Angiomatoid Fibrous Histiocytoma With Paraneoplastic Platelet Storage Pool Deficiency

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 histolo...

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Veröffentlicht in:Pediatrics (Evanston) 2018-03, Vol.141, p.1
Hauptverfasser: Morgan, Lerraughn M, Miller, Emily R, Raj, Ashok B, Coventry, Susan C, Elster, Jennifer D
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Sprache:eng
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Zusammenfassung: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.
ISSN:0031-4005
1098-4275