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鞍上 Rathke 裂囊肿:临床表现和治疗结果。

Suprasellar Rathke cleft cysts: clinical presentation and treatment outcomes.

机构信息

Department of Neurosurgery and California Center for Pituitary Disorders, University of California, San Francisco, California 94143-0112, USA.

出版信息

Neurosurgery. 2011 Nov;69(5):1058-68; discussion 1068-7. doi: 10.1227/NEU.0b013e318228bcea.

Abstract

BACKGROUND

Rathke cleft cysts (RCCs), benign remnants of the Rathke pouch typically arising in the sella, sometimes have suprasellar extension. Purely suprasellar RCCs are rarely reported.

OBJECTIVE

To compare the presentations, surgical outcomes, and pathology of purely suprasellar RCCs and sellar-based RCCs.

METHODS

We retrospectively reviewed records, magnetic resonance images, laboratory results, and pathology of 151 RCC patients surgically managed at our institution from 1989 to 2009. The RCCs were classified as purely sellar (type I, n = 76), sellar with suprasellar extension (type II, n = 56), or purely suprasellar (type III, n = 19).

RESULTS

The RCCs with a suprasellar component (types II and III) more commonly presented with visual dysfunction (P < .001). Complete cyst drainage occurred in 89%, 55%, and 38% of type I, II, and III RCCs, respectively (P < .001). Vision improved in 100%, 55%, and 33% and headache improved in 74%, 64%, and 29% of type I, II, and III patients, respectively (P = .02). Temporary or permanent postoperative diabetes insipidus occurred in 5%, 16%, and 21% of type I, II, and III patients, respectively. (P < .001). In a multivariate analysis, RCC type was the only factor predicting recurrence. Kaplan-Meier 3-year recurrence/progression rates were 0%, 16%, and 29% for type I, II, and III RCCs, respectively (P < .001, type I vs II, type I vs III; P = .5 type II vs III).

CONCLUSION

The RCCs with a suprasellar component are neurosurgically challenging because of their proximity to the optic chiasm and infundibulum. Compared with sellar-based RCCs, RCCs with a suprasellar component more frequently present with visual dysfunction, are more difficult to completely eliminate, recur more frequently, and are associated with higher postoperative endocrine morbidity, and their preoperative visual dysfunction and headache less frequently improve with surgery. These factors must be considered during the treatment of RCCs with a suprasellar component.

摘要

背景

Rathke 裂囊肿(RCC)是 Rathke 囊的良性残余物,通常发生在蝶鞍内,有时会向鞍上延伸。纯粹的鞍上 RCC 很少见报道。

目的

比较单纯鞍上 RCC 和鞍内 RCC 的表现、手术结果和病理学。

方法

我们回顾性分析了 1989 年至 2009 年我院收治的 151 例 RCC 患者的病历、磁共振成像、实验室结果和病理资料。RCC 分为单纯鞍内(I 型,n = 76)、鞍内伴鞍上延伸(II 型,n = 56)和单纯鞍上(III 型,n = 19)。

结果

伴有鞍上成分的 RCC(II 型和 III 型)更常见于视力障碍(P <.001)。I、II、III 型 RCC 的囊肿完全引流率分别为 89%、55%和 38%(P <.001)。视力改善分别为 100%、55%和 33%,头痛改善分别为 74%、64%和 29%,I、II、III 型患者的糖尿病多尿分别为 5%、16%和 21%(P <.001)。在多变量分析中,RCC 类型是唯一预测复发的因素。Kaplan-Meier 3 年复发/进展率分别为 0%、16%和 29%,I、II、III 型 RCC 患者(P <.001,I 型 vs II 型,I 型 vs III 型;P =.5 II 型 vs III 型)。

结论

由于其与视交叉和漏斗的接近,伴有鞍上成分的 RCC 神经外科具有挑战性。与鞍内 RCC 相比,伴有鞍上成分的 RCC 更常出现视力障碍,更难以完全消除,更常复发,术后内分泌发病率更高,术前视力障碍和头痛手术改善的情况也较少。在治疗伴有鞍上成分的 RCC 时,必须考虑这些因素。

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