金刚瘤性颅咽管瘤是一种罕见的肿瘤,起源于Rathkes袋的残余

Vanishree Murugavel, Sunderesh kamal Chander, Sonti Sulochana
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摘要

颅咽管瘤是一种罕见的肿瘤,起源于从鼻咽部到下丘脑的原始口(Rathke 's pouch)内陷的任何一点的鳞状突起。1硬瘤性颅咽管瘤的发生有两个高峰,一个高峰在第1 ~ 2年,另一个高峰在第5年。它们通常是散发性的,其分子发病机制尚不清楚。颅咽管瘤有两种临床病理形式:乳头状鳞状型和硬瘤型颅咽管瘤。经典的金刚素瘤变异,影响所有年龄的人,由栅栏柱状细胞组成,看起来像胚胎牙芽的成釉细胞。与乳头状颅咽管瘤相比,预后较差。它具有β-连环蛋白的潜在突变和蛋白质向细胞核的易位,这可以通过免疫组织化学证明。
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Adamantinomatous Craniopharyngioma-A rare Tumor arising from remnant of Rathkes pouch
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.
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