| Abstract|| |
Congenital epulis of the newborn is a very rare and unique tumor first described in 1871 by Neuman. It has a female predilection. It is a tumor with no tendency to recur after excision. The histogenesis of the lesion is unknown, but it is believed to be of mesenchymal origin. We report a 2-day-old female with tumor mass on the anterior mandibular alveolar ridge, which demonstrated immunoreactivity for vimentin, S-100 and neuron-specific enolase; thus, suggesting a similar histogenesis with granular cell tumor.
Keywords: Alveolar ridge, congenital epulis, female, granular cell, immunohistochemistry
|How to cite this article:|
Adeyemi BF, Oluwasola AO, Adisa AO. Congenital epulis. Indian J Dent Res 2010;21:292-4
Congenital epulis of the newborn (CENB), also commonly referred to as gingival granular cell tumor (GCT), is a rare and unique tumor described by Neumann in 1871. One hundred and sixty-seven cases have been reported, with a maxilla to mandible ratio of 3:1 and a female to male ratio of 10:1. , CENB is an intriguing lesion with unclear etiology, histogenesis and natural history. Several theories have been proposed to explain its histogenesis, such as the myoblastic, odontogenic, neurogenic, histiocytic and endocrinologic origin. , The treatment of choice is surgical excision, although a few cases of spontaneous regression have been reported; however, this occurred in lesions that were very small in size. , This case is being reported in view of the rarity of CENB and its immunoreactivity for S-100 protein.
| Case Report|| |
A 2-day-old female neonate presented at the children emergency ward of our hospital on account of a congenital intraoral mass that was noticed by the parents at birth. The lesion prevented the patient from feeding but did not interfere with respiration. There was no significant change in the size of the swelling after birth. Medical history indicated a full-term, uneventful pregnancy with no attendant complications during delivery. There was no positive family history of congenital anomaly.
Clinical examination revealed a full-term female neonate with a huge bilobed, exophytic growth protruding from the oral cavity [Figure 1]; the lobes measured approximately 5 cm x 3 cm x 2 cm and 2 cm x 2 cm x 1 cm in size. The tumor was attached by a pedicle to the anterior mandibular ridge. The lesion appeared erythematous with few focal areas of discoloration and isolated points of minimal bleeding. The mass was firm in consistency and was not tender to digital palpation. There was no other congenital anomaly.
Surgical excision with wide base resection was performed under general anesthesia without a prior incisional biopsy. The surgery was performed on the infant's third day of life mainly to allow feeding, which was being hampered by the mass. The surgical procedure was well tolerated by the baby and there was very minimal intraoperative bleeding. Postoperative recovery and surgical site healing was satisfactory. The patient was discharged home on the fifth postoperative day.
The excised mass was fixed in 10% neutral buffered formalin. The tissue was submitted for histopathological examination. Immunohistochemical analysis were also carried out using a panel of antibodies, including vimentin, desmin, cytokeratin AE1/AE3, S-100 and neurone-specific enolase (NSE). Positive and negative controls were performed in parallel for all the antibodies tested.
Microscopic examination showed a benign tumor composed of sheets of closely packed, large, polygonal cells, with abundant eosinophilic granular cytoplasm and small, central basophilic nuclei. The lesion was unencapsulated and highly vascularized [Figure 2]. The overlying mucosa showed a well-differentiated stratified squamous epithelium. The tumor stained diffusely and strongly for vimentin and NSE, focally and weakly positive for S-100 and negatively for desmin, and cytokeratin AE1/AE3 [Figure 3].
| Discussion|| |
The female gender of the patient presented in this case report is consistent with the sex predilection of CENB. An endogenous hormonal influence has been proposed to explain this gender bias, but this has not been supported by detectable estrogen and progesterone receptors within the lesion.  Another theory to explain this female preponderance is the possibility of an intrauterine stimulus from the fetal ovaries.  The lesion was attached by means of a thick pedicle to the alveolar ridge in the region of the future incisors. This is in consonance with most cases that are pedunculated; however, a few sessile cases have been reported. About 90% of CENB are solitary, as found in this neonate.  This patient had no other congenital anomaly, not even the occasional midfacial hypoplasia and anomalies of the nasal complex that is sometimes seen in some cases due to pressure effect from the lesion on the developing maxilla. 
The lesion was excised under general anesthesia because it was initially thought to be a malignant neoplasm. Reported cases have been excised under local anesthesia as well as under general anesthesia. ,, CENB is not prone to recurrence even if some tissue is left; no case of recurrence has been documented in the literature.  The lesion was very striking and impressive, being large and having multiple focal areas of discoloration, giving an impression of a soft tissue sarcoma, which informed the wide base surgical excision.
The diagnosis of CENB should essentially be clinical, but could pose some difficulty as seen in this case because of the low level of suspicion.  CENB is very rare, but it is important that dentists be able to recognize them as they may be asked to consult and provide crucial information on patient management as well as allay the anxiety of the parents. Polyhydramnios is common in patients with congenital epulis due to swallowing difficulties as a result of the size of the growth, but the mother did not attend antenatal clinics and gave birth at home. 
CENB is very similar to GCT histologically, but differs from it epidemiologically as well as in clinical behavior. It is seen exclusively on neonatal gingivae, presenting at birth, having a marked predilection for females, while GCT is rare in the first decade of life, being most frequently diagnosed between the third and sixth decades of life, and affecting a wide variety of visceral and cutaneous sites, also having a predilection for the female gender. ,
In contrast with GCT, CENB may occasionally undergo spontaneous regression and has no malignant variant. In addition, pseudoepitheliomatous hyperplasia does not occur in CENB. , However, both lesions are characterized by large polygonal cells with abundant eosinophllic granular cytoplasm and small basophilic nuclei.  CENB is thought to be a reactive or hamartomatous lesion of uncertain histogenesis unlike GCT, which is a neoplastic growth of neural origin. ,,
However, a particularly interesting finding in this study is the demonstration of strongly positive immune staining with NSE and focal weakly positive staining for S-100 protein. It is conventionally accepted that whereas GCT usually demonstrates strong staining for S-100, CENB is usually negative. This has been used to infer that both tumors have different histogenetic origins.  Similar to our finding of S-100 positivity, a few workers have also observed the same reaction. , The detection of positive staining for NSE and S-100 protein in this case of CENB seems to give credence to the possibility of similar histogenesis to GCT.
This case is similar to several others in being found at birth in a female baby but located on the mandibular ridge, which is the less common site for it. , Histopathological appearance is like the majority of cases in consisting of large eosinophillic granular cells supported by scant collagenous stroma. Unlike the majority of cases, this lesion demonstrated positive staining for S-100 protein. ,, In summary, we present a case of CENB in a female neonate. The lesion demonstrated immunohistochemical profile, which suggests that CENB and GCT might have a similar histogenesis.
| Acknowledgments|| |
The authors are grateful to Prof. EEU Akang of the Department of Pathology for editing the script and Mr. A Odetunde of the Institute of Advanced Medical Research and Training, College of Medicine, University of Ibadan, for assistance with the photomicrographs.
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Bukola F Adeyemi
Department of Oral Pathology, College of Medicine, University of Ibadan
[Figure 1], [Figure 2], [Figure 3]