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 po...

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Bibliographic Details
Published in:World neurosurgery 2015-09, Vol.84 (3), p.866.e11-866.e14
Main Authors: Ben Nsir, Atef, Thai, Quoc-Anh, Chaieb, Larbi, Jemel, Hafedh
Format: Article
Language:English
Subjects:
Adolescent
Amenorrhea - etiology
Calcinosis - pathology
Calcinosis - surgery
Cholesterol granuloma
Craniopharyngioma
Craniopharyngioma - pathology
Craniopharyngioma - surgery
Diagnosis, Differential
Female
Gonadal Steroid Hormones - blood
Granuloma - complications
Granuloma - pathology
Granuloma - surgery
Hormones - blood
Humans
Neurosurgery
Pituitary Neoplasms - complications
Pituitary Neoplasms - pathology
Pituitary Neoplasms - surgery
Sella Turcica - surgery
Suprasellar region
Surgery
Treatment Outcome
Xanthogranuloma
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Summary: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.
ISSN:1878-8750
1878-8769
DOI:10.1016/j.wneu.2015.04.024