Phenotypic Features of MEN Type IIB

Unlike MEN type IIA and FMTC, in which patients have a normal outward appearance, MEN type IIB is distinguished by characteristic physical features (Figure 17-6). As described earlier, mucosal neuromas and a marfanoid habitus are present. The mucosal neuromas are an unencapsulated, thickened proliferation of nerves that occur principally on the lips and tongue but can also be found on the gingiva, buccal mucosa, nasal mucosa, vocal cords, and con

Figure 17-6. Typical facial features of multiple endocrine neoplasia type IIB patients, with a prominent mid-upper lip, everted lower eyelid, and multiple tongue nodules. Reproduced with permission from Moley JF. American Society of Clinical Oncology's Cancer Genetics and Cancer Predisposition Testing curriculum; 1998.

Figure 17-6. Typical facial features of multiple endocrine neoplasia type IIB patients, with a prominent mid-upper lip, everted lower eyelid, and multiple tongue nodules. Reproduced with permission from Moley JF. American Society of Clinical Oncology's Cancer Genetics and Cancer Predisposition Testing curriculum; 1998.

junctiva. MEN type IIB patients can also develop ganglioneuromas of the intestine in the submucosal and myenteric plexus. Intestinal dysfunction from ganglioneuromatosis may manifest early in life with poor feeding, failure to thrive, constipation, or pseudo-obstruction. Adults with this disorder may have dysphagia from esophageal dysmotility. Rarely, a patient can present with toxic megacolon. Neonates and infants with severe intestinal gan-glioneuromatosis have a clinical course similar to that of Hirschsprung's disease.

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