Multiple Congenital Granular Cell Epulis: Case Report and Immunohistochemical Profile with Emphasis on Vascularization

Abstract

Congenital granular cell epulis is a rare benign soft tissue lesion arising from the alveolar ridge in neonates. A rare case of multiple congenital granular cell epulis is reported, alongside a description of its vascular immunohistochemical profile. A female newborn presented with two exophytic pedunculated red nodules located on the alveolar ridge between the future eruption sites of the incisors and canines of the mandible and maxilla. A conservative surgical excision was performed on the second day of life. Histology revealed proliferation of round granular cells containing an abundant eosinophilic cytoplasm with basophilic nuclei, ranging from round to oval in shape. Numerous blood vessels were also seen. Immunohistochemical analysis of the granular cells revealed positivity for CD68, D2-40, Ki67, VEGF, and FGF and negativity for S100, CD34, and CD105. Immunostaining for CD34, CD105, and D2-40 confirmed the presence of a large number of blood and lymphatic vessels. Although rare, an understanding of this lesion is paramount for correct diagnosis and appropriate treatment. In the present report, the immunohistochemical profile confirmed increased vascularization, proving that these lesions are composed of not only new and preexisting blood vessels, but also lymphatic vessels.

Congenital Granular Cell Tumor: Case Report and Review

“…Grossly, CGCT appears well developed in the newborn as a variably sized soft tissue mass, with a tan, pink, or red coloration, and an irregular, lobulated, and/or smooth surface, typically arising from the alveolar ridge [ 2 , 6 , 7 ]. Much emphasis of study has been made on pathological evaluation.…”

“…A multitude of immunoreactive studies may be performed including S-100, CD34, CD68, CD105, and many others. S-100 remains one of the most important immunohistochemical evaluations, particularly as adult granular cell tumor, and CGCT may grossly be difficult to distinguish, granted with different clinical presentations of patient age, staining positive in adult form and negative in CGCT [ 6 ]. While CGCT tends to be diagnosed upon delivery, documentation in the literature has shown diagnosis of larger lesions on prenatal ultrasonography during the third trimester as well as associated polyhydramnios secondary to poor swallowing en utero [ 5 ].…”

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

“…12 Vascular channels vary between granular cells, but fibrous stroma is minimally conspicuous. 13 Unlike the granular cell tumor (myoblastoma), the atrophic overlying epithelium never demonstrates the pseudoepitheliomatous hyperplasia. Granularity of the lesion is observed in other neoplastic maxillofacial or odontogenic lesions including granular ameloblastoma, verruciform xanthoma and others.…”

“…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 Granular Cell Tumour : A Rare Case Repor

“…On immunohistochemistry the adult granular cell tumor shows positivity for S100, laminin, nerve growth factor receptor/p75, smooth muscle actin. CGCE (Congenital granular cell epulis) shows positivity 17,18,19 for only vimentin, neuron specic enolase.…”