Ectopic cerebellum presenting as a suprasellar mass in infancy: implications for cerebellar development.
A 4-month-old infant with a history of nasopharyngeal teratoma developed progressive optic neuropathy. Neuroimaging studies demonstrated a solid, isointense, suprasellar mass impinging on optic nerves and chiasm superiorly. The mass was subtotally resected. No attachment of the mass to brain stem or cerebellar structures was noted. Histological examination identified the tissue as developing cerebellum. The cytoarchitecture and cellular constituents of the cerebellar tissue were only slightly distorted. All cerebellar cortical constituents were arranged anatomically, and an external granular cell layer was present superficially. The latter was actively proliferating and appropriately cellular for the infant's age. The clinical presentation of ectopic cerebellum as a suprasellar mass in an infant is highly unusual. Moreover, this example illustrates the ability of cerebellar tissue to mature appropriately in a site distant from the posterior fossa, removed from ascending and descending afferent projections. Intrinsic signaling mechanisms appear sufficient to direct histogenesis in developing cerebellar cortex.
Chang
A H
AH
Department of Pathology, Johns Hopkins University Medical School, Baltimore, MD 21287, USA.
Kaufmann
W E
WE
Brat
D J
DJ
D002528:Cerebellar Neoplasms / Q000175:diagnosis*; D002531:Cerebellum; D002828:Choristoma / Q000150:complications / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D009303:Nasopharyngeal Neoplasms / Q000150:complications / Q000473:pathology; D013724:Teratoma / Q000150:complications / Q000473:pathology