无2B型多发性内分泌肿瘤的牙龈孤立性粘膜神经瘤:一例罕见病例报告和文献复习

C. Qiu, Lizhen Wang, Huiwen Chen, Zhongchen Song
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引用次数: 0

摘要

粘膜神经瘤(MN)是周围神经的一种良性神经肿瘤,组织学特征为不规则扭曲的神经细胞束,神经周围膜突出,分散分布在粘膜下层。该肿瘤通常与多发性内分泌瘤2B型(MEN 2B)相关,但很少发生无MEN 2B其他成分的肿瘤。一名29岁女性,因上前牙牙龈无痛性肿块就诊2年。口腔内检查发现在上颌中切牙之间的唇龈乳头上有一个小的、坚韧的、基部宽的、界限清楚的肿块。切除活检示HE染色粘膜下层可见大小不等的神经束,周围为正常结缔组织;免疫组化结果显示S-100蛋白、NSE、NF呈强阳性,EMA呈弱阳性。实验室检查和超声检查未见MEN 2B征象。在第一个和第二个6个月的随访中,没有复发和其他MEN 2B成分的证据。患者无症状,仍在每6个月随访一次。基于这些特征,最终诊断为单纯性牙龈MN。综上所述,本研究发现一罕见的龈乳头单发MN,未见MEN 2B的报道。组织病理学评价有助于MN的鉴别诊断。我们希望通过对口腔内无MEN 2B的孤立性MN的更深入的了解,避免潜在的误诊,并有助于确定正确的治疗方法,即完全手术切除,密切随访复发和其他MEN 2B监测组成部分。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Solitary mucosal neuroma of the gingiva without multiple endocrine neoplasia type 2B: a rare case report and literature review
Mucosal neuroma (MN) is a benign neural tumor of peripheral nerves histologically characterized by irregular tortuous bundles of nerve cells with prominent perineurium that lie scattered throughout the submucosa. The tumor is usually associated with the multiple endocrine neoplasia type 2B (MEN 2B) but rarely occurs without the other components of MEN 2B. A 29-year-old woman presented to our outpatient department with an upper anterior teeth gingiva painless mass for 2-year. Intraoral examination revealed a small, tough, basal wide, well-defined mass in the labial gingival papilla between maxillary central incisor. Excisional biopsy showed that it was characterized by nerve bundles in various sizes surrounded by normal connective tissue in the submucosa under HE staining; Immunohistochemically, the mass showed strongly positive staining of S-100 protein, NSE, NF and weakly positive of EMA. The Laboratory examination and ultrasonography showed no signs of MEN 2B. In the first and second 6 months of follow-up, there was no evidence of recurrence and other components of MEN 2B. The patient was asymptomatic and she is still being followed up every 6 months. Based on these features, the lesion was finally diagnosed as a solitary gingival MN. In summary, this study showed a rare solitary MN in the gingiva papilla without MEN 2B that has not been reported yet. The histopathological evaluation can be helpful in the differential diagnosis of MN. It is hoped that a greater understanding of solitary MN without MEN 2B in the oral cavity will avoid potential misdiagnosis, and contribute to determining the correct management, which appears to be complete surgical excision with close follow-up for recurrence and other components of MEN 2B surveillance.
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