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Angiomatoid Fibrous Histiocytoma With Paraneoplastic Platelet Storage Pool Deficiency

Authors :
Jennifer D. Elster
Lerraughn M. Morgan
Susan Coventry
Emily R. Miller
Ashok Raj
Source :
Pediatrics. 141(3)
Publication Year :
2017

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.

Details

ISSN :
10984275
Volume :
141
Issue :
3
Database :
OpenAIRE
Journal :
Pediatrics
Accession number :
edsair.doi.dedup.....1a3b58cee42a1d01717b3b3f6ffdfdaa