Multiple endocrine neoplasia-2B presenting with orthodontic relapse

Abstract

The multiple endocrine neoplasia (MEN) syndromes are a relatively uncommon group of genetic disorders characterized by the development of tumors in various endocrine organs. MEN type 2B is of particular interest to the dental profession because of its oral manifestations, which are often some of the earliest clinically detectable signs of the disorder. Early identification of this syndrome is critical because affected patients often develop a characteristic malignancy, medullary carcinoma of the thyroid, at a very early age. We describe a 17-year-old male whose initial diagnosis of MEN-2B was triggered by his orthodontist's request for an oral and maxillofacial pathology consultation to evaluate the patient's oral abnormalities.

Figure 1

Prominent thickening and fullness of the patient's lips are evident.

Figure 2

Mucosal neuromas are seen on the margins of the eyelids.

Figure 3

Multiple sessile papules that histopathologically proved to be plexiform neuromas are seen on the lateral borders of the tongue.

Figure 4

Sessile mucosal papules, representing plexiform neuromas, are also noted on the anterior buccal mucosa bilaterally.

Figure 5

Occlusal view (top) and front view (bottom) demonstrating diastemata in maxillary anterior dentition.

Figure 6

Low (left) and high (right) power photomicrograph of plexiform neuroma from the patient's labial commissure.

Figure 7

Patient's panoramic radiograph.