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手掌大鱼际区大型神经纤维瘤的外科治疗:病例报告

Surgical management of a large neurofibroma in the thenar Region: A case report.

作者信息

Bachkira ELMehdi, Jadib Imad, Errajragi Amine, Ghannam Ahmed, Charafeddine Elkassimi, Messoudi Abdeljebbar, Rafai Mohamed

机构信息

Department of Orthopedics and Trauma-Surgery P32, University Hospital Center IBN Rochd, Casablanca, Morocco.

Department of Orthopedics and Trauma-Surgery P32, University Hospital Center IBN Rochd, Casablanca, Morocco.

出版信息

Int J Surg Case Rep. 2025 Jan;126:110742. doi: 10.1016/j.ijscr.2024.110742. Epub 2024 Dec 17.

DOI:10.1016/j.ijscr.2024.110742
PMID:39731804
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11743830/
Abstract

INTRODUCTION

Neurofibromas are rare benign tumors of peripheral nerve sheaths, and hand involvement is particularly uncommon. This case report presents a large neurofibroma located in the thenar region, a critical area for thumb opposition and hand dexterity, posing unique surgical challenges.

PRESENTATION OF CASE

A 23-year-old female presented with a 3-year history of a progressively enlarging mass in the thenar region of the right hand, accompanied by nocturnal pain but no neurological deficits. MRI revealed a well-circumscribed, multilobulated tumor measuring 54 × 55 × 33.4 mm. Surgical excision was successfully performed while preserving the median nerve. Postoperative recovery was assessed using the QuickDASH questionnaire, yielding a score of 6, indicative of excellent functional outcomes. Histopathology confirmed a benign neurofibroma.

CLINICAL DISCUSSION

The large size and rare location of this tumor, combined with the absence of neurofibromatosis type 1, make this case noteworthy. Early diagnosis and meticulous surgical planning enabled complete excision while preserving critical structures, resulting in full functional recovery with no recurrence after 18 months.

CONCLUSION

This case underscores the importance of individualized surgical strategies for managing neurofibromas in functionally critical areas. While excellent long-term outcomes were achieved in this instance, further studies are needed to validate these findings and refine management approaches.

摘要

引言

神经纤维瘤是周围神经鞘的罕见良性肿瘤,手部受累尤其少见。本病例报告介绍了一例位于大鱼际区域的大型神经纤维瘤,该区域对于拇指对掌和手部灵活性至关重要,给手术带来了独特挑战。

病例介绍

一名23岁女性,右手大鱼际区域有一逐渐增大的肿块,病史3年,伴有夜间疼痛,但无神经功能缺损。磁共振成像(MRI)显示一个边界清晰、多叶状的肿瘤,大小为54×55×33.4毫米。手术成功切除肿瘤,同时保留了正中神经。使用QuickDASH问卷评估术后恢复情况,得分为6分,表明功能预后良好。组织病理学证实为良性神经纤维瘤。

临床讨论

该肿瘤体积大且位置罕见,加上患者无1型神经纤维瘤病,使得本病例值得关注。早期诊断和精心的手术规划使得肿瘤得以完整切除,同时保留了关键结构,术后18个月功能完全恢复且无复发。

结论

本病例强调了针对功能关键区域神经纤维瘤的个体化手术策略的重要性。虽然本例取得了良好的长期效果,但仍需进一步研究来验证这些发现并完善治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/9c28863bd809/gr8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/530e4c904f2c/gr1.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/65d8a090048d/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/0f6ede02d572/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/9a44337ea122/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/935a93b54654/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/9f3fc1a3bdcb/gr7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/9c28863bd809/gr8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/530e4c904f2c/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/d364bbb845a4/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/65d8a090048d/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/0f6ede02d572/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/9a44337ea122/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/935a93b54654/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/9f3fc1a3bdcb/gr7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee19/11743830/9c28863bd809/gr8.jpg

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本文引用的文献

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Atypical presentation of long-standing hand neurofibroma: A case report.长期存在的手部神经纤维瘤的非典型表现:一例报告。
Int J Surg Case Rep. 2024 Nov;124:110474. doi: 10.1016/j.ijscr.2024.110474. Epub 2024 Oct 16.
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The SCARE 2023 guideline: updating consensus Surgical CAse REport (SCARE) guidelines.SCARE 2023 指南:更新共识外科病例报告(SCARE)指南。
Int J Surg. 2023 May 1;109(5):1136-1140. doi: 10.1097/JS9.0000000000000373.
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Histopathological Variants of Cutaneous Neurofibroma: A Compendious Review.皮肤神经纤维瘤的组织病理学变异:简要综述
Dermatopathology (Basel). 2022 Dec 26;10(1):1-19. doi: 10.3390/dermatopathology10010001.
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Atypically Located Solitary Neurofibroma of the Ulnar Nerve as Inaugural Presentation of a New Genetic Variant of Neurofibromatosis Type 1: A Case Report.尺神经非典型部位孤立性神经纤维瘤作为1型神经纤维瘤病新基因变异的首发表现:一例报告
J Hand Surg Glob Online. 2022 May 20;4(5):283-287. doi: 10.1016/j.jhsg.2022.04.008. eCollection 2022 Sep.
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Medicine (Baltimore). 2021 Jan 15;100(2):e23611. doi: 10.1097/MD.0000000000023611.
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