Adamantinomatous Craniopharyngioma-A rare Tumor arising from remnant of Rathkes pouch

Abstract

Craniopharyngioma is a rare tumor arising from squamous rests located at any point along the invagination of the primitive stomodeum, Rathke’s pouch, from the nasopharynx to the hypothalamus. 1 There are two peaks in the occurrence of adamantinomatous craniopharyngioma, one in the first to second decade and the other in the fifth decade. They are generally sporadic and their molecular pathogenesis is poorly defined. There are two clinicopathological forms of craniopharyngiomas-papillary squamous type, and adamantinomatous craniopharyngioma. The classical adamantinomatous variant, which affects people of all ages consists of palisading columnar cells that look like ameloblast of embryonic tooth buds. It has poor prognosis when compared to papillary craniopharyngioma. It has an underlying mutation in β-catenin and translocation of the protein to the nucleus, which can be demonstrated by immunohistochemistry.