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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
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.
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).
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.
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*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.
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Takeda Y, Kuroda M, Suzuki A, et al: Congenital Fibrous
Epulis: An undescribed fibrous lesion studied immunhistochemically and ultrastructurally.
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