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小儿骶尾部椎管外室管膜瘤的意外诊断:经验教训及文献综述

An unexpected diagnosis of paediatric subcutaneous sacrococcygeal extraspinal ependymoma: lessons learnt and review of the literature.

作者信息

Gupta Tanya, Patel Vikesh, El-Medani Faisal, Gupta Sanjay

机构信息

Department of General Surgery, Lister Hospital, Stevenage, UK.

出版信息

J Surg Case Rep. 2020 Jul 8;2020(7):rjaa177. doi: 10.1093/jscr/rjaa177. eCollection 2020 Jul.

DOI:10.1093/jscr/rjaa177
PMID:32665839
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7345838/
Abstract

This case describes a 9-year-old male who presented to general surgical clinic with a 3-year history of persistent natal cleft swelling, previously unsuccessfully treated as a pilonidal abscess in the community with multiple courses of antibiotics. In clinic, a 50 × 30-mm soft tissue swelling was found in the natal cleft and a clinical diagnosis of a pilonidal cyst was made. A cream-coloured solid mass measuring 50 × 35 × 30 mm was subsequently excised under general anaesthetic, with specialist histology and immunostaining confirming an unexpected diagnosis of a subcutaneous extraspinal myxopapillary ependymoma, a tumour usually found in the neuraxis. Given the atypical anatomical site of the tumour, the case presented a unique management challenge. Ultimately, the patient underwent a re-operation after specialist multi-disciplinary discussion and is currently disease free at 18 months post-surgery. The authors wish to contribute their experiences of managing this rare extraspinal ependymoma to the few existing reports in the literature.

摘要

本病例描述了一名9岁男性,因先天性臀裂持续肿胀3年就诊于普通外科门诊。该患者此前在社区被当作藏毛窦脓肿治疗,接受了多疗程抗生素治疗,但未成功。在门诊中,发现先天性臀裂处有一个50×30毫米的软组织肿胀,临床诊断为藏毛窦囊肿。随后在全身麻醉下切除了一个奶油色实性肿块,大小为50×35×30毫米,专业组织学和免疫染色证实了一个意外的诊断:皮下椎管外黏液乳头型室管膜瘤,这是一种通常发生于神经轴的肿瘤。鉴于肿瘤的非典型解剖位置,该病例带来了独特的管理挑战。最终,患者在经过多学科专家讨论后接受了再次手术,目前术后18个月无疾病复发。作者希望将他们处理这种罕见的椎管外室管膜瘤的经验贡献给文献中为数不多的现有报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a7a/7345838/457bcb04e4c1/rjaa177f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a7a/7345838/457bcb04e4c1/rjaa177f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a7a/7345838/457bcb04e4c1/rjaa177f1.jpg

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