Angiomatoid fibrous histiocytoma with paraneoplastic platelet storage pool deficiency

Lerraughn M. Morgan, Emily R. Miller, Ashok B. Raj, Susan C. Coventry, Jennifer D. Elster

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

Angiomatoid fibrous histiocytoma is a rare soft tissue tumor usually discovered in young individuals. This tumor is often mistaken for a hematoma and typically misdiagnosed. It is commonly found in the extremities and may be associated with a site of recent or previous trauma. Characteristic histology includes nodules of histiocytoid spindle cells with pseudoangiomatoid spaces, fibrous pseudocapsules, and lymphoid cuffing. We describe the case of an 8-year-old girl who presented after incision and drainage of a superficial thigh lesion and experienced subsequent chronic bleeding of her wound. Her initial presentation was concerning for an underlying bleeding disorder, and laboratory analysis uncovered a paraneoplastic platelet function disorder that resolved with therapy of the primary tumor.

Original languageEnglish (US)
Article numbere20162065
JournalPediatrics
Volume141
Issue number3
DOIs
StatePublished - Mar 2018

Bibliographical note

Publisher Copyright:
© 2018 by the American Academy of Pediatrics.

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