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伴有黄色肉芽肿性改变的拉克氏裂囊肿:一例报告并文献复习

Rathke's cleft cyst with xanthogranulomatous change: A case report and review of the literature.

作者信息

Sprau Annelise, Mahavadi Anil, Zhang Michael, Saste Micah, Deftos Michael, Singh Harminder

机构信息

Department of Neurosurgery, University of Miami, Miami, Florida, United States.

Department of Neurological Surgery, School of Medicine, Stanford University, Stanford, United States.

出版信息

Surg Neurol Int. 2020 Aug 15;11:246. doi: 10.25259/SNI_277_2020. eCollection 2020.

Abstract

BACKGROUND

Rathke's cleft cysts (RCCs) are benign, typically asymptomatic sellar lesions found incidentally in adults, with a dramatically lower incidence in pediatric patients (<18 years). We present a case of RCC with xanthogranulomatous change (XGC) - an even less common subtype of RCC - treated by endoscopic endonasal surgical resection. This is the second reported instance of an RCC with XGC occurring in a pediatric patient.

CASE DESCRIPTION

The patient is a 17-year-old male with delayed puberty who presented with bitemporal hemianopsia and was found to have a 2.6 cm lesion, initially thought to be a craniopharyngioma. He subsequently underwent uncomplicated transsphenoidal endoscopic endonasal resection. Histology confirmed the diagnosis of RCC and demonstrated marked degenerative XGCs with squamous metaplasia. The patient tolerated the procedure well with improvement in visual symptoms.

CONCLUSION

RCC with XGC is a very rare pathology, particularly in the pediatric population. These lesions, while benign, can manifest clinically with significant symptoms. While treatment paradigms are not fully established with a small cohort of cases, endoscopic endonasal approaches have made surgical resection of these lesions a safe and effective treatment strategy, even in the pediatric population.

摘要

背景

拉克氏囊肿(RCCs)是一种良性的鞍区病变,通常无症状,在成人中多为偶然发现,在儿科患者(<18岁)中的发病率显著较低。我们报告一例经鼻内镜手术切除治疗的伴有黄色肉芽肿性改变(XGC)的RCC,这是RCC中一种更为罕见的亚型。这是第二例报道的发生在儿科患者中的伴有XGC的RCC。

病例描述

该患者是一名17岁男性,青春期发育延迟,出现双颞侧偏盲,发现有一个2.6厘米的病变,最初认为是颅咽管瘤。随后他顺利接受了经蝶窦鼻内镜切除术。组织学检查确诊为RCC,并显示有明显的退行性XGCs伴鳞状化生。患者对手术耐受良好,视觉症状有所改善。

结论

伴有XGC的RCC是一种非常罕见的病理情况,尤其是在儿科人群中。这些病变虽然是良性的,但可出现明显的临床症状。虽然在少数病例中治疗模式尚未完全确立,但经鼻内镜手术方法已使这些病变的手术切除成为一种安全有效的治疗策略,即使在儿科人群中也是如此。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ffc/7468188/e4c8d0072544/SNI-11-246-g001.jpg

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