PEDIATRICS Vol. 107 No. 2
February 2001,
p. e22
ELECTRONIC ARTICLE:
Congenital Epulis
Oren Lapid, MD*,
Ruthy Shaco-Levy, MD
,
Yuval Krieger, MD*,
Leonid Kachko, MD, and
Amiram Sagi, MD*
From the Departments of * Plastic Surgery and Pathology, the
Soroka University Medical Center Ben-Gurion University of the Negev,
Negev, Israel.
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ABSTRACT |
Epulis is a rare tumor of the newborn, also known as granular cell
tumor of the newborn or Neumann's tumor. This tumor arises from the
mucosa of the gingiva, most commonly from the anterior part of the
maxillary alveolar ridge, and is typically seen as a mass protruding
out of the newborn child's mouth, which may interfere with respiration
or feeding. Epulis is seen only in the newborn and is a different
entity from other granular cell tumors. The tumor has a marked female
preponderance of 8:1. The recommended treatment is prompt surgical
resection. Recurrences of the tumor and damage to future dentition have
not been reported, suggesting that radical excision is not warranted.
A newborn female with such a mass is described. The tumor was resected
using a carbon dioxide laser; the postoperative course was uneventful.
On histologic examination, it was composed of diffuse sheets and
clusters of polygonal cells containing small round to oval nuclei and
abundant coarsely granular cytoplasm. The tumor cells stained positive
for vimentin, and negative for S100-protein, actin, desmin, laminin,
keratin, estrogen, and progesterone receptors. Electron microscopic
examination showed granular cells containing heterogeneous
electron-dense granules, lysosomes, and cytoplasmic lipid droplets. The
clinical and microscopic features of such tumors are reviewed.
Key words:
congenital epulis,
granular cell tumor,
gingival
granular cell tumor.
Epulis, or congenital granular cell tumor
(GCT), is a rare tumor of the newborn. It is seen as a mass arising in
the mouth from the alveolar ridge; this mass may interfere with
respiration or feeding. A case of congenital epulis is reported; the
tumor was resected using a carbon dioxide laser. The clinical aspects as well as the morphologic, histologic, and electron microscopic features of this lesion and its treatment are reviewed.
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CASE REPORT |
Presentation
A newborn girl was referred to the plastic surgery department at
the Soroka Medical Center immediately after birth because of a mass
protruding from her mouth (Figs 1 and
2). The infant was born on the 36th week
of gestation. Pregnancy and parturition were normal. The birth weight
was 2700 g (Apgar: 8-10). Ultrasound scans performed at the 25th
week of gestation did not reveal any abnormality. At birth a firm
pedunculated tissue mass was found protruding from the mouth. The mass
was attached to the gingiva of the left maxilla by a stalk measuring 2 cm by 1 cm. The mass prevented normal closure of the mouth and hindered breast or bottle-feeding, but did not cause airway obstruction or
respiratory distress. No other pathologic finding was noted. Nasogastric feeding was instituted and 2 days after birth, the infant
was operated on under general anesthesia with nasal intubation. Surgery
was performed using the carbon dioxide laser set at 15 watts continuous
wave (Sharplan Lasers Inc, Migdal Haemek, Israel). Blood loss was
insignificant. The area of the resection was left open for closure by
secondary intention. The operative and postoperative course was normal.
Oral feedings were instituted on the first postoperative day and the
child was discharged on the following day. Healing was uneventful and
the gingiva reepithelized within 10 days.
Pathologic evaluation of the tumor confirmed the diagnosis of a
congenital GCT. Macroscopically, the external surface was slightly
irregular and the cut surface was homogenous and tan (Fig
3). Histologically, it was composed of
diffuse sheets and clusters of polygonal cells containing small round
to oval nuclei and abundant coarsely granular cytoplasm. There was a
delicate plexiform network of capillaries (Figs
4 and 5).
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Fig. 4.
Low-power microscopy ×40. At the top of the slide, one may see the
normal squamous epithelium and below it the tumor, which is composed of
polygonal cells with a meshwork of delicate blood vessels.
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The tumor cells stained positively with Periodic acid-Schiff reagent
and the staining was not removed by pretreatment with diastase.
Immunohistochemical stains gave the following results: the tumor cells
were diffusely and strongly positive for vimentin, and negative for
S100-protein, actin, desmin, laminin, keratin, estrogen, and
progesterone receptors.
Portions of the tumor were obtained fresh and immediately fixed in
2.5% cold buffered gluteraldehyde and then processed routinely for
electron microscopy. Electron microscopic examination showed granular
cells containing heterogeneous electron-dense granules, lysosomes, and
cytoplasmic lipid droplets (Fig 6). The cells had irregular cytoplasmic borders with small extensions. There
was no basement membrane associated with the granular cells. There was
no evidence of any epithelial differentiation and no evidence of
Schwannian differentiation as well.
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Fig. 6.
Electron microscopy ×10 000. Electron-dense granules and numerous
lysosomes within the cytoplasm.
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DISCUSSION |
Epulis is also known as GCT of the newborn, or as Neumann's tumor
after the first published case.1
This tumor is rare, as reviewed by Zuker and Buenecha2 in
1993, who described only 167 reported cases.
The tumor is seen only in the newborn and is a different entity from
other GCTs. The tumor has a marked female preponderance of 8:1. It
arises from the mucosa of the gingiva most commonly from the anterior
part of the maxillary alveolar ridge, although it has been described as
arising from the mandibular gingiva as well as from several locations
simultaneously. Reported size varies from several millimeters to 7.5 cm.
There is a striking histologic similarity of granular cells of gingival
granular cell tumors (GGCTs) and the far more common GCT of any other
site. There are several distinguishing features of GGCT, such as
predilection for newborn females, anterior maxillary location, presence
at birth, plexiform arrangement of capillaries, and lack of
pseudoepitheliornatous hyperplasia.3 However, GGCTs
contain cells that are virtually indistinguishable by light microscopy
alone from those seen in GCTs. Although Shwann cells are considered to
give rise to most GCTs, and, hence, these tumors show diffuse and
strong staining for S100-protein, the histogenesis of GGCTs remains
unclear. A few theories exist as to the origin of GGCTs, and the most
popular ones favor gingival stromal (mesenchyrnal) origin and
odontogenic epithelial origin.4 The ultrastructural and
imniunohistochemical findings in our case are in agreement with those
supporting a mesenchymal origin; the cells have features of histiocytes
and fibroblasts. The ultrastructural findings of the tumor that we
describe are clearly different from those in GCT (irregular cell
borders with extensions, lack of basement membrane, and absence of
Schwanian cells). Furthermore, as in the case described by Larralde et
al,5 the tumor cells in our case were reactive for the
macrophage marker CD68. They were also diffusely and strongly positive
for vimentin. The tumor cells in our case did not react with
S100-protein, and this further supports the contention that GGCT and
GCT arise from different cells of origin.
Because GGCT is a congenital tumor in which spontaneous regression has
been reported,6-8 we performed immunohistochemical
stainings for estrogen and progesterone receptors, to see whether the
tumor is influenced by maternal hormones. The results of both of these
stainings were negative. Although the development of the tumor in the
uterus and the higher incidence in females imply a hormonal mechanism,
it probably takes place through different pathways.
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CONCLUSION |
Our case provides additional evidence that GGCT derives from a
mesenchymal origin, unlike the GCT of any other site.
The recommended treatment is prompt surgical resection. Recurrences of
the tumor and damage to future dentition have not been reported,
suggesting that radical excision is not warranted.
The tumor is often misdiagnosed before surgery because of its rarity
and lack of awareness by clinicians.2 The differential
diagnosis includes: hemangioma, lymphangioma, fibroma, rhabdomyoma, and
hetrotopic gastrointestinal cysts.7
In our case the tumor was not diagnosed by ultrasound on the 25th week
of gestation, suggesting that it may have developed later. There have
been reports of prenatal diagnosis and in those cases, in which the
tumor was detected late in gestation.9
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FOOTNOTES |
Received for publication May 22, 2000; accepted Sep 26, 2000.
Reprint requests to (O.L.) Department of Plastic Surgery,
Soroka Medical Center, 151, Beer-Sheba 84101, Israel. E-mail:
lapid{at}actcom.co.il
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ABBREVIATIONS |
GCT, granular cell tumor;
GGCT, gingival granular
cell tumor.
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REFERENCES |
-
Neumann E
Elin fall von congaliter epulis.
Arch Helik
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12:189
-
Zuker RM,
Buenecha R
Congenital epulis: review of the literature and
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Lifshitz MS,
Flotte TJ,
Greco MA
Congenital granular cell epulis:
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Larralde M,
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Pediatrics (ISSN 0031 4005). Copyright ©2001 by the American Academy of Pediatrics
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Abstract
Discussion
