Neural hyperplasia in maxillary bone of multiple endocrine neoplasia type 2B patient |
| |
| Authors: | Usami Yu Takenobu Toshihiko Kurihara Risa Imai Yukihiro Shinohara Shogo Fukuda Yasuo Toyosawa Satoru |
| |
| Affiliation: | aAssistant Professor, Clinical Laboratory, Osaka University Dental Hospital, Osaka, Japan;bClinical Fellow, Department of Clinical Pathology, Kobe City Medical Center General Hospital, Hyogo, Japan;cChief, Department of Oral and Maxillofacial Surgery/Head and Neck Surgery, Kobe City Medical Center General Hospital, Hyogo, Japan;dSenior Resident, Department of Otolaryngology/Head and Neck Surgery, Kobe City Medical Center General Hospital, Hyogo, Japan;eChief, Department of Clinical Pathology, Kobe City Medical Center General Hospital, Hyogo, Japan;fDirector, Department of Otolaryngology/Head and Neck Surgery, Kobe City Medical Center General Hospital, Hyogo, Japan;gAssociate Professor, Clinical Laboratory, Osaka University Dental Hospital, Osaka, Japan;hProfessor, Department of Oral Pathology, Graduate School of Dentistry, Osaka University, Osaka, Japan |
| |
| Abstract: | ![]()
Multiple endocrine neoplasia (MEN) type 2B is the rarest and most aggressive form of MEN syndrome. MEN 2B patients manifest characteristic oral and facial features besides the neural crest cell-derived tumors, including medullary carcinoma, pheochromocytoma, mucosal neuroma, and ganglioneuromatosis of the gut. We report a case of MEN 2B diagnosed on the basis of the warning signs of mucosal neuroma and multiple neural hyperplasias in the maxillary bone resected during orthognathic surgery. A subsequent systemic examination under the pathologic diagnosis of neural lesions revealed medullary thyroid carcinoma, megacolon, thickened corneal nerves, and RET gene mutation, thus verifying the diagnosis of MEN 2B. An immunohistochemical study revealed an increased number of unmyelinated Schwann cells in the hyperplastic nerves. We suggest that intraosseous neural hyperplasia is a specific finding of the MEN 2B syndrome in addition to the known oral and facial manifestations. |
| |
| Keywords: | |
| 本文献已被 ScienceDirect PubMed 等数据库收录！ |
|
