Retroperitoneal Angiomatoid Fibrous Histiocytoma Presenting as a Recurrent Spontaneous Retroperitoneal Hemorrhage in a 9-Year-Old Boy

J Pediatr Hematol Oncol. 2018 May;40(4):307-311. doi: 10.1097/MPH.0000000000001088.

Abstract

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that typically presents in children and young adults. Occurrence outside of the extremities and the head and neck region is exceedingly rare. We report the case of a 9-year-old boy who presented with recurrent retroperitoneal hemorrhage initially thought to be a manifestation of an underlying bleeding disorder. After comprehensive diagnostic work-up, including multiple negative biopsies, the patient underwent surgical resection of an extensively hemorrhagic intramuscular mass and to date remains well. Pathologic examination confirmed AFH with EWSR1 gene rearrangement. This first documented report of an AFH in a retroperitoneal location in a child highlights the diagnostic difficulties and clinical challenges of AFH arising in an atypical location.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Gene Rearrangement*
  • Hemorrhage* / genetics
  • Hemorrhage* / pathology
  • Hemorrhage* / surgery
  • Histiocytoma, Benign Fibrous* / genetics
  • Histiocytoma, Benign Fibrous* / pathology
  • Histiocytoma, Benign Fibrous* / surgery
  • Humans
  • Male
  • RNA-Binding Protein EWS / genetics*
  • Retroperitoneal Neoplasms* / genetics
  • Retroperitoneal Neoplasms* / pathology
  • Retroperitoneal Neoplasms* / surgery

Substances

  • EWSR1 protein, human
  • RNA-Binding Protein EWS