The authors report a case of a gingival granular cell tumor, also called "congenital epulis", occurring in a female newborn. It is a rare congenital, benign tumor of uncertain histogenesis, with marked predilection for female newborn. The tumor arises, as a polypoid mass, on the gingiva of the anterior maxillary or mandibular alveolar ridge. Typical histologic features are represented by sheets of large cells with pale granular cytoplasm. Despite remarkable similarities between gingival granular cell tumors and others ubiquitous granular cell tumors (granular cell myoblastoma or Abrikossoff's tumor), histogenesis of the gingival granular cell tumors seems different. Unlike those of granular cell myoblastoma, granular cells of gingival granular cell tumor do not express S-100 protein and Neuron-Specific-Enolase. Considering the positivity for oestrogen and progesterone receptors, a hormonal factor may be important in the development of gingival granular cell tumor. The generally accepted treatment is simple excision.
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