Congenital epulides: A rare case report :[PAUTHORS], Indian Journal of Dental Research (IJDR)

CASE REPORT
Year : 2014 | Volume : 25 | Issue : 6 | Page : 806--808

Congenital epulides: A rare case report

Saurabh Kumar¹, Chaya Prasad², Meera Thomas², Santosh Koshy¹,
¹ Department of Dental and Oral Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
² Department of Pathology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India

Correspondence Address:
Saurabh Kumar
Department of Dental and Oral Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu
India

Abstract

Congenital epulis (CE) or �DQ�Granular cell epulis�DQ� also previously termed as �DQ�Neumann�SQ�s tumor�DQ� is a benign growth arising from the mucosa of the gingiva, typically seen as a mass protruding from the infant�SQ�s mouth, often interfering with respiration and feeding. These tumors generally present as a single mass arising from the upper alveolus. We report a rare case of two congenital epulides arising from the maxillary and mandibular alveolus in a day old female infant, which was surgically excised, allowing for early initiation of breast feeding. The tumor cells stained negative for S100 protein hence differentiating from other granular cell tumors. The clinical presentation, differential diagnosis in regard to the various neonatal oral swellings and the management of CE has been discussed.

How to cite this article:
Kumar S, Prasad C, Thomas M, Koshy S. Congenital epulides: A rare case report.Indian J Dent Res 2014;25:806-808

How to cite this URL:
Kumar S, Prasad C, Thomas M, Koshy S. Congenital epulides: A rare case report. Indian J Dent Res [serial online] 2014 [cited 2023 Sep 25 ];25:806-808
Available from: https://www.ijdr.in/text.asp?2014/25/6/806/152209

Full Text

Congenital gingival granular cell tumor (GCT), also known as congenital epulis (CE) or "Granular cell epulis" was first described by Neumann in 1871 hence sometimes also referred to as Neumann's tumor. [1],[2] Seen in newborns, CE can be differentiated from adult GCTs. It usually occurs as a well-defined pedunculated mass, most commonly arising from the maxillary anterior alveolar ridge. When large or multiple these tumors can cause significant respiratory and feeding difficulty. This benign lesion is a rare entity whose origin remains unclear. [3] Several theories have been suggested, namely myoblastic, histiocytic, neurogenic and endocrinologic. CE usually is not associated with any congenital malformations except for occasional reports of hypoplastic or absent tooth with sometimes possibility of midfacial hypoplasia. Most often the diagnosis is made clinically and confirmed histologically by differentiating it from other GCT's. CE behaves in a benign manner and no recurrence or metastatic lesions have been reported. Although spontaneous regression has been reported recommended treatment includes surgical excision. This report documents the presentation and management of a 1-day-old female infant with two congenital epulides attached to maxillary and mandibular alveolar ridges treated with surgical excision.

CASE REPORT

A day old female infant was referred from the Neonatology Unit with two distinct growths in the oral cavity noticed at birth, one of the swelling was attached from the anterior maxillary alveolus and the other from the anterior mandibular alveolus [Figure 1]. No other structural deformities were present. There was no significant family history and the laboratory findings including hematological parameters were within the normal limits. On intra-oral examination, two independent pedunculated masses were seen arising from the maxillary (2.5 cm × 2 cm) and mandibular alveolus (1.5 cm × 1 cm) respectively. The swellings had a smooth surface covered with normal overlying mucosa. Each of the swelling was attached by a thin stalk to the alveolus. On palpation, they were firm, noncompressible and nontender. Due to the swellings being significantly large the baby had difficulty in feeding however there was no respiratory distress. The growths were excised under general anesthesia, with minimal postoperative bleeding. The baby had an uneventful recovery and started feeding the day subsequent to the surgery. Histopathological examination showed the masses to be lined by flattened stratified squamous epithelium and comprising of sheets of histiocytes with abundant eosinophilic granular cytoplasm, eccentrically placed round to oval bland nuclei with some showing prominent nucleoli with foci of mild chronic inflammation. There was no evidence of mitosis or necrosis [Figure 2]. Immunostaining showed that the histiocytes were negative for S100 [Figure 3], thus confirming the lesion as CE and differentiating it from other variants of GCTs.{Figure 1}{Figure 2}{Figure 3}

DISCUSSION

Since its first description in 1871, very few cases of CE have been reported in literature. [4] The word epulis is a Greek word which means "on the gum" or "gum boil", however there are reports of CE originating from the tongue. [5] CE has a female predilection, occurring 8-10 times more commonly in females than males. The most frequent location of the CE is the anterior maxillary alveolar ridge; it is seen less often on the mandible. While it usually occurs singly, in 10% it occurs as multiple masses. [3] In the patient described above, two separate epulides were seen on the anterior maxillary and mandibular alveolar ridges. The size of the lesion can range from a few millimeters to several centimeters. Larger tumors can cause mechanical obstruction of the oral cavity, leading to polyhydramnios prenatally and feeding or respiratory problems postnatally.

Congenital epulis can be clinically differentiated from hemangiomas and lymphangiomas, which are "spongy" on palpation and have a red or dark blue surface, in contrast to CE, which is covered with normal oral mucosa. Several other differential diagnosis needs to be considered including teratoma, congenital dermoid cyst, congenital fibro sarcoma, leiomyoma, rhabdomyoma and heterotrophic gastrointestinal cyst. [6] CE can be easily identified and morphologically differentiated from these childhood oral lesions. Malignant alveolar sarcoma has a close resemblance to CE but has a characteristic malignant cytological feature and evident vascular invasion, which differentiates it from CE. CE has a very close similarity with GCT however histologically it can be distinguished from GCT due to the absence of single cell or infiltrating cluster of cell growth pattern also termed as neuroid spindling which are characteristic of GCT. [7] The etiology of this condition is unclear. Several theories have been suggested including myoblastic, odontogenic, neurogenic, fibroblastic, histiocytic and endocrinologic. Immunohistochemical studies have suggested a neuroectodermal derivation. [8] Though the histiogenesis of this entity is not certain, the cell phenotype of CE is related to myofibroblast/pericyte or myopericyte and is not epitheliomatous or smooth muscle derived, moreover these lesions tend to self-regress with absence of local recurrence even after incomplete excision hence suggesting these to be nonneoplastic, degenerative or reactive lesion. [9] The pattern of immunostaining appears diverse contributing to the enigma of histogenesis. However, CE has a negative S100protein as was seen in the case reported, which differentiates it with other GCT, which are supposed to have neural histogenesis and stains positive with S100 protein markers [Figure 3]. Although most cases are detected at birth, diagnosis is even possible in the third trimester of pregnancy using ultrasound scan or fetal magnetic resonance imaging. Such prenatal diagnosis is ideal and allows for better planning of the delivery and postnatal management. [10] Primary management includes surgical excision however small and medium sized CE (<20 mm) that do not compromise feeding or respiration can even be treated conservatively as these tend to regress spontaneously. [11] Most reports however suggest immediate surgical removal under general anesthesia, especially in case of problems with feeding or respiration. Recurrence of the lesion and damage to future dentition has not been reported suggesting that radical excision is not warranted.

CONCLUSION

Congenital epulis is a benign oral mass that is seen in neonates and are often significantly disfiguring henceforth parent's assurance is paramount. Prompt surgical excision is required in case of airway obstruction or difficulty with feeding. Simple excision is advisable hence knowledge of this entity helps in avoiding over diagnosis and preventing overjudicious surgeries.

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