多発性内分泌腫瘍症(MEN2B)に伴う口腔粘膜神経腫の1例 A case of neuroma of the oral mucosa associated with multiple endocrine neoplasia (MEN 2 B)

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著者

    • 太田 和俊 OHTA Kazutoshi
    • 熊本大学医学部歯科口腔外科学講座 Department of Dentistry, Oral and Maxillofacial Surgery, Fuculty of Medicine, Kumamoto University
    • 大林 武久 OHBAYASHI Takehisa
    • 熊本大学医学部歯科口腔外科学講座 Department of Dentistry, Oral and Maxillofacial Surgery, Fuculty of Medicine, Kumamoto University
    • 右山 裕則 MIGIYAMA Hironori
    • 熊本大学医学部歯科口腔外科学講座 Department of Dentistry, Oral and Maxillofacial Surgery, Fuculty of Medicine, Kumamoto University
    • 牧 正啓 MAKI Masaaki
    • 熊本大学医学部歯科口腔外科学講座 Department of Dentistry, Oral and Maxillofacial Surgery, Fuculty of Medicine, Kumamoto University
    • 篠原 正徳 SHINOHARA Masanori
    • 熊本大学医学部歯科口腔外科学講座 Department of Dentistry, Oral and Maxillofacial Surgery, Fuculty of Medicine, Kumamoto University

抄録

Multiple endocrine neoplasia (MEN) include three genetically distinct disorders: MEN 1, MEN 2 A, and MEN 2 B. These three syndromes are inherited as autosomal dominant traits, although each may also occur sporadically. MEN 2 B is characterized by medullary thyroid carcinoma, pheochromocytomas, mucosal neuromas, ganglioneuromas of the intestinal tract, and Marfan's syndrome. The case of a 27-year-old man with MEN 2 B is reported. The lips were diffusely enlarged, with distinct projections at the central part of the upper and lower lips caused by neuromatous enlargement. The chief involvement of the tongue affected the dorsum and appeared as multiple nodules of varying size. Biopsies of some nodules of the tongue were obtained. Histological and immunohistochemical studies (HE, S-100 protein and NSE) showed hyperplasia and hypertrophy of nerves, suggesting neuroma. The oral aspects of MEN 2 B are important since they are the initial manifestations of this disorder, preceding thyroid carcinoma by as much as 10 to 20 years. Failure to recognize the syndrome may have unfortunate consequences for the patient, since proper treatment of the thyroid tumor that may arise in childhood will not be provided. Therefore, it is vary important for dentists and physicians who treat oral diseases to carefully examine the oral cavity.

収録刊行物

  • 日本口腔外科学会雑誌

    日本口腔外科学会雑誌 49(4), 276-279, 2003-04-20

    Japanese Society of Oral and Maxillofacial Surgeons

参考文献:  19件中 1-19件 を表示

被引用文献:  1件中 1-1件 を表示

各種コード

  • NII論文ID(NAID)
    10012629123
  • NII書誌ID(NCID)
    AN00189163
  • 本文言語コード
    JPN
  • 資料種別
    NOT
  • ISSN
    00215163
  • データ提供元
    CJP書誌  CJP引用  J-STAGE 
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