研究动态
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乳头状颅咽管瘤:综合全面的回顾。

Papillary Craniopharyngioma: an integrative and comprehensive review.

发表日期:2024 Oct 01
作者: Ruth Prieto, Tareq A Juratli, Evan D Bander, Sandro Santagata, Laura Barrios, Priscilla K Brastianos, Theodore H Schwartz, José M Pascual
来源: ENDOCRINE REVIEWS

摘要:

乳头状颅咽管瘤 (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细胞类型不同的病理实体。 PCP 是良性肿瘤,被列为 WHO 1 级,其特征是非角化鳞状上皮。它们通常生长为实心圆形乳头状肿块或具有菜花状赘生物的单眼囊肿。 PCP 主要发生在成人 (95%) 中,男性 (60%) 中发生频率较高,主要影响下丘脑。超过 80% 的这些肿瘤位于第三脑室,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)扩张,要么在第三脑室底的漏斗结节区域内扩张。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 可以通过基底导管样隐窝的存在来识别高达 50% 的 PCP。手术治疗具有挑战性,需要采用复杂的方法进入第三脑室,并带来下丘脑损伤的重大风险。内窥镜鼻内方法可以根治肿瘤切除并产生更有利的患者结果。有趣的发病机制是,超过 90% 的 PCP 含有体细胞 BRAFV600E 突变,该突变会激活丝裂原激活蛋白激酶 (MAPK/ERK) 信号通路。 2 期临床试验表明,PCP 对 BRAF/MEK 抑制剂反应良好。这篇全面的综述综合了 560 个详细描述的 PCP 和 99 个大型 CP 系列的信息,其中包括 1856 年至 2023 年出版的 PCP 案例,代表了迄今为止最广泛的 PCP 知识集合。© 作者 2024。出版者牛津大学出版社代表内分泌学会。版权所有。如需商业重复使用,请联系 reprints@oup.com 获取转载和转载的翻译权。所有其他权限都可以通过我们网站文章页面上的权限链接通过我们的 RightsLink 服务获得 - 如需了解更多信息,请联系journals.permissions@oup.com。有关附加条款,请参阅期刊“关于”页面。
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.© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. See the journal About page for additional terms.