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丛状神经纤维瘤的困惑:两个丛状神经纤维瘤的故事

Plexiform's perplexities: a tale of two plexiform neurofibromas.

作者信息

Pedaprolu Aditya Sriharsha, Gattani Rajesh, Jajoo Suhas, Rewale Venkatesh, Deshpande Swati, Chatterjee Priya, Semy Mehak Fayyaz

机构信息

Department of General Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Sawangi, Wardha, Maharashtra 442001, India.

Department of Pathology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Sawangi, Wardha, Maharashtra 442001, India.

出版信息

J Surg Case Rep. 2024 Aug 15;2024(8):rjae486. doi: 10.1093/jscr/rjae486. eCollection 2024 Aug.

DOI:10.1093/jscr/rjae486
PMID:39149528
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11324253/
Abstract

Plexiform neurofibroma (PF) is a rare benign variant belonging to a subtype of neurofibromatosis type 1 that forms bulging or deforming masses arising from the peripheral nerve sheath. These masses involve surrounding connective tissue or dermal layers, leading to multiple cutaneous changes and certain characteristic appearances. It is these appearances that aid in the diagnosis of PF. We have encountered two distinct patients diagnosed with this disorder. While one patient was clinically and pathologically confirmed for PF, the other had no characteristic cutaneous changes. The diagnosis was made with postoperative histopathology and confirmed with an immunohistochemical examination. There are various modalities in the management of PFs, with surgery being a mainstay in the treatment of disfiguring large PFs, especially in resource-restrained settings. In view of high recurrence rates, postoperative clinical follow-up is a must. This paper describes these patients' typical and atypical clinical presentation and subsequent management.

摘要

丛状神经纤维瘤(PF)是一种罕见的良性病变,属于1型神经纤维瘤病的一个亚型,它会形成源于周围神经鞘的隆起或变形肿块。这些肿块累及周围结缔组织或真皮层,导致多种皮肤改变和某些特征性表现。正是这些表现有助于PF的诊断。我们遇到了两名被诊断患有这种疾病的不同患者。其中一名患者在临床和病理上被确诊为PF,而另一名患者没有特征性的皮肤改变。诊断通过术后组织病理学做出,并通过免疫组化检查得以证实。PF的治疗有多种方式,手术是治疗造成容貌损毁的大型PF的主要手段,尤其是在资源有限的环境中。鉴于高复发率,术后临床随访是必不可少的。本文描述了这些患者典型和非典型的临床表现及后续治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b6b/11324253/e36886f923fb/rjae486f8.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b6b/11324253/742c8c45e145/rjae486f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b6b/11324253/cfc6baede68b/rjae486f7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b6b/11324253/e36886f923fb/rjae486f8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b6b/11324253/f1ee1a42bd9b/rjae486f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b6b/11324253/93ec62b68ccb/rjae486f2.jpg
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Ann Med Surg (Lond). 2020 Aug 14;57:346-350. doi: 10.1016/j.amsu.2020.08.015. eCollection 2020 Sep.
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