Papillary craniopharyngioma (PCP) is a rare type of tumor, comprising ∼20% of all craniopharyngioma (CP) cases. It is now recognized as a separate pathological entity from the adamantinomatous type. PCPs are benign tumors, classified as WHO grade 1, characterized by non-keratinizing squamous epithelium. They typically grow as solid and round papillomatous masses or as unilocular cysts with a cauliflower-like excrescence. PCPs primarily occur in adults (95%), with increased frequency in males (60%) and predominantly affect the hypothalamus. Over 80% of these tumors are located in the third ventricle, expanding either above an anatomically intact infundibulum (strictly third ventricle tumors) or within the infundibulo-tuberal region of the third ventricle floor. Clinical manifestations commonly include visual deficits and a wide range of psychiatric disturbances (45% of patients), such as memory deficits and odd behavior. MRI can identify up to 50% of PCPs by the presence of a basal duct-like recess. Surgical management is challenging, requiring complex approaches to the third ventricle and posing significant risk of hypothalamic injury. The endoscopic endonasal approach allows radical tumor resection and yields more favorable patient outcomes. Of intriguing pathogenesis, over 90% of PCPs harbor the somatic BRAFV600E mutation, which activates the mitogen-activated protein kinase (MAPK/ERK) signaling pathway. A phase 2 clinical trial has demonstrated that PCPs respond well to BRAF/MEK inhibitors. This comprehensive review synthesizes information from a cohort of 560 well-described PCPs and 99 large CP series including PCP cases published from 1856-2023 and represents the most extensive collection of knowledge on PCPs to date.

中文翻译：

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状颅咽管瘤：综合和全面的综述。


状颅咽管瘤 （PCP） 是一种罕见的肿瘤，约占所有颅咽管瘤 （CP） 病例的 20%。它现在被认为是与金刚质型不同的病理实体。PCP 是良性肿瘤，被归类为 WHO 1 级，其特征是非角化鳞状上皮。它们通常生长为实性圆形状瘤肿块或单房囊肿，带有花椰菜状分泌物。PCP 主要发生在成人 （95%），男性 （60%） 的频率增加，主要影响下丘脑。这些肿瘤中超过 80% 位于第三脑室，要么在解剖学上完整的漏斗部上方（严格来说是第三脑室肿瘤）上方，要么在第三脑室底的漏斗管区域内。临床表现通常包括视力缺陷和广泛的精神障碍 （45% 的患者），例如记忆缺陷和奇怪的行为。MRI 可以通过存在基底导管样隐窝来识别高达 50% 的 PCP。手术治疗具有挑战性，需要复杂的第三脑室入路，并存在下丘脑损伤的重大风险。内窥镜鼻内入路允许根治性肿瘤切除并产生更有利的患者预后。有趣的发病机制是，超过 90% 的 PCP 携带体细胞 BRAFV600E 突变，该突变激活了丝裂原活化蛋白激酶 （MAPK/ERK） 信号通路。一项 2 期临床试验表明，PCP 对 BRAF/MEK 抑制剂反应良好。本综述综合了来自 560 个描述良好的 PCP 和 99 个大型 CP 系列的信息，包括 1856-2023 年发表的 PCP 病例，代表了迄今为止最广泛的 PCP 知识集合。