Calcified Suprasellar Xanthogranuloma Presenting with Primary Amenorrhea in a 17-Year-Old Girl: Case Report and Literature Review

Atef Ben Nsir, Quoc Anh Thai, Larbi Chaieb, Hafedh Jemel

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

Abstract

Background Xanthogranuloma, also known as cholesterol granuloma, is an extremely rare intracranial neoplasm most commonly located in the middle ear, petrous apex, or choroid plexus. Exclusively suprasellar xanthogranulomas are exceptional and this report presents a very rare case in the pediatric population, particularly unique due to the presence of calcification. Case Description A 17-year-old girl presented with primary amenorrhea with computed tomography and magnetic resonance imaging showing a large calcified enhancing suprasellar mass, which was presumptively diagnosed as a craniopharyngioma on the basis of its clinical and radiologic appearance. Gross total resection of a well-encapsulated, exclusively suprasellar tumor was achieved, without postoperative neurologic deficits. Histologic examination found fibrous tissue with abundant cholesterol clefts, multinucleated giant cells, and hemosiderin deposits but no epithelial cells. The final histologic diagnosis was a xanthogranuloma. Conclusions Xanthogranuloma, although extremely rare in the pediatric population, may present as a calcified suprasellar mass and manifest with primary amenorrhea. The prognosis after gross total resection is likely favorable; however, long-term follow-up is indicated for these rare neoplasms.

Original languageEnglish (US)
Pages (from-to)866.e11-866.E14
JournalWorld neurosurgery
Volume84
Issue number3
DOIs
StatePublished - Sep 1 2015

Keywords

  • Cholesterol granuloma
  • Craniopharyngioma
  • Suprasellar region
  • Surgery
  • Xanthogranuloma

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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