多发性内分泌肿瘤 2B 综合征的口腔表现：罕见病例报告

IF 0.9 Q3 DENTISTRY, ORAL SURGERY & MEDICINE

Contemporary Clinical Dentistry Pub Date : 2024-01-01 Epub Date: 2024-03-23 DOI:10.4103/ccd.ccd_215_23

Sohini Banerjee, Debkamal Kar

{"title":"多发性内分泌肿瘤 2B 综合征的口腔表现：罕见病例报告","authors":"Sohini Banerjee, Debkamal Kar","doi":"10.4103/ccd.ccd_215_23","DOIUrl":null,"url":null,"abstract":"Multiple endocrine neoplasia (MEN) 2B syndrome is a rare autosomal dominant hereditary condition of neuroendocrine origin characterized by pheochromocytoma, marfanoid habitus, and mucocutaneous neuromas. Multiple mucosal neuromas on the tongue, lips, cheeks, and inner eyelids are often appeared as the earliest signs in most of the undiagnosed cases. Early diagnoses, recognition of phenotype, adequate surgery, and appropriate genetic counseling are very much essential as patients often develop medullary thyroid carcinoma (MTC) and pheochromocytoma. This case report would highlight the oral manifestations of MEN 2B in a 22-year-old female patient with multiple mucosal neuromas and a previous history of thyroidectomy due to MTC.","PeriodicalId":10632,"journal":{"name":"Contemporary Clinical Dentistry","volume":null,"pages":null},"PeriodicalIF":0.9000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11068242/pdf/","citationCount":"0","resultStr":"{\"title\":\"Oral Manifestations of Multiple Endocrine Neoplasia 2B Syndrome: A Rare Case Report.\",\"authors\":\"Sohini Banerjee, Debkamal Kar\",\"doi\":\"10.4103/ccd.ccd_215_23\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Multiple endocrine neoplasia (MEN) 2B syndrome is a rare autosomal dominant hereditary condition of neuroendocrine origin characterized by pheochromocytoma, marfanoid habitus, and mucocutaneous neuromas. Multiple mucosal neuromas on the tongue, lips, cheeks, and inner eyelids are often appeared as the earliest signs in most of the undiagnosed cases. Early diagnoses, recognition of phenotype, adequate surgery, and appropriate genetic counseling are very much essential as patients often develop medullary thyroid carcinoma (MTC) and pheochromocytoma. This case report would highlight the oral manifestations of MEN 2B in a 22-year-old female patient with multiple mucosal neuromas and a previous history of thyroidectomy due to MTC.\",\"PeriodicalId\":10632,\"journal\":{\"name\":\"Contemporary Clinical Dentistry\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2024-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11068242/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Contemporary Clinical Dentistry\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/ccd.ccd_215_23\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/3/23 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q3\",\"JCRName\":\"DENTISTRY, ORAL SURGERY & MEDICINE\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Contemporary Clinical Dentistry","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/ccd.ccd_215_23","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/3/23 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"DENTISTRY, ORAL SURGERY & MEDICINE","Score":null,"Total":0}

引用次数: 0

摘要

多发性内分泌肿瘤（MEN）2B 综合征是一种罕见的常染色体显性遗传性神经内分泌疾病，以嗜铬细胞瘤、马凡诺型体型和粘膜神经瘤为特征。在大多数未确诊的病例中，舌头、嘴唇、脸颊和眼睑内侧的多发性粘膜神经瘤往往是最早出现的症状。由于患者通常会发展为甲状腺髓样癌和嗜铬细胞瘤，因此早期诊断、识别表型、适当手术和适当的遗传咨询非常重要。本病例报告将重点介绍一名22岁女性患者的MEN 2B口腔表现，该患者患有多发性粘膜神经瘤，既往曾因MTC而进行过甲状腺切除术。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

Oral Manifestations of Multiple Endocrine Neoplasia 2B Syndrome: A Rare Case Report.

Multiple endocrine neoplasia (MEN) 2B syndrome is a rare autosomal dominant hereditary condition of neuroendocrine origin characterized by pheochromocytoma, marfanoid habitus, and mucocutaneous neuromas. Multiple mucosal neuromas on the tongue, lips, cheeks, and inner eyelids are often appeared as the earliest signs in most of the undiagnosed cases. Early diagnoses, recognition of phenotype, adequate surgery, and appropriate genetic counseling are very much essential as patients often develop medullary thyroid carcinoma (MTC) and pheochromocytoma. This case report would highlight the oral manifestations of MEN 2B in a 22-year-old female patient with multiple mucosal neuromas and a previous history of thyroidectomy due to MTC.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

Contemporary Clinical Dentistry DENTISTRY, ORAL SURGERY & MEDICINE-

CiteScore

1.50

自引率

0.00%

发文量

审稿时长

23 weeks

期刊介绍： The journal Contemporary Clinical Dentistry (CCD) (Print ISSN: 0976-237X, E-ISSN:0976- 2361) is peer-reviewed journal published on behalf of Maharishi Markandeshwar University and issues are published quarterly in the last week of March, June, September and December. The Journal publishes Original research papers, clinical studies, case series strictly of clinical interest. Manuscripts are invited from all specialties of Dentistry i.e. Conservative dentistry and Endodontics, Dentofacial orthopedics and Orthodontics, Oral medicine and Radiology, Oral pathology, Oral surgery, Orodental diseases, Pediatric Dentistry, Periodontics, Clinical aspects of Public Health dentistry and Prosthodontics. Review articles are not accepted. Review, if published, will only be by invitation from eminent scholars and academicians of National and International repute in the field of Medical/Dental education.