congenital epulis is a rare lesion of the newborn , presenting as mass in the oral cavity which can interfere with respiration and feeding . it should be distinguished from other lesions which can occur in newborns , both clinically and histopathologically . here , we report a case of congenital epulis in a newborn female on the right alveolar ridge , along with an extensive review of literature and discuss the immunoprofiling . early diagnosis of ce in a newborn is of paramount importance in the successful management of these rare cases . congenital gingival granular cell tumor ( cgct ) of the newborn , also known as congenital granular cell lesion , congenital epulis , congenital myoblastoma ( historically ) , or neumann 's tumor , is a rare non - neoplastic lesion seen only in newborns ( 1 ) . it presents in the mouth most commonly in the maxillary alveolar ridge as a smooth - surfaced sessile or pedunculated mass with normal to reddish colour mucosa . it varies in size from several millimeters to few centimeters in diameter and can interfere with respiration or feeding . in recent years , prenatal detection of such oral lesions has facilitated the narrowing down of differential diagnosis and proper treatment planning through multidisciplinary approach . although , histopathologically this lesion shows similarity with granular cell tumour which occurs in adults , the two are separate entities with different histogenesis . a newborn female child was referred to our institute , immediately after delivery for examination of a mass protruding from her mouth . a round , soft pedunculated mass of 4 cm diameter , exhibiting a smooth erythematous surface was located on the right side of the maxillary alveolar ridge ( fig 1 ) . the mass prevented normal closure of the mouth and interfered with breastfeeding , but did not pose an immediate airway concern . lesion attached to maxillary alveolar ridge protruding from the mouth on the second day after birth , the tumor was completely resected by surgical excision following anaesthesia , and subjected to histopathological examination . the newborn recovered with no complications , and breastfeeding was initiated on the subsequent day of operation . the gross specimen measured 3.5 cm  3.5 cm  2 cm and was pink in color with a smooth surface and firm consistency . this tissue was processed for routine histopathological examination and embedded in paraffin.4 m - thick sections were cut from these paraffin - embedded tissue blocks and stained with hematoxylin and eosin . sections revealed lesional tissue comprising large sheets of polygonal or rounded cells with a centrally placed small dark basophilic nucleus with an abundant eosinophilic granular cytoplasm , abutting the overlying parakeratinized stratified squamous epithelium . the tissue was non - reactive to s-100 protein and cd68 ( fig 3 ) ; but reactive to vimentin . h&e stained sections showing stratified squamous epithelium and underlying tissue with granular cytoplasm immunostaining showing negative staining for cd68 congenital epulis of the new born is a widely accepted term and few prefer it over congenital granular cell tumor , which is suggestive of a neoplasm ( 4 ) . however , epulis is a non - specific term used to designate hyperplastic gingival tissue or gingival tumor masses . since there are cases which are not exclusively related to the gingiva , ( 1,3 ) seems that the term congenital granular cell lesion would be a more appropriate term ( 5).since its first description in 1871 in germany as  congenital epulis  by neumann ( 6 ) , over 200 cases of this rare lesion have been reported ( 2 ) . ce is usually seen at birth and has a site predilection for the maxillary alveolar process , lateral to the midline in the region of the primary canine and lateral incisor . less frequently , it has been reported in the mandibular alveolus , tongue and one case with involvement of alveolar ridge as well as the tongue ( 6 ) . ce usually occurs as a solitary lesion , although in 10% of the cases , it occurs as multiple masses ( 4,7 ) . it presents as a mass with a smooth normal colored surface , pedunculated , sometimes lobulated , and varying in size from a few millimeters to 9 cm ( 8) . ce is usually diagnosed at birth ; although , if the lesion is large , it may be diagnosed in utero by 3d ultrasound and magnetic resonance imaging ( mri ) examinations . in utero diagnosis is important in choosing the delivery method , since large lesions may compromise a normal vaginal delivery and a cesarean operation may be necessary ( 8) . although there are studies that affirm successful prenatal diagnosis of ce , these studies actually obtained images of the tumor mass , but the diagnosis could not be conclusive ( 5 ) . a list of differential diagnosis thus obtained is valuable in treatment planning and a multidisciplinary approach during delivery . the tumor is also postulated to originate from undifferentiated mesenchymal cells , fibroblasts , myofibroblasts , histiocytes , pericytes , schwann cells or odontogenic epithelial cells . few immunohistochemical study findings support a mesenchymal origin ( 3 , 5 , 10).ultrastructural studies showed presence of many autophagosomes containing collagen precursors , suggesting the tumor cells represent early mesodermal cells that express pericytic and myofibroblastic features that undergo cytoplasmic autophagocytosis ( 11 ) . there are usually no associated dental abnormalities or congenital malformations ( 2 ) , except for occasional reports of a hypoplastic or absent tooth and the possibility of mild midface hypoplasia ( 2,4 ) . ce has been reported in infants with polydactyly , goiter , triple  syndrome , maxillary hypoplasia , neurofibromatosis and polyhydraminos ( 2,12 ) . clinical differential diagnoses for congenital lesions of oral mucosa depend on site of involvement , size , velocity of growth , and possible accompanying lesions . this includes teratoma ( epignathus)(13 ) , hemangioma , fibroma , choristoma and hamartoma , melanotic neuroectodermal tumour of infancy , rhabdomyoma , rhabdomyoscarcoma , lymphangioma , osteogenic and chondrogenic sarcomas , and granular cell tumor ( 3,12,13 ) . however , some congenital lesions occur predominantly on the alveolar ridge and others on tongue , thus narrowing the list of possible differentials in a particular site . leiomyomatous hamartoma has the appearance of congenital epulis and is often seen on the median anterior alveolar ridge and the tip of the tongue ( 1 ) . they are two different entities which may be differentiated on histological and epidemiological grounds ( 14 ) . granular cell tumor is more commonly seen , with few reported cases of malignant transformation , whereas ce is a rare lesion with an incidence of 0.0006% ( 9 ) and no evidence of malignant transformation . ce occurs in neonates predominantly in females on the right side of the maxillary alveolar ridge , whereas , granular cell tumor occurs in adults on the tongue and a wide variety of visceral and cutaneous sites ( orbit , lung , mastoid , tongue , infra and supraglottic regions ) , with no sex predilection . granular cell tumour may show pseudoepitheliomatous hyperplasia often with squamous pearl formation in the epithelium and is less vascular with prominent nerve bundles . ultrastructurally , in ce , membrane bound granules or phagolysosomes are present in the cytoplasm , many of which contain collagen precursors , but angulate bodies are absent unlike in granular cell tumor ( 11 ) . immunohistochemical study shows no reactivity of lesional cells to s-100 protien , ngfr / p75 , and inhibin - alpha in ce but both ce and granular cell tumor have stained positive for macrophage markers like cd68 and ki - m1p . however , the statement is equivocal and few cases have demonstrated no reactivity to cd 68 ( 15 ) . in line with these cases ce also shows positive immunohistochemical staining to hla - dr antigen , vimentin , nki / c3 , and pgp9 and occasionally nse and cea(3,16,17,18 ) . although immunohistochemical profiling has not confirmed the cells of origin of this lesion , it has proved useful in confirming that ce is non - neoplastic and aids in differentiating it from granular cell tumor histologically ( 19 ) . the treatment of choice is surgical excision , when the lesion is obstructing feeding or respiration . it can be excised either under general anesthesia within hours to days after birth or local anesthesia where intubation is not possible or in cases of small lesions ( 2 ) . there is also the possibility of removal during the delivery , in cases where the lesion was detected during pregnancy ( 20 ) . this approach provides the newborn a free airway and an unobstructed oral cavity immediately after birth eliminating additional procedures such as anesthesia and intubation . surgical excision of ce using carbon dioxide laser and erbium , chromium : yetrium - scandium - galliumgarnet ( er , cr : ysgg ) laser have also been reported . there have been eight case reports that have documented spontaneous regression . in cases where there is no interference with feeding or respiration , regular monitoring of the lesion for regression has been advocated as an acceptable clinical approach . in our case , the lesion interfered with feeding and was thus excised at the earliest so to avoid any further dehydration in the newborn . ce has not recurred even after incomplete excision , and has no tendency for malignant transformation .
backgroundcongenital epulis is a rare lesion of the newborn , presenting as mass in the oral cavity which can interfere with respiration and feeding . 
 it should be distinguished from other lesions which can occur in newborns , both clinically and histopathologically.case detailshere , we report a case of congenital epulis in a newborn female on the right alveolar ridge , along with an extensive review of literature and discuss the immunoprofiling.conclusionearly diagnosis of ce in a newborn is of paramount importance in the successful management of these rare cases .