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 Table of Contents  
Year : 2021  |  Volume : 33  |  Issue : 1  |  Page : 99-102

A rare case report of congenital granular cell epulis

1 Department of Oral Medicine and Radiology, Government Dental College and Research Institute, Bangalore, Karnataka, India
2 Department of Pathology, Bangalore Medical College and Research Institute, Bangalore, Karnataka, India

Date of Submission17-Jul-2020
Date of Decision10-Jan-2021
Date of Acceptance13-Jan-2021
Date of Web Publication26-Mar-2021

Correspondence Address:
Dr. R Priyanka
Department of Oral Medicine and Radiology, Government Dental College and Research Institute, Bangalore 560002, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaomr.jiaomr_148_20

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Congenital granular cell epulis is a very rare tumor first described by Neumann in 1871. It is of mesenchymal origin with the female predilection in the ratio of 8:1. The lesion appears as a large protuberant mass and is commonly associated with obstruction causing difficulty in feeding and deglutition. We report a case of a newborn female who had feeding difficulty because of a protuberant mass arising from the oral cavity. The mass was surgically excised under general anesthesia and was histopathologically proven as congenital granular cell epulis. The follow-up for 3 months showed no recurrence. There are reported complications prenatally and postnatally. Hence, the treatment should be initiated early. The treatment of choice is complete surgical excision and no recurrence has been reported.

Keywords: Congenital epulis, congenital granular cell epulis, Neumann's tumor, newborn

How to cite this article:
Vijayalakshmi K R, Priyanka R, Pai AS. A rare case report of congenital granular cell epulis. J Indian Acad Oral Med Radiol 2021;33:99-102

How to cite this URL:
Vijayalakshmi K R, Priyanka R, Pai AS. A rare case report of congenital granular cell epulis. J Indian Acad Oral Med Radiol [serial online] 2021 [cited 2021 Sep 19];33:99-102. Available from: https://www.jiaomr.in/text.asp?2021/33/1/99/312196

   Introduction Top

Congenital granular cell epulis (CGCE), also known as Congenital epulis of the newborn, Granular cell tumor of the gingiva, Congenital granular cell fibroblastoma, and Congenital granular cell myoblastoma, was first described by Neumann in 1871 and hence it can also be called the Neumann's tumor.[1] World Health Organization in 2005 recommended the use of “Congenital granular cell epulis” as the specific term and has categorized it in their recent classification in 2017 as a “tumor of uncertain histogenesis.”[2] It has been estimated that the incidence of congenital epulis is 0.0006% with only a few hundred cases have been reported so far. The occurrence is higher in the Caucasian population.[3] CGCE usually shows a strong predilection for females. The common clinical presentation includes the presence of a solitary, pedunculated nodule noted in the maxillary anterior region. The size usually varies from several mm to a few cm in size. Though the lesions show no propensity towards recurrence or malignant transformation, surgical excision is the treatment of choice, especially for larger lesions with the tendency for interference with breathing and feeding. We hereby report a case of CGCE in a newborn female with a solitary, pedunculated, and lobulated mass with its stalk attached to the lower gum pad with interference in feeding. The case was managed surgically and the follow-up for 3 months showed no recurrence.

Patient information

A full-term female neonate with a birth weight of 2700 g was immediately referred to the Department of Oral Medicine and Radiology after birth for the examination of a mass protruding from her mouth. The review of the neonate's systems was unremarkable. The mother's pregnancy was uneventful with normal vaginal delivery. Family history revealed first-degree consanguineous marriage. No other symptoms were reported.

Clinical findings

On clinical examination, a pedunculated reddish pink, well-circumscribed lobulated mass, smooth in surface texture, uniformly firm in consistency with broad base noted on the lower anterior gum pad with its stalk attached just anterior to left lateral sulcus in the place of future canine and lateral incisor of size 3 × 2 cm noted as shown in [Figure 1]. No bleeding was elicited on provocation. There was difficulty in feeding and no respiratory obstruction.
Figure 1: Pedunculated mass with a lobulated surface noted on the lower anterior gumpad

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[Figure 2] shows the timeline of the patient.
Figure 2: Timeline of the case according to CARE guidelines

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Diagnostic assessment

Considering these findings, the provisional diagnosis of congenital epulis of the newborn was established. The clinical differential diagnosis of Hemangioma, teratoma and melanotic neuroectodermal tumor of infancy (MNTI) were considered [Table 1].
Table 1: Clinical differential diagnosis of CGCE

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[Figure 3](a) shows the excised specimen measuring 20 ×23 cm. The histopathological section of the excised specimen showed polypoidal mass lined by stratified squamous epithelium. The underlying epithelium showed large polygonal cells with abundant granular cytoplasm. Round to oval nuclei and prominent nucleoli with no evidence of cellular atypia noted. Tumor cells were seen extending up to the smooth muscle layer with prominent vascularity seen between the tumor cells. These findings were suggestive of congenital granular cell tumor. Immunohistochemistry with S-100 was negative and strong positivity with diffuse strong cytoplasmic staining of almost all the tumor cells to NSE and Vimentin confirmed the diagnosis of Congenital epulis as shown in [Figure 3](b).
Figure 3: (a) Excised specimen. (b) Histopathological section of the specimen showing polypoidal mass lined by stratified squamous epithelium and immunohistochemistry with S-100 negativity; NSE and Vimentin positivity in their cytoplasm, respectively. (c) Surgical site following surgery. (d) Complete healing with no recurrence after 3 months of follow-up

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Therapeutic information

After preoperative evaluation, the mass was surgically excised on the 7th day under general anesthesia with no intraoperative and postoperative complications. The neonate was able to breastfeed normally the next day following surgery. [Figure 3](c) shows the postoperative site following the excision.


The patient is presently under periodic follow-up for 3 months. No recurrence has been noted so far as seen in [Figure 3](d). However, there was an eruption of a neonatal tooth noted at the excision site after a week following the excision, which eventually shed off on its own in 3 days.

   Discussion Top

Even though the exact etiology has not been established, various theories like myoblastic, odontogenic, neurogenic, fibroblastic, histiocytic, endocrinologic, or reactive origin have been proposed to explain the histogenesis of the tumor.[4] There is a strong female predominance with a female to male ratio of 8-10. Female predominance could be due to an endogenous hormonal influence, but it has fallen into despair due to the absence of detectable estrogen or progesterone receptors in the tumor.[5] Though congenital epulis usually presents as an incidental finding, large lesions often obstruct the feeding or respiration and no familial incidence has been established.[6]

Congenital epulis clinically appears as a pedunculated protuberant mass and is most commonly seen in the maxillary alveolar process than the mandibular alveolus in the ratio 3:1 in the region of primary canine and lateral incisor. It varies from a few mm to 9 cm in size. The present case was a female neonate and the lesion was in the mandibular gum pad. It is usually seen as a solitary lesion, but multiple in 10% of cases.[7] Rarely, it can also involve the tongue, palate, skin, subcutaneous tissue, skeleton muscles, and the vocal cord. It is known to be associated with various congenital anomalies like neurofibromatosis, polydactyly, Binder syndrome, congenital goiter, and bilateral transverse facial cleft.[1],[8]

The common clinical differential diagnosis include hemangioma, teratoma, and MNTI. Hemangioma is the most common benign tumor of the blood vessels in infancy and childhood with an estimated incidence of 3%-10% by the age of one year. This commonly affects the lips, tongue, & buccal mucosa and presents as a sessile or pedunculated, soft, smooth, or lobulated mass and is usually dark red. It is usually observed during the neonatal period. Within the first 6 months of life, the tumor exhibits rapid growth and expansion which is followed by a gradual self-involution. In 50–80% of cases, it disappears by 5 years of age. Teratomas are true neoplasms composed of tissues from all three germinal layers and it occurs commonly in the midline or paraxial location from the brain to the sacral area with a predilection to sacrococcygeal region. Oral involvement is extremely rare comprising about 1–10% with reported occurrence in the tongue and the hard palate. MNTI is a rare, rapidly growing benign tumor that arises from the neural crest. It typically involves the infants with the mean age of 6.5 months and slight male predominance is noted. The maxilla is the most common site followed by the skull and the mandible.

The fact that CGCE develops after the 22nd week of gestation and the lack of specific signs makes the prenatal diagnosis difficult. However, the diagnosis of CGCE can be established with the aid of fetal three-dimensional (3D) ultrasound and MRI as early as the 26th week of the gestational period. Doppler ultrasonography demonstrates multiple branching feeding vessels entering the mass through the peduncle which can be differentiated from the disorganized high-flow vascularity seen in hemangioma. Fetal 3D ultrasound better demonstrates the facial anomalies by providing a better overview of the appearance of the tumor and its relation to the lips and airway.

Prenatally, the tumor tissue in MRI demonstrates low homogeneous intensity than cerebral parenchyma in T2-weighted images. Postnatally, MRI features are considered non-specific; but helps in determining the characteristics of the tumor and in treatment planning. CGCE demonstrates iso intensity to muscles on T1-weighted MRI images and isogenous to heterogeneous signal intensity on T2-weighted MRI images with a rim of low signal intensity indicating the more pronounced cellularity at its periphery.[9]

Histologically, congenital epulis is similar to granular cell tumor and can be differentiated by the presence of pseudoepitheliomatous hyperplasia, less vascular supply, and prominent nerve bundles in the latter. Also, granular cell tumor affects all the age groups whereas congenital epulis is seen at birth. A histological variant of CGCE with increased fibrosis and spindle cells has also been reported. Furthermore, Immunohistochemical studies of Congenital Epulis demonstrate the negativity to S-100 protein in contrast to granular cell tumor, which is a derivative of Schwann cells. Similarly, the lesion removed from our patient was negative for S-100 protein. Immunoreactivity with Neuron-specific enolase (NSE) indicates the neural origin. Vimentin is a marker of intermediate filaments present in cells of mesenchymal origin which showed strong positivity in the present case. Most of the reported immunohistochemical studies for CGCE showed negative for S-100 and positivity for NSE and vimentin which is in accordance with the present study.

Complete surgical excision of the lesion without damaging the underlying alveolar bone and developing tooth buds is the treatment of choice. However, few case reports have documented the spontaneous regression, especially in the case of small lesions.[10] No recurrence has been reported even after incomplete excision. The complications can range from polyhydramnios and obstructed deglutition of amniotic fluid prenatally and midface hypoplasia, incisor hypoplasia, feeding, and respiratory/aesthetic problems following birth.[1]

   Conclusion Top

CGCE is a rare lesion noted as the protuberant lobulated mass in the oral cavity of newborns. Though it is a benign tumor, there are complications associated with CGCE both prenatally and postnatally. The unenlightenment in conjunction with its rare occurrence often leads to misdiagnosis. Prenatal imaging can help in the diagnosis which becomes advantageous for parental counseling and for monitoring the airway and fetal swallowing obstruction. Hence, a proper understanding of the lesion is of absolute necessity for its proper diagnosis and management.

Declaration of patient consent

The authors certify that they have obtained all appropriate informed assent forms for the patient as the patient was below 18 years of age. In the form, the patient's parent has given his/her/their consent for the patient's images and other clinical information to be reported in the journal. The patient's parents understand that the names and initials of the patient will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Yuwanati M, Mhaske S, Mhaske A. Congenital granular cell tumor–a rare entity. J Neonatal Surg 2015;4:17.  Back to cited text no. 1
Van der Waal I. Congenital granular cell epulis. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editos. World Health Organization Classification of Tumours. Pathology and Genetics. Head and Neck Tumours. Lyon: IARC Press; 2005. p. 198.  Back to cited text no. 2
Edetanlen EB, Ogboh CB. Congenital granular cell epulis: Report of a case and review of the literature. Afr J Oral Health 2019;9:46-50.  Back to cited text no. 3
Jain N, Sinha P, Singh L. Large congenital epulis in a newborn: Diagnosis and management. Ear Nose Throat J 2020;99:NP 79-81.  Back to cited text no. 4
Lapid O, Shaco-Levy R, Krieger Y, Kachko L, Sagi A. Congenital epulis. Pediatrics 2001;107:e22.  Back to cited text no. 5
Dzieniecka M, Komorowska A, Grzelak-Krzymianowska A, Kulig A. Multiple congenital epulis (congenital granular cell tumors) in the newborn: A case report and review of literature. Pol J Pathol 2011;62:69-71.  Back to cited text no. 6
Saki N, Araghi S. Multiple congenital epulis in alveolar ridges of maxilla and mandible in a newborn: A rare case report. Case Rep Otolaryngol 2014;2014:606985.  Back to cited text no. 7
Padhy S, Mishra BN, Joshi RK, Pahi PP. Congenital epulis: A case report. IOSR J Dent Med Sci 2020;19:41-49.  Back to cited text no. 8
Kim YD, Kim HJ, Lee NK, Ha WH, Lee CH, Park SE. Congenital epulis: Prenatal ultrasonographic and postnatal MR features with pathologic correlation. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106:743-8.  Back to cited text no. 9
Cheung JM, Putra J. Congenital granular cell epulis: Classic presentation and its differential diagnosis. Head Neck Pathol 2020;14:208-11.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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