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丛状神经纤维瘤简要报告

A brief report of plexiform neurofibroma.

作者信息

Khajavi Mahdi, Khoshsirat Shahrokh, Ahangarnazari Lida, Majdinasab Niloofar

机构信息

Hearing Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Hearing Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

出版信息

Curr Probl Cancer. 2018 Mar-Apr;42(2):256-260. doi: 10.1016/j.currproblcancer.2018.01.007. Epub 2018 Jan 10.

DOI:10.1016/j.currproblcancer.2018.01.007
PMID:29449010
Abstract

Plexiform neurofibroma (PNF) is a rare variant of neurofibromatosis type1 (NF-1), which histopathologically, is a subtype of benign nerve sheath tumors, neurofibromas (NF). It develops as a result of proliferation in all parts of peripheral nervous system and can cause the functional damage, deformities, pain, considerable mortality, and morbidity and even the increasing risk of malignant transformation in some critical cases. Currently, the surgical intervention is the treatment of choice for PNF patients, which due to the tumor invasion, massive growth, and the chance of postoperative regrowth is not possible. The diagnosis of isolated tumor is an uncommon event. Considering the rarity of this neoplasm, herein, we describe a case of isolated PNF, so the purpose of this presenting is the rarity of recording. We describe a case of isolated plexiform neurofibroma presented with 7-year history of a slowly growing postauricular soft subcutaneous mass in a 14-year-old boy, which caused the right auricular deformity. After initial evaluation by imaging studies, the patient underwent to surgical resection of the mass and the diagnosis of plexiform neurofibroma was confirmed by histopathologic examination. Surgical excision of the mass had been done before which concluded the satisfactory result and based on oncologist diagnosis, further intervention such as radiotherapy or chemotherapy was not needed. The patient left the hospital with a clinical stability and was suggested to continue the regular follow-up. In conclusion, considering neurofibroma (NF) as differential diagnosis for subcutaneous masses in head and neck area is critical for early diagnosis and treatment procedure.

摘要

丛状神经纤维瘤(PNF)是1型神经纤维瘤病(NF-1)的一种罕见变体,在组织病理学上,它是良性神经鞘瘤——神经纤维瘤(NF)的一种亚型。它是由于外周神经系统各部位的增殖而发展形成的,可导致功能损害、畸形、疼痛、相当高的死亡率和发病率,在某些严重情况下甚至会增加恶变风险。目前,手术干预是PNF患者的首选治疗方法,但由于肿瘤侵袭、大量生长以及术后复发的可能性,手术无法进行。孤立性肿瘤的诊断并不常见。鉴于这种肿瘤的罕见性,在此我们描述一例孤立性PNF病例,本病例报告的目的是记录其罕见性。我们描述了一例孤立性丛状神经纤维瘤病例,该病例表现为一名14岁男孩耳后皮下缓慢生长的肿块,病史长达7年,导致右耳畸形。经影像学检查初步评估后,患者接受了肿块切除术,组织病理学检查证实为丛状神经纤维瘤。此前已对肿块进行了手术切除,结果令人满意,根据肿瘤学家的诊断,不需要进一步的放疗或化疗等干预措施。患者临床状况稳定出院,并被建议继续定期随访。总之,将神经纤维瘤(NF)作为头颈部皮下肿块的鉴别诊断对于早期诊断和治疗至关重要。

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