|Year : 2014 | Volume
| Issue : 4 | Page : 220-222
Congenital epulis: A rare lesion
Divya Gupta1, Shasanka Shekhar Panda2, Devendra Kumar Yadav1, Basant Kumar1, Deepak Bagga1, Samir Kant Acharya1
1 Department of Pediatric Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
2 Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||14-Nov-2014|
Devendra Kumar Yadav
Department of Pediatric Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Congenital epulis, a rare benign soft tissue tumour from the alveolar crest is smooth-surfaced, pedunculated and lobulated. It is almost always located on the gingiva and is found predominantly on the maxilla. It can obstruct the oral passage by its size and induce airway obstruction and feeding problems. Spontaneous involution after birth is rare. Newborns with a diagnosis of congenital epulis should be immediately subjected to the simple conservative surgical excision to restore vital functions, improve quality-of-life and decrease parental anxiety. Wide radical excision is not recommended as no recurrences have been described even after incomplete excision. The parents of the newborn with congenital epulis should be assured of this benign pathology and the simple treatment. This report documents the presentation and management of a congenital epulis of the maxillary alveolar ridge found in a newborn female and treated with simple conservative surgical excision.
Keywords: Alveolar ridge, congenital epulis, conservative surgery, intraoral tumor of newborn
|How to cite this article:|
Gupta D, Panda SS, Yadav DK, Kumar B, Bagga D, Acharya SK. Congenital epulis: A rare lesion. J Clin Neonatol 2014;3:220-2
| Introduction|| |
Congenital epulis is a rare benign hamartoma of the alveolar ridge found in the newborn, also known as congenital gingival granular cell tumor or Neumann's tumor, first described by Neumann in 1871. , The Greek word "epulis" means "swelling on the gingiva."  It is extremely rare intraoral tumor of the newborn and is much more common in females (8 to10:1) with a Caucasian predisposition. ,, Usually, it arises from the anterior part of the maxillary alveolar ridge but may occur in the mandible, maxilla or tongue. ,, Epulis is seen only in the newborn and is thought to be a different entity from other adult granular cell tumors. ,
| Case report|| |
A 1 day old, full term, vaginal delivered, female baby weighing 2500 g was referred immediately after delivery for examination of a mass protruding from her mouth [Figure 1]a with difficulty to feed. There was no respiratory distress, but the child had problems in sucking. On examination, a firm, smooth, nontender mass measuring 4 cm × 4 cm was present arising from the left upper alveolar margin with a broad base [Figure 1]a. Palate was normal. The mass prevented normal closure of the mouth and interfered with breastfeeding. The mass posed no immediate airway concerns. Feeding by a nasogastric tube was instituted. No family history of hereditary diseases was reported.
|Figure 1: (a) A smooth and lobulated mass protruding from the left upper alveolar margin with a broad base (b) no significant residual lesion after surgery without any problem of opening of mouth|
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An ultrasound performed in the 28 th week of gestation showed no abnormalities. Hematological and biochemical blood analyses, ultrasound of the head and the abdomen were within normal limits. Ultrasonography with Doppler of the mass showed a nonhomogeneous, solid, space-occupying lesion without significant vascularity.
Total excision of the mass was done by the use of cautery under general anesthesia and nasal endotracheal intubation. There was minimal intraoperative hemorrhage.
Postoperative period was uneventful. There was no significant residual lesion [Figure 1]b without any problem of closing and opening of the mouth. Oral feeding (expressed breast milk) was initiated immediately after surgery and was well tolerated. The infant was able to breastfeed on the 4 th day after surgery and was discharged with her mother on the 7 th day. Histopathology of the resected specimen showed mucosa with sheets of large granular cells with centrally placed round nuclei, consistent with congenital epulis [Figure 2]a and b.
|Figure 2: (a) Photomicrograph showing an overlaying stratified squamous epithelium and vascularized stroma with benign proliferation of round cells (b) photomicrograph showing clusters of plump, polygonal cells with granular eosinophlic cytoplasm and small uninform basophilic nuclei (H and E, ×400)|
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Patient was gaining weight and thriving well at first follow-up 2 weeks after surgery. After 9 months, child was doing well without recurrence. There is no problem in dentition.
| Discussion|| |
Congenital oral tumors are commonly recognized at birth or just after birth except in instances where the tumor is very small and causing no obvious symptoms. Clinically, lesions of the oral mucosa are divided into surface lesions and soft tissue enlargements, which are either reactive enlargements or tumors. Tumors can be solid or cystic, benign or malignant, and congenital or noncongenital. In utero, obstruction of the oral cavity can result in ineffective swallowing and can cause polyhydramnios.  Postnatally, clinical manifestations depend on the size and location of the lesions and include respiratory insufficiency, difficulty in sucking and swallowing and/or inadequate closure of mouth.  The diagnosis is usually clinical, although difficulties may occur when the index of suspicion is low or when the origin of the tumor is hard to determine. In such cases, the differential diagnosis is wide and imaging has a contributing role to play. Differential diagnosis includes granular cell tumor, hemangioma, fibroma, granuloma, dermoid cyst or teratoma, encephalocele, embryonal rhabdomyosarcoma, malignant granular cell myoblastoma, alveolar rabdomyosarcoma, chondrogenic and osteogenic sarcoma, schwannoma.
It may or may not be associated with other congenital anomalies. , In general, it occurs as solitary lesion but multiple lesions may also occur in up to 10% of cases. , Occurrence is sporadic and no familial tendencies have been described. , The etiology is unknown and there is still controversy regarding exact cell of origin.  An undifferentiated mesenchymal cell origin, fibroblastic and myoblastic, histiocytic, odontogenic, neurogenic, endothelial and endocrinologic etiologies had been proposed but most of the reported cases support a mesenchymal origin. 
Congenital epulis is rarely diagnosed prenatally, but prenatal imaging by ultrasonography or magnetic resonance imaging (MRI) is possible.  Postnatally computed tomography or MRI of the head is useful in demonstrating the extent and differential diagnosis of congenital maxillofacial mass lesions and for planning surgical treatment.  The striking predilection of this tumor for female infants (10:1) suggests the presence of an endogenous hormonal stimulus in utero, however, estrogen, and progesterone receptors have not been detected. , The clinical course suggests a degenerative or reactive, rather than a neoplastic etiology. , Malignant transformations, local recurrences, malocclusion of the jaws and damage to future dentition have not been reported. ,,
Spontaneous involution after birth is rare but reported in literature and therefore conservative treatment is sometimes sufficient. ,, When the lesion is obstructing feeding or respiration, surgical removal is indicated. Simple conservative surgical excision at the tumor base should be done to allay the parent agitation. Wide radical excision is not recommended. There are no reports of local recurrence after incomplete excision. , In our case, there was no local recurrence of the tumor 9 months after local excision despite positive surgical margins after the procedure. Therefore, a conservative and nonmutilating approach is preferred. There are also reports of conservative surgical removal with CO 2 laser.  We do not recommend "watchful waiting" as spontaneous involution is very rare. Although this lesion is rare, but every practitioner should be aware of this so that they can tell the parents that despite its dangerous look, it is easy to treat this lesion without any residual impacts.
| Conclusion|| |
Congenital epulis is a rare benign tumor that most often presents on the maxillary or mandibular alveolar ridges. Newborns with a diagnosis of congenital epulis should be immediately subjected to the simple conservative surgical excision to restore vital functions and improve quality-of-life. No recurrences have been described even after incomplete excision. Therefore, wide surgical excision is unnecessary. The family of an infant with congenital epulis should be assured of the benign nature and the simple treatment of the condition.
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[Figure 1], [Figure 2]