Neural hyperplasia in maxillary bone of multiple endocrine neoplasia type 2B patient

Cowden syndrome—a case report emphasizing the role of the dental surgeon in diagnosis

“…The multiple endocrinal neoplasia type 2B also is characterized by multiple oral papules and high prevalence of thyroid carcinomas; however, those papules characterize neuromas histopathologicaly, not epithelial hyperplasia, as in CS. Moreover, this condition presents other skeletal characteristics, and the manifestations in head and neck include marfanoid facies, the presence of neuromas on the eyelids (which causes eversion), and swollen lips …”

Multiple Endocrine Neoplasia Type 2B: Maxillofacial Significance in 5 Cases

“…Skeletal apertognathia associated with mandibular prognathia or retrognathia requiring orthodontic treatment, preferably combined with orthognathic surgery, has been reported often. 13,23,24,26,27,30 Edwards and Reid 24 described mandibular prognathia being evident in a 3-year-old child. Usami et al 26 described ' 'multiple intraosseous neural hyperplastic lesions' ' discovered incidentally at the time of elective maxillary osteotomy in a patient with MEN2B.…”

“…[24][25][26][27]29,32 Although true, or at least relative, macroglossia can be part of the syndrome, 29 there is no written support for tongue lesions playing a role in diastemas. Nonetheless, Accurso et al 25 recorded an instance in which orthodontic treatment failed owing to intractable diastemas after orthodontic treatment; the authors also have seen this (see below).…”

“…Several researchers have noted dental imaging irregularities, 26,29,32 most specifically widening of the inferior alveolar canal, blunting of permanent tooth roots, and delayed permanent tooth eruption; however, there have been no reports of directly related tooth loss.…”