Abstract
Congenital epulis is a rare tumor of the the newborns. We found a case
of soft elastic pedunculated masses in the maxillary (26 x 14 x 11 mm)
and mandibular (55 x 44 x 11 mm) anterior areas, protruding out of the
oral cavity. The large multiple masses interfered with suckling. These
masses were excised using a carbon dioxide laser under general
anesthesia. They were diagnosed histopathologically and immunocamically
as congenital granular cell epulis. These were no complication after
surgery.
key words : congenital epulis,surgical excision,maxillary
anterior,mandibular anterior
Key clnical message
Congenital epulis, which are soft elastic pedunculated masses in the
left maxillary and mandibular anterior areas protruding out of the oral
cavity, is a rare tumor of the the newborns. These masses were no
complication after dioxide laser surgery.
1. Introduction
Congenital epulis is a localized benign mass that develops in the
gingiva of newborns and it was initially reported by Neumann in 1871. To
our knowledge, approximately 200 cases have been reported, but
development in the upper and lower jaws is very rare.
We report a patient with large congenital epulis, which developed in
the gingiva of the left maxillary and mandibular anterior tooth regions.
2. Case report
Patient: Newborn at one day after birth
First examination: May 2013
Chief complaint: Impaired suckling.
Familial medical history: None in particular
History of present illness: No abnormalities were noted during
pregnancy. The patient was delivered normally (3,094 g, 38 weeks and 5
days of gestation) and the Apgar score was 10/10. As masses were noted
in the left maxillary and mandibular anterior tooth regions at birth,
the patient was transferred from an obstetric clinic to a nearby
emergency hospital. No concomitant physical or visceral malformation was
observed, but the masses were judged as requiring treatment. The patient
was transferred to the NICU of our hospital and then referred to our
department for close examination of the masses.
Present status:
Systemic findings: None in particular
Local findings: In the oral cavity, elastic soft pedunculated masses,
with sizes of 26 x 14 x 11 and 55 x 44 x 11 mm, were present in the left
maxillary and mandibular anterior tooth regions, respectively. The
masses extraorally protruded. The surfaces of the maxillary and
mandibular masses were smooth, their color was bright red, and flare was
partially noted (Fig. 1).
Imaging findings: On MRI, both maxillary and mandibular masses
demonstrated slightly higher intensity than muscle on T1-weighted
imaging, and 20 x 15 x 10-mm and 50 x 40 x 10-mm solid masses exhibiting
slightly high intensity were detected on T2-weighted imaging. Their
content was mostly homogeneous.
Clinical diagnosis: Congenital epulis
Treatment and course: After admission to the NICU, tube feeding was
performed for impaired suckling under whole body management by the
pediatric department and the respiratory condition was stable. As
necrosis of the mass surface progressed, the epulis was resected by
carbon dioxide laser for the clinical diagnosis of congenital epulis
under general anesthesia 5 days after admission. The resected specimen
was solid elastic and soft. After surgery, no problems, such as
infection, occurred. Training of suckling was initiated 3 days after
surgery and the patient was discharged at 10 days with a favorable
recovery (Fig. 2).
Histopathological findings: Under the mucosal epithelium, solid cells
containing cytoplasm filled with eosinophilic granules and oval-shaped
dark-stained nuclei with a clear nucleolus had densely proliferated. The
interstitium comprised narrow vascular connective tissue, and hemorrhage
and necrosis were partially noted. The superficial layer was covered
with squamous epithelium, demonstrating parakeratosis and prickle cell
hypertrophy.
On immunohistochemical staining, the masses were PAS staining (+),
alcian blue staining (+), NSE (+), S-100 protein (-), Vimentin (+), CD68
(-), neurofilament (-), and GFAP (-).
Histopathological diagnosis: Congenital granular cell epulis.
3. Discussion
Congenital epulis is a rare disease developing in newborns. It was
initially reported by Neumann in 1871[1] and approximately 200 cases
have been reported[2]. The incidence is higher in females at a sex
ratio of female: male=8:1. It is more frequently observed in maxillary
than mandibular gingival mucosa (2:1 or 3:1), and lesions are bright
red, elastic soft, and pedunculated with a smooth surface[3,4]. The
size of lesions varies up to 90 mm[3].
Course observation may be prioritized when there is no functional or
esthetic disturbance. In the present patient, the lesions were large
congenital epulis that developed on the same side of the upper and lower
jaws, and as impaired suckling and esthetic disturbance were observed,
surgical resection was indicated. No case of recurrence after resection
has been reported, but extensive resection may cause eruption disorder
of deciduous teeth[5], to which attention should be paid.
The main diseases included in the differential diagnosis in newborns
are Epstein pearl, granular cell tumor, vascular malformation, and
neuroectodermal tumor in childhood. The histopathological findings of
congenital epulis are particulate granules, eosinophilic cytoplasm, and
slightly abnormal nuclei. No pseudocarcinomatous hyperplasia is observed
in the capsular mucosa in congenital epulis in contrast to granular cell
tumors[6]. Similar findings were noted in the present patient.
Immunohistologically, congenital epulis and granular cell tumors are
S100 protein (-) and vimentin (+)[7], although vimentin (-) granular
cell tumors have been reported[10]. In addition, granular cell
tumors are NSE (+), GFAP (-), CD68 (-), and desmin (-)[7]. Both
types of lesions are PAS staining (+) in many cases[6].
In the present patient, cytoplasm with eosinophilic granules was
noted on HE staining, and the cells contained glycoprotein based on PAS
staining (+) and Alcian blue staining (+). On immunohistochemistry, the
masses were found to have originated from granular cells because they
were NSE (+), S-100 protein (-), vimentin (+), GFAP (-), CD-68 (-), and
desmin (-).
Many points remain unclear regarding the pathogenesis of this
disease. Fibroblasts, histiocytes, myogenic, neurogenic, reactive
proliferation, and hamartoma of mesenchymal cells have been reported as
the tissue of origin [2, 3]. Miura et al. [8] considered
mechanical stimulation of the oral mucosa by the finger tips and nails,
such as sucking, to be behaviors exhibited in the fetal period that can
induce epulis. Furthermore, Mori et al. [9] reported that fetuses in
the uterus start sucking behavior at approximately 17 weeks of
gestation, and the sucking force is sufficiently strong to form sucking
blisters on the 1st and 3rd fingers, wrist, and dorsum of the hand.
Thus, epulis formation may be consistent with inflammatory reactions
caused by sucking stimulation.
In a study by Ohkawa et al. [10], congenital epulis was
accompanied by granular cells in all cases treated by resection earlier
than 20 days after birth, but it was not accompanied by granular cells
in cases at 2 or more months after birth, and both types were present in
cases treated surgically between 20 days and 2 months after birth. They
considered the internal environment, such as hormonal and immunological
factors, to change after birth, causing granular cells to disappear and
be replaced with fibrous connective tissue. This disappearance of
granular cells and replacement with fibrous connective tissue may occur
after birth, i.e., transition to fibrous connective tissue does not
occur in the fetal environment.
In cases of spontaneous regression after birth, the final step of
inflammation, absorption and replacement (tissue regeneration) of
inflammatory tissue, may have occurred after birth. Based on the above
findings, mechanical stimulation starts from 17 weeks of gestation, at
which sucking starts and the epulis is formed through inflammation.
Moreover, the final step of inflammation, absorption and replacement,
does not progress during the fetal period. Therefore, the epulis grows
to a large size because mechanical stimulation by sucking starts
earlier.
In our patient, surgical resection was selected because impaired
suckling and esthetic disturbance were noted due to large lesions in the
upper and lower jaws on the same side, and a favorable clinical course
was acquired. As the delayed eruption of deciduous teeth and
malalignment may occur in the future, long-term follow-up is important.
4. Conclusion
We report a 1-day-old newborn girl with large congenital epulis in
the left anterior deciduous tooth regions of the upper and lower jaws,
accompanying impaired suckling. The histopathological finding of the
masses was congenital granular cell epulis.
There is no conflict of interest for this report.
Fig. 1
Intraoral Figgraph 5 days after admission: Pedunculated tumorous lesions
with sizes of 26 x 14 x 11 and 55 x 44 x 11 mm were present in the left
maxillary and mandibular anterior tooth regions, respectively. The mass
surface was necrotized.
Fig. 2
Intraoral Figgraphs 10 days after surgery
References
1) Neumann E: Ein von congenital epulis of newborn. Arch Heilkd.1871; 12: 189-190.
2) Nakata M, Annol K, et al: Prenatal diagnosis of congenital epulis: A
case report. Ultrasound Obstet Gynecol. 2002; 20: 627-629.
3) Chami RG, Wang HS: Large congenital epulis of newborn. J
Pediatr Surg. 1986; 21: 189-190.
4) Bowe JJ: Congenital epulis tumor. Case report. Plast Reconstr
Surg. 1974; 5: 227-229.
5) Küpers AM, Andriessen P, et al: Congenital epulis of the jaw: a
series of five cases and review of literature. Pediatr Surg Int.2009; 25: 207-210.
6) Prigkos AC, Nikolakis MD, et al:Spindle cell epulis in an
8-month-old child: a histologic variant of congenital granular cell
epulis? Head Neck Pathol. 2012; 6: 467-470.
7) Sahu S, Maurya R, et al:Multiple congenital epulis in newborn - A
rare presentation. J Oral Maxillofac Pathol . 2009; 13: 78-80.
8) Miura M,Takagishi M, et al :A case of congenital epulis in a
neonate.POMS. 2006; 16: 36-39.
9) Mori G :Natural history of sucking blisters. Obstetrics and
Gynecology. 1990; 6: 1361-1365.
10) Ohkawa M, Hiramoto M, et al : A case of congenital epulis which
decreased in size. Practica Oto-Rhino-Laryngologica. 1981; 75:
329-355.
AUTHOR CONTRIBUTIONS
Fumishige Oseko and Toshiro Yamamoto: have made substantial contribution
to conception and design of the manuscript as they interpreted data,
drafted the work, approved the final version, and agreed to be
ac-countable for all aspects of the work. Shigeta Takizawa and Keiji
Adachi: have been involved in drafting the manuscript, approving the
final ver-sion, and agreed to be accountable for all aspects of the
work. Narisato Kanamura: has critically revised the work, given the
final approval of the version to be published, and agreed to be
accountable for all aspects of the work.