Angiomatoid Fibrous Histiocytoma With Paraneoplastic Platelet Storage Pool Deficiency

作者:Morgan Lerraughn M*; Miller Emily R; Raj Ashok B; Coventry Susan C; Elster Jennifer D
来源:Pediatrics, 2018, 141(3): e20162065.
DOI:10.1542/peds.2016-2065

摘要

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.

  • 出版日期2018-3