The congenital gingival granular cell tumor (CGGT) or congenital epulis is a rare lesion of unknown origin found only in newborn infants. The tumor consists mainly of large eosinophilic granular cells arranged in solid nests that are separated by thin fibrovascular areas. In addition, there are some spindle-shaped cells and medium-sized polygonal cells (so-called interstitial cells) among the neoplastic granular cells. Three CGGTs were investigated with a panel of poly- and monoclonal antibodies, using immunoperoxidase methods on formalin fixed paraffin embedded sections. Neoplastic granular cells of these three cases show cytoplasmic staining for neuron-specific enolase (NSE) and vimentin. However, all other reactions were negative. Our results suggest that the lesion may be derived from uncommitted nerve-related mesenchymal cells. On the other hand, interstitial cells show strong S-100 protein-, cytokeratin-, vimentin-, and NSE-immunostainings, and these cells are consistent with neuroendocrine nature. The presence of a biphasic cell population with granular cells and interstitial cells must be considered the main immunohistochemical feature.

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.

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