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


METHODS
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.
Department of Neurosurgery, Fattouma Bourguiba University Hospital - University of Medicine of Monastir, Monastir, Tunisia. Electronic address: atefbn@hotmail.fr.
Atef
A
Ben Nsir
Department of Neurosurgery, The Johns Hopkins Hospital - The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Quoc-Anh
QA
Thai
Department of Endocrinology, Farhat Hached University Hospital - University of Medicine of Sousse, Sousse, Tunisia.
Larbi
L
Chaieb
Department of Neurosurgery, The Tunisian National Institute of Neurology - University of Medicine of Tunis El Manar, Tunis, Tunisia.
Hafedh
H
Jemel
D000293:Adolescent; D000568:Amenorrhea / Q000209:etiology*; D002114:Calcinosis / Q000473:pathology / Q000601:surgery*; D003397:Craniopharyngioma / Q000473:pathology / Q000601:surgery*; D003937:Diagnosis, Differential; D005260:Female; D012739:Gonadal Steroid Hormones / Q000097:blood; D006099:Granuloma / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006728:Hormones / Q000097:blood; D006801:Humans; D010911:Pituitary Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D012658:Sella Turcica / Q000601:surgery; D016896:Treatment Outcome
D012739:Gonadal Steroid Hormones; D006728:Hormones
Cholesterol granuloma; Craniopharyngioma; Suprasellar region; Surgery; Xanthogranuloma