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ISSN (Print) 1013-9052
EISSN 1658-3558

The Saudi Dental Journal,
P.O. Box 52500,
Riyadh 11563,
Kingdom of Saudi Arabia
Tel.
966-1-467-7328
Fax.
933-1-467-7308 /
966-1-467-7534
Email
saudidj@ksu.edu.sa

Congenital Granular Cell Epulis: Report of 2 cases

Fahri Yilmaz, AN Kemal Uzunlar, Adem Arslan,
Mehmet Yaldiz,Mehmet Ozaydin Huseyin Buyukbayram
Dicle UniversitiesL Tip Facultesi, Patoloji ABD 21280 Diyarbakir, TURKEY

 

Abstract 

 

Congenital granular cell epulis of the newborn is a rare lesion whose histogenesis and natural clinical history have remained obscure. It is important that new cases of this lesion are reported from different populations so that its occurrence and frequency may be ascertained more accurately. Furthermore, the lesion is important in the differential diagnosis of the more aggressive lesions seen in early life. The lesion occurs more often in female infants, but in our cases, one was a male and the other was a female.
 

Introduction

 

The congenital granular cell epulis of the newborn is a rare benign tumor that occurs on the gingiva in the anterior alveolar ridge of the jaws.' Originally described by Neumann2 in 1871, it is also known as congenital gingival granular cell tumor.

This lesion is a pedunculated tumor of firm consistency with a smooth or lobulated surface. The size of the tumor varies from several millimeters to centimeters in diameter.3 Females are affected ten times more often than vmales. The tumor is seen twice as often in the maxilla than in the mandible and usually in the incisor canine region. It does not grow bigger after birth and normally there is no recurrence after surgical excision.1

On microscopic examination, a central mass of closely packed granular cells is seen. The tumor is covered by a stratified squamous epithelium with a flat epithelium-connective tissue junction. A prominent arborizing fibrovascular network in the thin connective tissue septa is usually noted throughout the tumor.4

Since the light microscopic appearance of the granular cells is similar to that seen in granular cell myoblastomas, some investigators have described congenital epulis as congenital gingival granular cell myoblastoma.5 In contrast with congenital epulis, granular cell myoblastoma occurs anywhere in the skin or mucous membranes and in any age group.6 It is rarely congenital, and may recur when incompletely excised. Histomorphologic features such as the prominent fibrovascular network and the absence of pseudoepitheliomatous hyperplasia of the overlying epithelium present in congenital epulis help to differentiate these two entities.7 Case 1: An otherwise normal twenty- day old male infant presented with a grape- sized growth of the mandibular alveolar process. Clinical examination showed a pedunculated mass measuring 1.5x1x0.5 cm in the incisive region arising from the gingiva of the mandibular alveolar ridge and covered with the normal mucosa (Fig. 1). The tumor was surgically excised.

Macroscopic examination showed a lesion approximately 1.5x0.8x0.5 cm; the cut surface was white-gray and smooth. Histologic examination of the tumor revealed a surface border of typical squamous epithelium and a narrow zone of subepithelial connective tissue.

There was no pseudoepitheliomatous hyperplasia of this covering epithelium (Fig. 2). The tumor consisted of closely packed relatively large cells with eosinophilic, granular cytoplasm and small, basophilic nuclei (Fig. 2). There was no sign of cellular pleomorphism or mitotic activity. Many capillaries were present. Immunohistochemically, S-100 protein was negative. A diagnosis of congenital epulis was made.

Case 2: A ten-day old female infant, product of a normal full-term pregnancy was observed at birth to have a tumor attached to the anterior ridge of the maxilla. The oral mass was a well encapsulated, lobulated and pedunculated tumor measuring 2x2x1 cm. The mass interfered with normal breast-feeding. Under a local anaesthetic, the lesion was completely removed surgically.

Macroscopically, the excised lesion measured 2x1.8x0.8 cm and its cut surface was white-gray and smooth. The histologic report was similar to that of Case 1 (Fig. 3).


Discussion

 

Congenital epulis is a rare tumor of the newborn. One hundred and sixty-seven cases (195 lesions) have been reported since 1871. Females are affected 8 to 10 times more often than males,1-8-9 and the lesion was reported to be seen usually 2-4 times more often in the anterior maxilla than in the mandible.'-9-'0

Despite the characteristic anatomical location of congenital epulis on the anterior alveolar ridge of the maxilla and mandible, correct preoperative clinical diagnosis was seldom made.9-'0-" Only three of 21 cases after reported by Lack et al" were correctly diagnosed clinically as congenital epulis. Diagnoses such as haemangioma, fibroma and granuloma were made.

Although fibro-blastic, histiocytic myogenic, neurogenic, odontogenic and endocrine origins have been discussed,'2 immu- nohistochemical and ultrastructural inves- tigations point to an origin from primitive mesenchymal cells with differentiation toward myofibroblasts.'3'4

The preponderant female occurrence suggests an in utero hormonal stimulus. The lesion is also known to regress spontaneously by birth.8 Estrogen receptor studies of epulis tissue, however have proved negative'5 and exogenous mater- nal steroid used during pregnancy has not been associated with occurrence of congenital epulis.9

A similar congenital tumor which does not show granular cells microscopically al- though densely collagenized has been de- scribed.'6

When congenitality, anatomical site location, female gender preponderance, ab- sence of epithelial pseudoepitheliomatous hy- perplasia and S-100 protein are considered, the differential diagnosis of congenital epulis is not difficult.8

The preferred treatment for congenital epulis remains surgical resection at the level of the alveolus. Deeper resections will likely dam- age the underlying unerupted dentition.

References

 

  1. Zuker RM, Buenechea R. Congenital Epulis: Review of the Literature and Case Report. J Oral Maxillofac Surg 1983; 51:1040-43.
  2. Neumann E. Ein Fall Von Congenitaler Epulis. Arch Heilk 1871: 12:189-90.
  3. Chami RG, Wong HS. Large congeni tal epulis of newborn. J Paediatric Surg 1986; 21:929-30.
  4. Zarbo RJ, Hoyd RV, Beals TF, et al. Congenital gingi- val granular cell tumor with smooth muscle cytodif- ferentiation. Oral Surg Oral Med Oral Pathol 1983; 56:512-20.
  5. Koppang HS. Congenital gingival granular cell myo- blastoma. Oral Surg 1972; 34:98-100.
  6. Regezzi JA, Batsakis JC, Courtney RM. Granular cell tumor of the head and neck. J Oral Surg 1979; 37:402-6.
  7. Lack EE, Worsham GF. Callihan MD. Granular cell tumor: A clinicopathologic study of 110 patients. J Surg Oncol 1980; 13:301-16.
  8. Subramaniam R, Shah R. Kapur V. Congenital epulis. J Postgrad Med 1993; 39 (1):36.
  9. Eppley BL, Sadove AM, Campell A. Obstructive congenital epulis in newborn. Ann Plast Surg 1991; 27:152-55.
  10. Rainey JB, Smith IJ. Congenital epulis of the new- born. J Pediatr Surg 1984;19:305-6.
  11. Lack EE, Worsham GF, Callihan MD, et al. Gingival granular cell tumors of the newborn (Congenital Epulis). A clinical and pathologic study of 21 patients. Am J Surg Pathol 1981;15:37-46.
  12. Cigliana B, Fazio PD, Esposido, Pinsabato L. Neonatal Congenital Epulis. Int J Oral Surg 1985;14:456-57.
  13. Damm DD, Cibull Ml, Geissler RH. Investigation into the histogenesis of congenital epulis of the newborn. Oral Surg Oral Med Oral Pathol 1993;76:205-12.
  14. Lifshitz MS, Flotte TJ, Greco MA: Congenital Granu- lar Cell Epulis: Immunhistochemical and ultrastruc- tural observation. Cancer 1984;53:1845-8.
  15. *Lack EE, Perez-Atayde AR, Mc Gill TJ, et al. Gingival granular cell tumors of the newborn (Congenital epulis): Ultrastructural observations relating to histo- genesis. Hum Pathol 1982;13:686-9.
  16. Takeda Y, Kuroda M, Suzuki A, et al: Congenital Fibrous Epulis: An undescribed fibrous lesion studied immunhistochemically and ultrastructurally. Bull To- kyo Dent Coll 1990;31:53-7.

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