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Rathke 氏裂隙囊肿:从病理生理学到治疗管理。

Rathke's cleft cysts: from pathophysiology to management.

机构信息

School of Medicine, Koc University, Istanbul, Turkey.

Department of Neurosurgery, Korle Bu Teaching Hospital, Accra, Ghana.

出版信息

Neurosurg Rev. 2024 Sep 3;47(1):522. doi: 10.1007/s10143-024-02742-0.

Abstract

Rathke's cleft cysts (RCCs) are benign, non-neoplastic lesions located in the sellar and suprasellar regions of the brain, originating from remnants of Rathke's pouch, an embryonic precursor to the anterior pituitary gland. Although RCCs are frequently asymptomatic and discovered incidentally during imaging studies, they can present with a variety of symptoms, including headaches, visual disturbances, and endocrine dysfunction due to the compression of adjacent neural structures. The management of RCCs is particularly challenging, as the decision to pursue conservative monitoring or surgical intervention depends heavily on the cyst's size, growth potential, and the severity of symptoms. Transsphenoidal surgery is the primary treatment for symptomatic RCCs, offering effective relief from symptoms through decompression of the cyst. However, recurrence remains a significant issue, with rates reported up to 33%, prompting debates about the extent of cyst wall removal during surgery. Recent advancements in minimally invasive endoscopic techniques have improved surgical outcomes, yet the risk of postoperative complications such as hypopituitarism and cerebrospinal fluid leaks persists. Additionally, stereotactic radiosurgery has emerged as a potential alternative for patients with recurrent RCCs or those who are not suitable candidates for repeat surgery. Despite its promise, the long-term safety and efficacy of radiotherapy in RCC management require further investigation. This narrative review aims to provide a comprehensive overview of RCCs, integrating the latest research and clinical guidelines to discuss pathophysiology, clinical presentation, and management strategies, emphasizing the need for a personalized approach to treating this complex condition.

摘要

Rathke 氏囊囊肿(RCC)是良性、非肿瘤性病变,位于脑垂体窝和鞍上区,起源于 Rathke 氏囊的残余物,Rathke 氏囊是前垂体的胚胎前体。虽然 RCC 通常无症状,在影像学研究中偶然发现,但它们可表现出多种症状,包括头痛、视觉障碍和由于邻近神经结构受压导致的内分泌功能障碍。RCC 的管理特别具有挑战性,因为决定进行保守监测还是手术干预主要取决于囊肿的大小、生长潜力和症状的严重程度。经蝶窦手术是治疗有症状的 RCC 的主要方法,通过减压囊肿可有效缓解症状。然而,复发仍然是一个重大问题,报道的复发率高达 33%,这引发了关于手术中囊肿壁切除程度的争论。最近微创内镜技术的进步改善了手术结果,但术后并发症如垂体功能减退和脑脊液漏的风险仍然存在。此外,立体定向放射外科已成为复发性 RCC 或不适合重复手术的患者的潜在替代方法。尽管有希望,但放疗在 RCC 管理中的长期安全性和疗效仍需要进一步研究。本综述旨在全面概述 RCC,整合最新的研究和临床指南,讨论其病理生理学、临床表现和管理策略,强调需要针对这种复杂情况采用个性化方法进行治疗。

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