Congenital Granular Cell Tumor – A Rare Entity

Monal Yuwanati*, 1; Shubhangi Mhaske1; Ashok Mhaske2

1. Dept of Oral Pathology and Microbiology, Peoples Dental academy, Bhopal , 2. Dept of General Surgery, Peoples College of Medical Sciences, Bhanpur bypass road, Bhopal

Correspondence: *. Correspondence: Dr. Monal Yuwanati, MDS (Oral Pathology), Reader, Department of Oral Pathology, Peoples Dental Academy, Peoples Campus, Bhanpur, Bhopal Bhopal-462037, Madhya Pradesh (MP), India. E-mail: E-mail:


Congenital granular cell tumor is a rare benign neoplastic growth affecting the gingival mucosa of neonates. Prenatal ultrasound diagnosis has recently come to focus and in spite of several reports on immune-histochemical and other advanced marker studies, the cause and origin of the lesion remains debatable till date. Review of literature on prenatal diagnosis and histopathology along with immunohistochemistry is discussed.

Received: 2015 March 21; Accepted: 2015 March 27

J Neonat Surg. 2015 Apr 1; 4: 17


Copyright JNS 2015

Keywords: Neumann’s tumor, Congenital granular cell Myoblastoma, Granular cell fibroblastoma, Congenital granular cell epulis, Congenital epulis.


Congenital Epulis (CE), or congenital granular cell tumour (CGCT), is a congenital benign rare tumour of the newborn. The original first description of the lesion was dated in 1871 by Neumann [1]; approximately 250 cases have been reported since then [2]. Other terminologies for CGCT described in literature are congenital epulis, granular cell rabdomyoma, congenital myoblastoma, or Neumann’s tumour. The term 'epulis' has been used, but this simply means swelling on the gingival. It was suggested to be discontinued and congenital granular cell tumour to be used in the literature. Currently “congenital epulis of newborn” is universally accepted and frequently employed terminology in the literature [3].

The congenital gingival granular cell tumour is most frequently seen on the alveolar median ridge of the maxilla than alveolar ridge of mandible (1:3). Incisor-canine area is commonly affected. It has a female preponderance. Incidence rate of the CGCT is unknown but suggested to be 6 per million [4]. The clinical diagnosis of CGCT is almost obligatory when a solitary tumour of the gingiva is present at birth. The presentation is unique at birth as fibrous mass arising from the gingival mucosa or alveolar ridge of the maxilla or mandible. CGCT appears sporadically and has no familial tendency [5]. It most commonly originates from the anterior alveolar ridge, with the maxilla being involved twice as much as the mandible [6], though the lesion can arise from the tongue, palate, skin, the subcutaneous tissue, skeleton muscles, the vocal chords, and in small number cases from the rest of the body. This pedicular tumor has a smooth or lobular surface and a firm, rubbery consistency [7]. Ultrasonography confirms diagnosis as early as at 26 weeks of gestation or in third trimester of pregnancy [8]. The histogenesis of CGCT has been long debatable, as various authors suggested different source of origin of the tumour. The proposed source of origin includes undifferentiated mesenchymal cells [9], odontogenic epithelial, pericytic, and fibroblastic, histiocytes, nerve-related, smooth muscle, and primitive mesenchymal cells [10]. The histogenesis remains still unclear despite several studies. Though spontaneous regression of the lesion has been reported [11, 12], surgical excision is the only valuable therapeutic option which can be beneficial to both mother and newborn. Recurrence or malignant transformation is not yet mentioned in available literature.


Typically, a single tumour is present (90%), ranging in size from several millimetres to several centimetres [13]. Multiple lesions (10%) has been reported involving either or both jaws and have been described with associated abnormalities of the nasal bridges and septum [13-15]. Classically, it arises from the pre-incisor-canine area [11]. Literature survey has suggested that maxilla as most common site of involvement [Fig. 1].

[Figure ID: F1] Figure 1: Site distribution

Prenatal Diagnosis

Prenatal imaging of congenital lesions of oral cavity is possible by ultrasound (USG) or Magnetic resonance Imaging (MRI) and can be helpful in planning the delivery as well as postnatally in demonstrating the congenital lesion and treatment planning. With the help of Ultrasonography or MRI, tumor mass can be identified, mainly in last weeks of pregnancy [16]. Ultrasonography or even MRI can only show the presence of tumor mass suggestive of epulis, but the diagnosis could not be conclusive. However, few studies indicated in past has state successful prenatal diagnosis of congenital epulis using ultrasonography [5]. Postnatally, it may interfere with feeding [14], or respiration. Prenatal diagnosis of CGCT can aid in counselling the parents as to the nature and treatment of the abnormality, as well as the potential risk of airway obstruction and intraoperative complications. Prenatal ultrasound investigation is important, since large tumours may interfere with vaginal delivery and a caesarean may be required [5, 11, 13]. The discovery of a tumor mass on a foetus may help the intervention planning as well as to prepare the parents mentally for eventual measures that may occur in the newborn. Prenatal diagnosis of the lesion on ultrasonography was reported in the literature as early as 26 to 38 weeks [Table 1] which is mostly coincide with third trimester of the pregnancy. It’s very striking that lesion is not detectable till that period, reason for this are unclear. Kusukawa et al. have demonstrated a positive correlation between tumor size and estimated fetal weight [17].


It has unique female preponderance [Table 1, 2 and Fig. 2]. Based on the theory supported by the experimental production of CGCT in a mouse following injection of endogenous hormone, this characteristic finding was suggested to be attributable to presence of endogenous hormones and sudden regression of mass after birth [14]. This was subsequently failed proved as there was no detectable presence of these endogenous hormone receptors with the lesion tissue. Trauma, due to finger sucking in utero, was suggested not considered significant [11, 12, 18]. Recently, it was suggested that it may be a local metabolic or reactive change [19].

Table 1Supplementary PDF file supplied by authors.
Table 2Supplementary PDF file supplied by authors.

[Figure ID: F2] Figure 2: Gender distribution

It most commonly presents as an isolated finding with no known association with other congenital anomalies though reported to be associated with neurofibromatosis, polydactyly, binder syndrome, congenital goiter [20], and bilateral transverse facial cleft [21]. Rarely, shows association with absence of underlying tooth/germ. Majority prenatal associated complications reported are polyhydramnios, obstructed deglutition of amniotic fluid whereas postnatally causes midface hypoplasia, incisor hypoplasia, severe feeding and respiratory/aesthetic problems. As there is potential risk of neonatal respiratory distress, it was suggested that prenatal diagnosis becomes prerequisite for uneventful pregnancy. Ultrasonography is crucial in such cases.

Differential Diagnosis

Diagnosis of congenital epulis can usually be made on characteristic clinical findings. Clinical differential diagnosis (D/D) is broad based and is depends upon site of involvement, size velocity of growth and possible accompanying lesion or other developmental anomalies like Epstein pearls, granular cell tumour, vascular malformations and neuroectodermal tumours of infancy.

Predominance of female patients, tumor location on maxillary anterior region, presence at birth and absence of growth potential and the possibility of spontaneous remission without any intervention could rule out majority of the D/D. Various lesions that can be considered prenatally are congenital malformations, dermoid cyst, haemangioma, lymphatic malformations, melanotic pigmentation neuroectodermal tumours of infancy, rhabdomyosarcoma, granular cell tumor, oral teratoma-epignathus as well as other possible diagnoses such as fibroma, lipoma, leiomyoma, rhabdomyoma, peripheral giant cell granuloma, pyogenic granuloma, neurofibroma, myxoma, hemangioma, lymphangioma, and alveolar lymphangioma [10]. The location and sonographic appearance aid in differentiating these masses. Teratomas often contain calcifications. Hemangiomas arise externally from subcutaneous tissues and may be solid or cystic in appearance. The accurate diagnosis has been further complicated by its similarity, histologically, with the Granular cell tumor/ myoblastoma (GCT), which occurs in adults at a number of intraoral sites, such as tongue. CGCT can be separated from Granular cell tumor (GCT) by location, patients age, absence of cytoplasmic hyaline granules, solid growth pattern, pericytic proliferation, attenuated overlying epithelium, negativity of lesion tissue to S- 100 [Table 2, 3 and Fig. 3]. d.

[Figure ID: F3] Table 3: Difference between the Adult granular cell tumor and congenital Granular cell tumor [24]

[Figure ID: F4] Figure 3: Immunohistochemistry marker used in congenital Granular cell tumor cases


The immunohistochemistry of the tumor is diverse in newborns and adults. Congenital granular cell tumor/epulis is S-100 negative and does not show differentiation to specific cell type [4, 19]. Possible histological origins of the epulis may include epithelial and undifferentiated mesenchymal cells, pericytes, fibroblasts, smooth muscle, nerve-related cells, and myofibroblasts [22]. It has been suggested to be a nonneoplastic, degenerative, or reactive lesion [23].


Characteristic histological findings shown by these congenital epulides include large round cells with granular, eosinophilic cytoplasm and small eccentric nuclei and a delicate fibrovascular network separating the cells. Histopathologically, it known that congenital epulis consist of granular cell and is similar to the adult granular cell tumor, but there are some differences such as pseudo-epithelialomatous hyperplasia, lesser vascularity, more conspicuous nerve bundles than congenital epulis [Table-3] [24]. Pseudoepitheliomatous hyperplasia of the oral mucosa on the tumor surface is seen in 50 % of adult GCT cases. but is usually absent in CGCT cases. Spindle cell variant of granular cell Cases with accompanying pseudo-epitheliomatous hyperplasia or ulceration may mimic squamous cell carcinoma [25].


Treatment is with surgical excision; with few cases of spontaneous regression has been reported [26]. After surgical removal, no recurrence or malignant changes have been reported even after incomplete excision, and spontaneous regression may occur [12]. Any Delay in operation can lead to airway obstruction and feeding difficulty. The tumour should be removed during the immediate postnatal period and is usually achieved without serious consequences. If the lesion is smaller conservative approach can be taken to avoid unwanted surgery. Prognosis in CGCT is good as there no reported case of recurrence even after the incomplete removal of lesion [10].


The main areas of controversy surrounding the congenital epulis remain with the exact aetiology, growth, and progression of the lesion in the foetus. The lesion still poses scope for further research in exact etiopathogenesis. Prenatal Diagnosis is an important aspect to be considered specifically in this entity. Treatment planning should be based on the prenatal diagnosis, size of the lesion etc.


Source of Support: Nil

Conflict of Interest: None

1. E. Neumann. “Ein fall von kongenitaler epulis,” Arch Heilkd. 1871; 12:189–90.
2. Tokar B, Boneval C, Mirapoğlu S, Tetikkurt S, Aksöyek S, Salman T, et al. Congenital granular-cell tumor of the gingiva. Pediatr Surg Int. 1998; 13:594-6.
3. Ruschel HC, Beilke LP, Beilke RP, Kramer PF. Congenital epulis of newborn: report of a spontaneous regression case. J Clin Pediatr Dent. Winter 2008; 33:167-9.
4. Bosanquet D, Roblin G. Congenital epulis: a case report and estimation of incidence. Int J Otolaryngol. 2009; 2009:508780.
5. Kumar P, Kim HH, Zahtz GD, Valderrama E. Steele AM. Obstructive congenital epulis: prenatal diagnosis and perinatal management. Laryngoscope. 2002; 112:1935-9.
6. Kim SK, Won HS, Lee SW, Kim JK, Shim JY, Lee PR, Kim A. Prenatal diagnosis of congenital epulis by three-dimensional ultrasound and magnetic resonance imaging. Prenat Diagn. 2006; 26:171-4.
7. Dash JK, Sahoo PK, Das SN. Congenital granular cell lesion “congenital epulis”- Report of a case. J Indian Soc Prev Dent. 2004; 22:63-7.
8. Nakata M, Anno K, Matsumori LT, Sumie M, Sase M, Nakano T, Hara H, et al. Prenatal diagnosis of congenital epulis: a case report. Ultrasound Obstet Gynecol. 2002; 20:627-9.
9. Mirchandani R, Sciubba JJ, Mir R. Granular cell lesions of the jaws and oral cavity: a clinicopathologic, immunohistochemical and ultra structural study. J Oral Maxillofac Surg. 1989; 47:1248-55.
10. Fister P, Volavsek M, Novosel Sever M, Jazbec J. A newborn baby with a tumor protruding from the mouth. Diagnosis: congenital gingival granular cell tumor. Acta Dermatovenerol Alp Pannonica Adriat. 2007; 16:128-30.
11. Koch BL, Myer C, Egelhoff JC. Congenital epulis. Am J Neuroradiol. 1997; 18:739–41.
12. Kershisnik M, Batsakis JG, Mackay B. Granular cell tumors. Ann Otol Rhinol Laryngol 1994; 103:416–9.
13. Wittebole A, Bayet B, Veyckemans F, Gosseye S, Vanwijck R. Congenital epulis of the newborn. Acta Chir Belg. 2003;103:235-7.
14. Jenkins HR. Spontaneous regression of congenital epulis of the newborn. Arch Dis Child. 1989; 64: 145-7.
15. Rainey JB, Smith IJ. Congenital epulis of the newborn. J Pediatr Surg. 1972: 19: 305-6
16. Song WS, Kim JW, Kim YG, Ryu DM. A case report of congenital epulis in the fetus. J Oral Maxillofac Surg. 2005; 63:135-7.
17. Kusukawa J, Kuhara S, Koga C, Inoue T. Congenital granular cell tumor (congenital epulis) in the fetus: a case report. J Oral Maxillofac Surg. 1997; 55:1356-9.
18. Leocata P, Bifaretti G, Saltarelli S, Corbacelli A, Ventura L. Congenital (granular cell) epulis of the newborn: a case report with immunohistochemical study on the histogenesis. Ann Saudi Med. 1999; 19:527–9.
19. Vered M, Dobriyan A, Buchner A. Congenital granular cell epulis presents an immunohistochemical profile that distinguishes it from the granular cell tumor of the adult. Virchows Arch. 2009; 454:303-10.
20. Godra A, D’Cruz CA, Labat MF, Isaacson G. Pathologic quiz case: a newborn with a midline buccal mucosa mass. Congenital gingival granular cell tumor (congenital epulis). Arch Pathol Lab Med. 2004 May; 128:585-6.
21. JM Su, JM Wang, WZ Gu, Congenital granular cell tumour in a newborn: a case report and literature review in China. HK J Paediatr 2010; 15:165-9.
22. Damm DD, Cibull ML, Geissler RH, Neville BW, Bowden CM, Lehmann JE. Investigation into histogenesis of congenital epulis of the newborn. Oral Surg Oral Med Oral Pathol. 1993; 76:205-12.
23. Roher MD, Young SK. Congenital epulis (gingival granular cell tumor): ultra structural evidence of origin from pericytes. Oral Surg Oral Med Oral Pathol. 1982; 53-6.
24. Kannan SK, Rajesh R. Congenital epulis - congenital granular cell lesion: a case report. J Indian Soc Pedod Prev Dent. 2006; 24:104-6.
25. Regezi JA, Sciubba JJ, Jordan RCK. Oral pathology: clinical pathologic correlations. 5th ed., St. Louis, Elsevier Saunders, 2008: 168-70.
26. Sakai VT, Oliveira TM, Silva TC, Moretti AB, Santos CF, Machado MA. Complete spontaneous regression of congenital epulis in a baby by 8 months of age. Int J Paediatr Dent. 2007; 17:309-12.
27. Charrier JB, Droulle P, Vignaud JM, Chassagne JF, Stricker M. Obstructive congenital gingival granular cell tumor. Ann Otol Rhinol Laryngol. 2003; 112:388-91.
28. Adeyemi BF, Oluwasola AO, Adisa AO. Congenital epulis. Indian J Dent Res 2010; 21:292-4.
29. Hasanov A, Musayev J, Onal B, Rahımov C, Farzaliyev I. Gingival granular cell tumor of the newborn: a case report and review of literature. Turk Patoloji Derg.2011; 27:161-3.
30. Pellicano M, Zullo F, Catizone C, Guida F, Catizone F, Nappi C. Prenatal diagnosis of congenital granular cell epulis. Ultrasound Obstet Gynecol. 1998; 11:144–6.
31. Pinto JGS, Gassen HT, Piazza JL, Hernandez PAG, Isolan TMP, Silva Jr AN. A rare case of congenital epulis of the newborn with multiple lesions. Rev. odonto ciênc. 2008; 23:403-6.
32. Sigdel B, Baidya R, Shrestha P, Shrestha S. Congenital granular cell epulis: a rare diagnosis. J Path Nepal. 2011; 1:69-72.
33. Olson JL, Marcus JR, Zuker RM. Congenital Epulis. J Craniofac Surg. 2005;16:161-4.
34. Childers EL, Fanburg-Smith JC. Congenital epulis of the newborn: 10 new cases of a rare oral tumor. Ann Diagn Pathol. 2011; 15:157--61.
35. Tellado MG, Míguez JM, Gallart TM, Rey JL, Montero M, Pineiro EP, et al. Tumor gingival benigno de células granulares en el recién nacido: epulis congénito. An Esp Pediatr. 1996; 45:519-21.
36. Abo-Hager EA, Khater DS, Ahmed MM. Exploration of the Histogenesis of Congenital Granular Cell Epulis: An Immunohistochemical Study. J Egyptian Nat Cancer Inst. 2009; 21: 77-83.
37. Rehman MU, Khanani MF, Beckdache G, Durrani NUR, Jamil A, Rehmani A, et al. Congenital epulis. J Coll Physicians Surg Pakistan. 2012; 22: 56-7.
38. Lapid O, Shaco-Levy R, Krieger Y, Kachko L, Sagi A. Congenital epulis. Pediatrics 2001;107 (2):E22.
39. Dhingra M, Pantola C, Agarwal A. Congenital granular cell tumor of the alveolar ridge. Indian J Pathol Microbiol 2010; 53:327-8.
40. Okina T, Esaki K, Nagata A, Kusukawa J, Koga C, Nagao Y, et al. Congenital epulis: an electron microscopic observations of two cases. Kurume Med J. 1990; 37:293-9.
41. Anderson PJ, Kirkland P, Schafler K, Moss AL. Congenital gingival granular cell tumour. J R Soc Med. 1996;89:53P-4P.
42. Dzieniecka M, Komorowska A, Grzelak-Krzymianowska A, Kulig A. Multiple congenital epuli (congenital granular cell tumours) in the newborn: a case report and review of literature. Pol J Pathol. 2011; 62:69-71.
43. Kayiran SM, Buyukunal C, Ince U, Gürakan B. Congenital epulis of the tongue: A case report and review of the literature. JRSM Short Rep. 2011; 2:62.
44. Prigkos AC, Nikolakis MD, Kyriakopoulos VF, Tosios KI. Spindle cell epulis in an 8-month-old child: a histologic variant of congenital granular cell epulis? Head Neck Pathol. 2012;6:467-70.
45. Inan M, Yalçin O, Pul M. Congenital fibrous epulis in the infant. Yonsei Med J. 2002; 43:675-7.
46. Filie AC, Lage JM, Azumi N. Immunoreactivity of S100 protein, alpha-1-antitrypsin, and CD68 in adult and congenital granular cell tumors. Mod Pathol. 1996; 9:888-92.
47. Kaiserling E, Ruck P, Xiao JC. Congenital epulis and granular cell tumor: a histologic and immunohistochemical study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995; 80:687-97.
48. Takahashi H, Fujita S, Satoh H, Okabe H. Immunohistochemical study of congenital gingival granular cell tumor (congenital epulis). J Oral Pathol Med. 1990; 19:492-6.
49. Junquera LM, de Vicente JC, Vega JA, Losa JL, Albertos JM, López-Arranz JS. Granular-cell tumours: an immunohistochemical study. Br J Oral Maxillofac Surg.1997; 35:180-4.
50. Messina M, Severi FM, Buonocore G, Molinaro F, Amato G, Petraglia F. Prenatal diagnosis and multidisciplinary approach to the congenital gingival granular cell tumor. J Pediatr Surg. 2006; 41(10): E35-8.
51. Majid ZA, Siar CH, Ling KC. Congenital fibrous epulis: a case report. Med J Malaysia. 1986; 2:179 - 82.
52. Hoyme HE, Musgrave SD Jr, Browne AF, Clemmons JJ. Congenital oral tumor associated with neurofibromatosis detected by prenatal ultrasound. Clin Pediatr (Phila). 1987; 26:372-4.
53. Raissaki MT, Segkos N, Prokopakis EP, Haniotis V, Velegrakis GA, Gourtsoyiannis N. Congenital granular cell tumor (epulis): postnatal imaging appearances. J Comput Assist Tomogr. 2005; 29:520-3.
54. McGuire TP, Gomes PP, Freilich MM, Sándor GK. Congenital epulis: a surprise in the neonate. J Can Dent Assoc 2006; 72:747–750.
55. McMahon MG, Mintz S. In utero diagnosis of a congenital gingival granular cell tumor and immediate postnatal surgical management. J Oral Maxillofacial Surg. 1994; 52:496–8.
56. Lopez de Lacalle JM, Aguirre I, Irizabal JC, Nogues A. Congenital epulis: prenatal diagnosis by ultrasound. Pediatr Radiol. 2001; 31:453–54.
57. Szlachetk, Lemcke-Berno EM, Ozcan T. Prenatal diagnosis of a rare gingival granular cell tumor of the fetal mouth (clinical letter). J Ultrasound Med. 2012; 31:123–9.
58. Bornstein E, Boozarjomehri F, Monteagudo A, Santos R, Milla SS, Timor-Tritsch IE. Diagnostic and prognostic aspects in the sonographic evaluation of a fetus with an oral mass. J Ultrasound Med. 2009;28:689-3.
59. Lu W, Hu JG, Yu L, Xing FZ, Huang XY. Congenital granular cell epulis in the newborn. Chin J Pediatr Surg (Chin) 2007; 28: 335-6.
60. Shaw L, Al-Malt A, Carlan SJ, Plumley D, Greenbaum L, Kosko J. Congenital epulis: three-dimensional ultrasonographic findings and clinical implications. J Ultrasound Med. 2004; 23:1121-4.
61. Thoma V, Idrissi B, Kohler M, Becmeur F, Viville B, Favre R. Prenatal diagnosis of congenital epulis. A case study. Fetal Diagn Ther. 2006; 21:321-5.
62. Yvonne Nam RT. Prenatal sonographic diagnosis of congenital Epulis. J Diagn Med Sonogr. 2003; 19:255-7.
63. Meizner I, Shalev J, Mashiach R, Vardimon D, Ben-Rafael Z. Prenatal ultrasonographic diagnosis of congenital oral granular cell myoblastoma. J Ultrasound Med. 2000; 19:337-9.
64. Hulett RL, Bowerman RA, Marks T, Silverstein A. Prenatal ultrasound detection of congenital gingival granular cell tumor. J Ultrasound Med. 1991; 10:185-7.


  • There are currently no refbacks.

Copyright (c) 2015 Journal of Neonatal Surgery

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

The Journal of Neonatal Surgery has been indexed Google Scholar, NewJour, Open J-gate, Science Central, eDoctoronline, Ulrichsweb, Scirus, JournalTOCs, ResearchGate, Scribed, FreeJournalAct, Anoox, News-Digest, CORE.

EL-MED-Pub Publishers