Ectopic craniopharyngioma of the orbit: illustrative case

Abstract

BACKGROUND Craniopharyngiomas are uncommon malformations of the sellar or parasellar region that are partly cystic and calcified and have low histological grade. The typical age of presentation is bimodal, with peak incidence rates in children at age 5 to 14 years and in adults at age 50 to 74 years. The usual clinical manifestations are related to endocrine deficiencies due to mass effect along with visual impairment and increased intracranial pressure. If a tumor is favorably localized, the treatment of choice is complete resection. OBSERVATIONS The authors presented a unique case of a 61-year-old man with a suspicious cystic lesion in the right orbital roof that was causing right-sided headaches with pressure and pain in the right eye. Both computed tomography and magnetic resonance imaging were used for further evaluation and showed a suspicious lytic bone lesion that had an epicenter within the orbital rim, which was highly suggestive of a tumor of interosseous origin. After removal, the tumor was identified by pathology as a craniopharyngioma. LESSONS The importance of this case report is in documenting a unique case of an ectopic craniopharyngioma in the orbit, adding to current hypotheses of the pathogenesis of ectopic craniopharyngiomas, and presenting an extensive review of literature.

Isolated fourth ventricle craniopharyngioma: Representative case illustration and review of literature

“…e proposed hypothesis by John et al denotes that the abnormal migration of the neural crest cells, in addition to mutations of oncogenes and tumor suppressor genes, are responsible for the "de novo" origin of ectopic craniopharyngioma. [5,10] From an embryological point of view, neural crest cell migrates from the metencephalon and rhombomeres (R1-R8) toward the pharyngeal arches (PA1-PA6) around the 5 th week of gestation following the dorsolateral pathway [Figure 2]. [6] During their migration, ectopic cell rest can reside within the metencephalon, dorsolateral pathway, and pharyngeal arches, which later on give rise to ectopic craniopharyngioma.…”