Epulis presenting as congenital multiple polypoidal oral mass lesion: A surprising case report

Himanshu Shukla; Chaturbhuj Ramanand Agrawal; Chandra Sarat; Dharma Ram

doi:10.4103/archms.archms_19_17

CASE REPORT

Year : 2017 | Volume : 2 | Issue : 1 | Page : 32-34

Epulis presenting as congenital multiple polypoidal oral mass lesion: A surprising case report

Himanshu Shukla¹, Chaturbhuj Ramanand Agrawal², Chandra Sarat³, Dharma Ram¹
¹ Department of Surgical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
² Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
³ Department of Surgical Oncology, Indraprastha Apollo Hospital, New Delhi, India

Date of Web Publication

13-Sep-2017

Correspondence Address:
Chaturbhuj Ramanand Agrawal
Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Sector 5, Rohini, New Delhi - 110 085
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/archms.archms_19_17

Abstract

A newborn infant with congenital epulis (CE) can be a striking miserable sight for both parents and health care professionals involved in neonatal care. These tumours of the infant mouth can be remarkably large, occupying much of the oral cavity and posing a risk of airway obstruction or interfering with feeding with marked facial deformity. Neumann first described congenital epulis of newborn in 1871. It is most frequently located on the anterior maxillary alveolar ridge with slight female predilection. It clinically appears as a pedunculated protuberant mass. In cases with large lesions, mechanical oral and nasal obstruction can impair fetal deglutition and neonatal respiratory efforts resulting in polyhydramnios prenatally or respiratory impairment postnatally. Histologically Congenital epulis shows characteristic large cells with granular cytoplasm and spindle cells resembling fibroblasts. The exact histogenesis is still uncertain, various theories of origin are epithelial, undifferentiated mesenchymal cells, pericytes, fibroblast, smooth muscle cells and nerve related cells. Herein we report a newborn female child with Multiple Congenital epulis arising from maxilla and mandible with investigation, management and follow-up.

Keywords: Congenital granular cell epulis, multiple congenital epulis, odontogenic epithelium

How to cite this article:
Shukla H, Agrawal CR, Sarat C, Ram D. Epulis presenting as congenital multiple polypoidal oral mass lesion: A surprising case report. Arch Med Surg 2017;2:32-4

How to cite this URL:
Shukla H, Agrawal CR, Sarat C, Ram D. Epulis presenting as congenital multiple polypoidal oral mass lesion: A surprising case report. Arch Med Surg [serial online] 2017 [cited 2023 Jun 4];2:32-4. Available from: https://www.archms.org/text.asp?2017/2/1/32/214558

Introduction

Congenital epulis (CE) is a reactive or degenerative lesion with a mesenchymal origin rather than a true neoplasm.^[1],[2] This lesion is rare as reviewed by Zuker and Buenecha in 1993, who described only 167 reported cases in the literature. Neumann first described CE in 1871, hence also known as Neumann's tumor.^[3],[4] CE usually occur as a well-defined pedunculated mass with smooth or lobulated surface. It commonly arises from the anterior maxillary alveolar ridge of newborn, not associated with any other congenital malformations.^[3],[4] CE has a female predilection with 8:1 ratio; an endogenous (intrauterine) hormonal stimulus is proposed but disproved by the absence of receptor for estrogen and progesterone.^{[3],[4],[5],[6]} Its differentiation from malignant granular cell tumor is important for proper segregation and management of this rare entity.

Case Report

A 7-day-old female child presented to us with multiple pink-colored masses protruding from the mouth which were noticed at birth [Figure 1]. She was delivered as a full-term normal vaginal delivery with uneventful antepartum period. No significant family or hereditary history was noted. There was also slight difficulty in feeding; however, there was no increase in the size of swelling in the postpartum period and there was no difficulty in sucking milk.

Figure 1: Preoperative photograph showing large mass protruding from mouth and attached to maxilla; three small masses attached to mandible two laterally and one in midline

Click here to view

On clinical examination, a large round, pedunculated, midline soft tissue mass, around 3 cm diameter, protruding from mouth and exhibiting erythematous and smooth surface was found to be attached to the anterior gingival margin of the maxilla. Three other soft tissue masses of 1.5 cm each were also found attached to the mandible which was also smooth surfaced. Two swellings were found attached to the left lateral margin and one in midline to the mandible. The patient also had depressed nasal bridge with decreased nasal patency and no other structural abnormality [Figure 1]. At presentation, the child was asymptomatic except mass, and there was no respiratory difficulty. Feeding was started with infant feeding tube. All other milestones were normal according to age. General physical examination was unremarkable otherwise. Routine laboratory tests were normal. The patient underwent fine-needle aspiration cytology from the mass which was suggestive of the benign fibrous lesion.

In consultation with anesthetist, midline lesion was gently pushed laterally, and oral intubation was performed because of the depressed nasal bridge. The lesion was completely excised under general anesthesia with minimal intraoperative blood loss. Immediate postoperative period was uneventful, and regular oral feed was started on the 7^th postoperative day which was well tolerated. The patient was discharged with her mother on the 9^th postoperative day. Cut surface of the specimen showed homogeneous grayish white tissue which on histopathological examination revealed a growth covered with mildly acanthotic squamous epithelium and showed closely packed polygonal cells with centrally placed nuclei and markedly granular cytoplasm without any cross striations. There was increased vascularity without any evidence of atypia or malignancy. These findings were consistent with diagnosis of congenital granular cell epulis.

Congenital granular cell epulis

At 6 months of follow-up, the baby was thriving and gaining weight normally without any recurrence [Figure 2].

Figure 2: Postoperative photograph at 6 months showing no evidence of recurrence

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Discussion

CE usually occur in the mucosa of the maxillary alveolus as smooth-surfaced pink mass without any other dental malformation or other congenital abnormalities. CE causing superior deviation of upper lip, flat nose with the absence of anterior nasal spine, and reduced anteroposterior dimension of maxilla have been reported previously by Koch et al.^[3] Similar findings were noted in our case with depressed nose and deviated upper lip. The exact histogenesis is still uncertain, but various proposed cell of origin are odontogenic epithelium, undifferentiated mesenchymal cell, pericytes, fibroblast, smooth muscle cell, nerve-related cell, and histiocytes.^[3]

CE has an 8:1 female predilection and 3:1 maxillary alveolar site predilection.^[6] Our case is a female child, and lesions occurred in both maxilla and mandible. It usually occurs as a single mass although 10% cases occur as multiple.^[3],[4] In our case, there were multiple CE originating from both mandible and maxilla. CE of the newborn is the widely accepted term and is preferred over congenital granular cell tumor (CGCT) which suggests a neoplastic origin not applicable to CE. The absence of local recurrence even after incomplete excision, the possibility of spontaneous regression, and the lack of a malignant counterpart all favor CE as a nonneoplastic lesion.^[2],[4],[7]

Based on the above findings, it is concluded that the biologic behavior of CGCT is compatible with embryonic hamartoma. The term tumor does not hold true, as it does not behave in a true neoplastic manner. The use of term epulis has been disagreed because of the presence of the same type of lesion in other part of the body apart from alveolar ridge. CE is a lesion found on the alveolar ridge of newborns. Histologically, these lesions appear similar to the granular cell tumor; however, CE is negative for S-100 and other markers found in the malignant granular cell tumor. The CE, however, is vimentin positive, unlike granular cell tumor.

In this case, there was no recurrence of the lesions after 6 months of follow-up. Hence, we conclude that congenital granular cell lesion is a benign tumor, and treatment of choice is complete surgical excision.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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