| Abstract|| |
Congenital epulis or neonatal granular cell tumor is a tumor whose histogenesis has long been debated. It is dissimilar to other granular cell tumors; the latter having derivation from Schwann cells and hence being S-100 positive. We report a case of congenital granular cell tumor in a newborn female child that was uneventfully operated. The case is reported here because of its rarity.
Keywords: Congenital epulis, congenital granular cell tumor, granular cell tumor
|How to cite this article:|
Dhingra M, Pantola C, Agarwal A. Congenital granular cell tumor of the alveolar ridge. Indian J Pathol Microbiol 2010;53:327-8
| Introduction|| |
Granular cell tumor is a fairly common entity in adults. The behavior is mostly benign with the malignant counterpart accounting for less than 2% of all cases. However, a malignant counterpart is unknown in the congenital form.
| Case Report|| |
A newborn girl child (born at 35th week of gestation) was referred to pediatric surgery immediately after birth because of a mass protruding from the upper gingiva. The mass measured 5 x 4 x 4 cm [Figure 1], attached to the alveolar ridge by a broad stalk measuring 3 x 0.8 cm, because of which mouth could not be closed and feeding was not possible. Respiration was normal. The antenatal history of the mother was unremarkable. Ultrasonography at 24th week of gestation was normal. In view of feeding problems, immediate surgery was planned. The child was operated on the fourth postnatal day. The mass was removed and submitted for histopathological evaluation.
A partially skin covered firm nodular mass was received. External surface was smooth with lobulated appearance. It was encapsulated without any breech. Cut surface was homogenous gray-white. Multiple sections were studied after routine paraffin embedding and hematoxylin and eosin staining. Microscopic examination revealed a well circumscribed tumor lying beneath unremarkable stratified squamous epithelium [Figure 2]. There were solid sheets of monotonous round-to-polygonal cells. The cells had centrally placed nucleus with condensed chromatin and inconspicuous nucleoli. Cytoplasm in all cells was abundant and brightly eosinophilic [Figure 3]. The tumor was moderately vascular. There was no necrosis or mitoses. Considering the possibility of congenital granular cell tumor (GCT), further histochemical and immunohistochemical evaluation was done. There was intense diastase resistant periodic acid Schiff (PAS) positivity. Immunohistochemically, the tumor cells were positive for vimentin but negative for S100 [Figure 4], desmin and actin.
| Discussion|| |
Congenital granular cell tumor (CGCT), also known as congenital epulis or Neumann's tumor, occurs typically in newborns. The female : male ratio is 8 : 1.  Though morphologically indistinguishable, its histogenesis is different from adult granular cell tumors. The tumor arises mostly from mucosa over the alveolar ridge, thus interfering with mouth closure and feeding. When multiple, the tumor may cause respiratory obstruction. 
Congenital epulis bears a striking resemblance to adult GCT on light microscopy. It typically presents in newborn females and the characteristic site provides diagnostic clue. However, unlike the adult GCT (myoblastoma), the neonatal counterpart does not have Schwannian origin. This is reflected by its S100 negativity. Various theories have been put forward to explain the controversial origin of CGCT. Most favored theories are gingival epithelial and odontogenic epithelial theories, which support its origin from the mesenchyme. ,
Arrangement of cells in GCT may be as ribbons, nests or as patternless diffuse sheets as seen in our case. Overlying epithelium may be unremarkable or show acanthosis or pseudoepitheliomatous hyperplasia. Mild to moderate nuclear atypia may occur but does not represent malignancy, which is decided by hyperchromasia, vesicular nuclei with prominent nucleoli, necrosis and mitosis >10/HPF. A variety of soft tissue tumors can have similar histological features. Coarse cytoplasmic granularity and absence of lipid droplets help to distinguish it from rhabdomyoma and hibernoma. The CGCTs are S100 negative and vimentin positive. Further, they are negative for NSE, actin, desmin, laminin and keratin. Most of the tumor cells have reactivity for CD68. Most of the cases reported are benign. They do not metastasize. A malignant counterpart of CGCT is unknown. Usual treatment is complete surgical excision.
| References|| |
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|2.||Parmigiani S, Giordano G, Fellegara G, Brevi B, Magnani C J, Matern F, et al. Neonatal Med 2004;16:55-8. |
|3.||Lack EE, Perez-Atayde AR, McGill TJ, Vawter GF. Gingival granular cell tumor of the newborn (congenital epulis): ultrastructural observations leading to histogenesis. Hum Pathol 1982;13:686-9. [PUBMED] |
|4.||Gunhan O, Gunhan M, Cetinkursun S, Celasun B. Congenital gingival granular cell tumor. A case report. J Nihon Univ Sch Dent 1993;35:130-3. |
Department of Pathology, Nizamís Institute of Medical Sciences, Punjagutta, Hyderabad
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]