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快速复发性难治性 Rathke 裂隙囊肿:病例报告及文献复习。

Rapidly recurrent recalcitrant Rathke Cleft Cyst: Case report and review of the literature.

机构信息

Department of Neurological Surgery, University of California, San Francisco, San Francisco, California; Department of Neurosurgery, The George Washington University, Washington, District of Columbia, United States.

Department of Neurosurgery, The George Washington University, Washington, District of Columbia, United States.

出版信息

Neurochirurgie. 2022 Oct;68(5):535-539. doi: 10.1016/j.neuchi.2022.01.002. Epub 2022 Mar 5.

DOI:10.1016/j.neuchi.2022.01.002
PMID:35260274
Abstract

BACKGROUND

Rathke Cleft Cysts (RCC) are cystic sellar or suprasellar nonneoplastic lesions that are thought to originate from remnants of the rathke pouch. Postoperatively, RCCs have a tendency to reaccumulate, after which preoperative symptoms may recur. However, there exists very little evidence on which treatments are most effective in these patients.

CASE-DESCRIPTION: In this report, we present a unique case of a symptomatic RCC that recurred four times after initial transsphenoidal surgery. Following each surgery, the patient had significant visual improvement with post-op imaging displaying decompression of the neural elements. However, RCC reaccumulated in strikingly rapid time intervals of 1.5 months, 0.5 months, 1.5 years, and 5 months after each respective prior surgery. Repeat interventions with transsphenoidal, pterional and supraorbital approaches were unsuccessful in providing a durable treatment response. The patient ultimately underwent radiotherapy after a final surgical marsupialization of the cyst and has since displayed stable imaging with improved vision. This patient represented a 'perfect storm' of factors that may contribute to cyst recurrence, including substantial visual field deficits, large cyst size, peripheral wall enhancements on MRI, an intraoperative CSF leak, use of a fat graft, subtotal resection of the portion of cyst wall that adhered to important suprasellar structures, squamous metaplasia noted in cyst wall, and suprasellar extension.

CONCLUSIONS

We demonstrate that different surgical approaches through repeat surgeries may not assist in prevention of further recurrence; instead, we propose that radiotherapy should be offered early in the treatment course of recurrent cases that have additional risk factors for further reoccurrence.

摘要

背景

Rathke 裂隙囊肿(RCC)是一种囊性鞍内或鞍上非肿瘤性病变,被认为起源于 Rathke 囊的残余物。术后,RCC 有再积聚的倾向,此后术前症状可能会再次出现。然而,对于这些患者,哪种治疗方法最有效,证据非常有限。

病例描述

在本报告中,我们介绍了一例独特的有症状的 RCC 病例,该病例在初次经蝶窦手术后复发了 4 次。每次手术后,患者的视力均有显著改善,术后影像学显示神经元素减压。然而,RCC 在令人惊讶的短时间内再次积聚,分别在每次手术前 1.5 个月、0.5 个月、1.5 年和 5 个月。经蝶窦、翼点和眶上入路的重复干预措施均未能提供持久的治疗反应。在最后一次手术囊袋切开术和放射治疗后,患者最终接受了放射治疗,此后影像学显示稳定,视力改善。该患者代表了可能导致囊肿复发的多种因素的“完美风暴”,包括明显的视野缺损、囊肿大、MRI 上周边壁增强、术中脑脊液漏、使用脂肪移植物、对与重要鞍上结构粘连的囊壁部分进行次全切除、囊壁鳞状化生以及鞍上延伸。

结论

我们表明,通过重复手术进行不同的手术方法可能无助于预防进一步复发;相反,我们建议对于有进一步复发风险因素的复发性病例,应在治疗过程中尽早提供放射治疗。

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Rapidly recurrent recalcitrant Rathke Cleft Cyst: Case report and review of the literature.快速复发性难治性 Rathke 裂隙囊肿:病例报告及文献复习。
Neurochirurgie. 2022 Oct;68(5):535-539. doi: 10.1016/j.neuchi.2022.01.002. Epub 2022 Mar 5.
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Nuclear translocation of beta catenin in patients with Rathke cleft cysts-clinical and imaging characteristics and risk of recurrence.Rathke 裂囊肿患者中β连环蛋白的核转位——临床和影像学特征及复发风险。
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Characteristics of Rathke's cleft cyst based on cyst location with a primary focus on recurrence after resection.基于囊肿位置的拉克氏裂囊肿的特征,主要关注切除术后的复发情况。
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Surgical outcomes in 118 patients with Rathke cleft cysts.118例拉克氏囊肿患者的手术结果
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Presentation and outcomes in surgically and conservatively managed pediatric Rathke cleft cysts.手术治疗与保守治疗的小儿拉克氏裂囊肿的临床表现及预后
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Long-term outcome of surgically treated and conservatively managed Rathke cleft cysts.经手术治疗和保守治疗的 Rathke 裂隙囊肿的长期结果。
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Endoscopic endonasal resection of Rathke cleft cysts: a single-institution analysis of 148 consecutive patients.经鼻内镜下Rathke裂囊肿切除术:单机构对148例连续患者的分析
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Surgical treatment of rathke cleft cysts in children.儿童拉克氏裂囊肿的外科治疗
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引用本文的文献

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Rathke's cleft cyst: From history to molecular genetics.拉克氏裂囊肿:从历史到分子遗传学
Rev Endocr Metab Disord. 2025 Apr;26(2):229-260. doi: 10.1007/s11154-025-09949-6. Epub 2025 Feb 13.
2
Huge intrasphenoidal Rathke's cleft cyst: a case description and analysis of the literature.巨大蝶鞍内拉克氏囊肿:病例描述及文献分析
Quant Imaging Med Surg. 2023 Dec 1;13(12):8864-8868. doi: 10.21037/qims-23-780. Epub 2023 Nov 7.
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Different Faces of Rathke's Cleft Cyst.拉克氏裂囊肿的不同表现
J Belg Soc Radiol. 2023 Mar 3;107(1):15. doi: 10.5334/jbsr.3047. eCollection 2023.