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granular cell epulis

Thursday 26 May 2011

gingival granular cell tumor; granular cell congenital epulis; congenital granular cell myoblastoma, granular cell epulis of infancy, granular cell fibroblastoma

Digital cases

- HPC:63 : Gingival granular cell tumor (Neonatal epulis)

Definition: The congenital granular cell epulis is a unique and rare congenital tumor of the alveolar mucosa of the jaws. The exact nature of this entity is not clear.

Clinical Features

The congenital epulis is almost exclusively found on the anterior alveolar ridges of newborns, although a few cases have reportedly developed shortly after birth.

Approximately 90% of cases occur in girls and 10% present with multiple lesions. It presents as a 0.5-2.0 cm. soft, pedunculated and perhaps lobulated nodule of the alveolar mucosa, especially the mucosa of the maxilla.

A few lesions have been as much as 9 cm. in size at birth and several cases have had involvement of both jaws.

The earliest reported case was identified by ultrasound in a 31 week fetus.

There is no tenderness or surface change and the lesion does not increase in size after birth.

In fact, many of the smaller examples will spontaneously regress after birth.

Pathology

The mucosal mass is comprised almost entirely of large, rounded and polyhedral, histiocyte-like cells with small, dark oval nuclei and abundant eosinophilic granular cytoplasm.

Lesional cells are usually rounded but may be somewhat spindled.

There are vascular channels between granular cells, but fibrous stroma is minimally present and often appears to be completely lacking.

The tumor cells extend to the overlying epithelium, which is atrophic and never demonstrates the pseudoepitheliomatous hyperplasia so commonly seen in the granular cell tumor of adults.

Immunochemistry

Lesional cells do not immunoreact for laminin or S-100 protein, as do the granular cells of the granular cell tumor.

They are also negative for Leu7, NSE and other neural markers, and reactive only toward vimentin.

These cells also are strongly positive for acid phosphatase.

Differential Diagnosis

There is no other congenital alveolar mucosal lesion which is similar to the congenital epulis, but oral involvement by Langerhans cell disease might have enough tissue histiocytes to mimic the epulis somewhat.

Occasional odontogenic tumors contain abundant granular cells, but these are almost never congenital and seldom located outside the bone.

Conversely, 30-50% of cases show odontogenic epithelial rests among the granular cells.

The granular cell tumor (myoblastoma) has cells which are histopathologically identical to those of the granular cell epulis, but the early onset, unique location and pedunculated appearance make the epulis easily differentiated from the tumor.

The tumor, moreover, is not encapsulated and infiltrates into underlying tissues, and many of the lesional cells have a spindled appearance, especially at the deep margin of the tumor.

Similar granular cells are found in the connective tissue papillae of verruciform xanthoma, but the association of this lesion with overlying papillomatosis and the older age at onset make it easily distinguished from granular cell epulis.

Treatment and Prognosis

Prior to birth, the congenital epulis enlarges at a rate similar to that of the growing fetus, but after birth the mass tends to spontaneously regress and disappear over the first 8 months of life.

Residual remnants do not interfere with tooth eruption.

There is, therefore, no need to treat a small congenital lesion.

A larger lesion may interfere with eating or drinking, requiring conservative excision as soon as the child is large enough to safely undergo surgery.

There is no tendency for recurrence and malignant transformation has not been reported.

See also

- granular cell tumor
- gingival tumors
- neonatal epulis

References

- Neumann E. Ein Fall von congenitaler Epulis. Arch Heilk 1871; 12:189-194.

- O’Brien FV, Pielou WD. Congenital epulis: its natural history. Arch Dis Child 1971; 46:559-560.

- Lack EE, et al. Gingival granular cell tumors of the newborn (congenital "epulis"): a clinical and pathologic study of 21 patients. Am J Surg Pathol 1981; 5:37-46.

- Slootweg P, de Wilde P, Vooijs P, et al. Oral granular cell lesions. An immunohistochemical study with emphasis on intermediate-sized filament proteins. Virchows Arch (Pathol Anat Histopathol) 1983; 402:35-45.

- Tucker MC, Rusnock EJ, Axumi N, et al. Gingival granular cell tumors of the newborn: an ultrastructural and immunohistochemical study. Arch Pathol Lab Med 1990; 114:895-898.

- Damm DD, et al. Investigation into the histogenesis of congenital epulis of the newborn. Oral Surg Oral Med Oral Pathol 1993; 76:205-212.

- Kaiseling E, Ruck P, Xiao JC. Congenital epulis and granular cell tumor – a histologic and immunohistochemical study. Oral Surg Oral Med Oral Pathol Oral Radiol Endodont 1995; 80:687-697.

- Kusukawa J, Kulara S, Koga C, Inoue T. Congenital granular cell tumor (congenital epulis) in the fetus: a case report. J Oral Maxillofac Surg 1997; 55:1356-1359.