Juvenile Psammomatoid Ossifying Fibroma of the Orbital Roof: A Rare Cause of Proptosis among Children

Pediatr Neurosurg. 2020;55(3):163-168. doi: 10.1159/000508691. Epub 2020 Aug 5.

Abstract

Introduction: Juvenile psammomatoid ossifying fibroma (JPOF) is a rare bone tumor characterized by a predilection for the sinonasal region and a tendency to affect younger patients, with a potential for aggressive growth and high recurrence (30-56%). JPOF warrants complete surgical resection to avoid recurrence.

Case presentation: In this article, we report a young boy who presented with unilateral prop-tosis with an expansile bony tumor with ground glass appearance involving the left frontal bone and orbital roof on his images. Complete surgical resection was done, and histopathological examination revealed JPOF with abundant psammomatoid bodies.

Discussion: This patient is a rare case of neurocranial JOPF and adds new features to the typical features already described for JPOF.

Keywords: Orbital tumor; Ossifying fibroma; Proptosis.

Publication types

  • Case Reports

MeSH terms

  • Bone Neoplasms / complications
  • Bone Neoplasms / diagnostic imaging
  • Bone Neoplasms / surgery*
  • Child
  • Exophthalmos / diagnostic imaging
  • Exophthalmos / etiology
  • Exophthalmos / surgery*
  • Fibroma, Ossifying / complications
  • Fibroma, Ossifying / diagnostic imaging
  • Fibroma, Ossifying / surgery*
  • Follow-Up Studies
  • Humans
  • Male
  • Orbit / diagnostic imaging
  • Orbit / surgery*