Congenital granular cell epulis presents an immunohistochemical profile that distinguishes it from the granular cell tumor of the adult

Abstract

Congenital granular cell epulis (CGCE), a rare benign lesion arising from the mucosa of the alveolar ridges of the jaws in newborns, has a clinical course characterized by lack of further growth after birth. Histomorphologically, it resembles a granular cell tumor (GCT) of the adult. The histogenesis of this lesion is unclear. We submitted a series of five CGCEs to a large panel of antibodies in order to trace the origin of the constituent granular cells. The resultant immunohistochemical profile showed positivity of these cells to vimentin, NKI/C3, and PGP9.5. This does not confirm any particular cell type for the histogenetic origin of CGCE but may rather reflect a local metabolic or reactive change, providing supporting evidence that the lesion is of a non-neoplastic nature. In addition, the granular cells were non-reactive for S-100, NGFR/p75, and inhibin-alpha, which further contributes to the distinction between a CGCE and the adult GCT.

Diagnosis and management of congenital epulis of new-born: a rare case report and literature review

“…6 Concerning its uncertain histogenesis, odontogenic epithelium, fibroblasts, 7 Langerhans cells or histiocytes, 8 smooth muscle, nerve-related cells, 9 endothelial cells, pericytes, myofibroblasts, and undifferentiated mesenchymal cells 10 have been implicated in the pathogenesis of CEN, which, however, remains undefined. 11 Microscopically, CEN is composed of sheets of large to medium-sized, round to polyhedral cells that have a discrete cellular membrane, abundant granular cytoplasm, and a single darkly staining nucleus with an eccentrically positioned nucleolus. The covering epithelium is thin and attenuated.…”

“…Such cells are, moreover, strongly positive for acid phosphatase. [9][10][11]13 Slootweg et al 14 concluded that peanut lectin binding protein might be considered a common feature of granular cells regardless of their histogenesis. Lysosomes are supposed to represent the intracellular binding sites for this marker.…”

Congenital epulis: a report of two cases and review of the literature

“…It has been reported to occur primarily in neonates, and more in females. [9] This suggests a hormonal mechanism in the aetiology. Some studies have shown no evidence of either oestrogen or progesterone receptors, and as such suggest an alternative histogenesis.…”

“…Some studies have shown no evidence of either oestrogen or progesterone receptors, and as such suggest an alternative histogenesis. [6,7] Vered et al [9] in their review of 33 lesions concluded that the immunohistochemical profile does not implicate any specific cell types for the histogenetic origin of the benign lesion. There has not been any reported case of recurrence after excision.…”

“…[8] MRI shows the gingival origin of the lesion without local extension. [9] Management is with surgical excision, although spontaneous regression has been documented. [10] The two cases reported were surgically excised.…”