-up was obtained for 9 patients: 4 died of disease (range, 12 to 49 mo; mean, 25 mo) and 5 patients were alive without disease (18 mo to 28 y). Five patients had metastatic disease; sites of involvement included the lung, soft tissue, lymph node, pleura, liver, and bone. Cutaneous angiosarcomas in children are rare tumors, which are commonly associated with a preexisting condition, suggesting a greater role for genetics as opposed to environmental factors in the pathogenesis of these tumors.

*Pathology Consultants, Greenville, SC

Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC

Department of Pathology, Medical College of Wisconsin, Milwaukee, WI

§Quest Diagnostics, Pathology Department, Las Vegas, NV

Departments of Biostatistics, Winship Cancer Institute

††Pathology, Emory University, Atlanta, GA

Department of Oncology, St Jude Children's Research Hospital

Department of Pediatrics, University of Tennessee Health Science Center, Memphis, TN

**Department of Pathology, University of Oklahoma, Oklahoma City, OK

Correspondence: Andrea T. Deyrup, MD, PhD, Pathology Consultants of Greenville, Greenville, SC 29605 (e-mail:

© 2011 Lippincott Williams & Wilkins, Inc.