A rare case of multiple congenital epulis

Abstract

We report a case in a female newborn infant of multiple congenital epulis, i.e. granular cell tumor, that was undetected during regular pregnancy ultrasound monitoring. At birth the neoplasms appeared as two voluminous lesions protruding from the newborn's mouth. The greater of them (5.5 cm × 4 cm × 3 cm) was pedunculated and attached to the external superior gingiva, shifting the alae nasi and making it difficult to enter the coanae. The second mass was somewhat smaller (3 cm × 4 cm × 2.5 cm), pedunculated and attached to the external inferior gum. A third smaller mass was less evident, unpedunculated and attached to the rim of the lower gingiva. Histologically the lesions were characterized by large cells, which had abundant pale acidophilic granular cytoplasm. A round-oval nucleus was located centrally. The cell membranes were distinct. Neither mitosis nor necrosis was found. Staining for cytoplasmic granules was intensely periodic acid-Schiff (PAS) positive and diastase resistant. Immunohistochemical negativity for S100 protein, positivity for lysozyme and numerous phagolysosomes in the cytoplasm of neoplastic elements, observed on ultrastructural examination, supported the hypothesis that the congenital type of granular cell tumor cannot have a Schwannian origin like that of the adult type, but is probably a mesenchymal lesion which, for unknown cause, regresses by a degenerative process.

• CONGENITAL EPULIS
• GRANULAR CELL TUMOR
• PRENATAL DIAGNOSIS