恶性周围神经鞘肿瘤的鼻腔:罕见的诊断和治疗的挑战。

IF 0.4 Q4 SURGERY
Journal of Surgical Case Reports Pub Date : 2025-05-30 eCollection Date: 2025-05-01 DOI:10.1093/jscr/rjaf349
Amine Oussalem, Anas Azgaoui, Bouchra Dani, Malik Boulaadas
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引用次数: 0

摘要

恶性周围神经鞘肿瘤(MPNSTs)是一种罕见的侵袭性肉瘤,常累及头颈部,在1型神经纤维瘤病中发病率较高。早期诊断对预防复发和转移至关重要。我们报告一位78岁男性病患,右鼻腔肿块迅速扩大超过4个月,并伴有复发性鼻出血。他的病史包括糖尿病、高血压和吸烟。检查发现一个坚固的非粘附性肿块阻塞鼻腔。计算机断层扫描(CT)成像定位于右下鼻甲。由于其大小和位置,未行活检。手术切除证实为高度MPNST (FNCLCC 3级)。术后开始化疗,但患者很快就去世了,这突出了肿瘤的侵袭性。在鼻肿块鉴别中应考虑mpnst,尤其是老年吸烟者。早期手术和化疗可能改善预后,强调需要多学科方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Malignant peripheral nerve sheath tumor of the nasal cavity: a rare diagnostic and therapeutic challenge.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive sarcomas, often affecting the head and neck, with higher incidence in neurofibromatosis type 1. Early diagnosis is crucial to prevent recurrence and metastasis. We report a 78-year-old male with a rapidly enlarging right nasal cavity mass over 4 months, associated with recurrent epistaxis. His medical history included diabetes, hypertension, and tobacco use. Examination revealed a firm, non-adherent mass obstructing the nasal cavity. Computed tomography (CT) imaging localized it to the right inferior turbinate. Due to size and location, biopsy was not performed. Surgical resection confirmed a high-grade MPNST (FNCLCC grade 3). Chemotherapy was initiated postoperatively, but the patient passed away soon after, highlighting the tumor's aggressiveness. MPNSTs should be considered in nasal mass differentials, especially in elderly smokers. Early surgery and chemotherapy may improve outcomes, emphasizing the need for a multidisciplinary approach.

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来源期刊
CiteScore
0.70
自引率
0.00%
发文量
559
审稿时长
11 weeks
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