Epulis

Congenital epulis (i.e. “NeumannTumour”), also called congenital granular cell tumour or congenital granular cell epulis, is a soft lesion that protrudes from the oral cavity. This lesion is formed mostly in the maxillary alveolar ridge, but can also arise from the mandibular alveolar ridge. (1,2)

ICD code: K06.8

Epulis is considered rare; approximately 250 cases have been reported around the world. The incidence of epulis is 0.0006%. (2) Multiple lesions are found only in 10% of the cases and it is found two to three times more commonly in the maxillary than the mandibular  alveolar ridge. (1-7) It is found more frequently in female fetuses with a female-male ratio of 10:1. (1, 5)

 A role for maternal hormones has been implicated in the development and growth of epulis. This theory is controversial however, because the presence of estrogen and progesterone receptors in the lesion has never been demonstrated. Epulis grows most rapidly during the third trimester. This lesion exhibits a disorganized and undifferentiated growth of cells of mesenchymal origin. It is composed of large polygonal cells with eccentric nuclei, abundant eosinophilic granular cytoplasm, and slightly visible nucleoli. (1) The cells comprising the epulis are surrounded by abundant vascular vessels and a stromal space that occasionally exhibits chronic inflammatory cells (such as lymphocytes). (1)

The etiology of epulis is not clear. In addition to the theory purporting an influence of maternal hormones on epulis development, tsome authors have suggested a reactive or degenerative nature of the lesion. (1) The growth of undifferentiated cells is considered the most accepted etiology. (1,2,4,7)

Epulis is a benign soft lesion that is formed in the maxillary or mandibular ridge. Despite its benign nature, it typically displays  growth mainly during the third trimester of pregnancy. (1) Usually, small in size, up to 1-2 cm but it has been reported in some cases  to attain growth up to 9 cm resulting in obstruction of the fetal airway. (1) Involvement of the tongue larynx, and trachea is  rare. (2, 4, 6,7)

Epulis is not usually associated with other anomalies or deformities of dentition. (2,5,8,10) Usually, epulis is found in patients with normal karyotype, but an association with 47 XXX and polydactyly has been reported. (4)

Usually, epulis does not recur after the surgical intervention even with incomplete removal. (1,8,9)
 

Diagnosis can be made by prenatal ultrasound usually in the second and third trimester. (1- 5,7) Also, nuclear magnetic resonance imaging can be useful to determine extension of the lesion into surrounding tissues. (1,5) Epulis can be seen as a soft “polypoid” and homogeneous lesion with a base that can be wide or thin, covered by mucosa and with organized blood flow. (1-5,8,9) Doppler ultrasound can help toexclude differential diagnoses. (2) Three-dimensional ultrasound can be more useful than two-dimensional ultrasound to evaluate the extension of the mass. (2,5) The definitive diagnosis is made by histopathological examination. (2,9,10,11)

The list of differential diagnoses includes, rhabdomyoma, infantile myofibroma, hemangioma (a mass with disorganized pattern), teratomas, macroglossia, lymphatic malformation, cephaloceles, epignathus and dermoid cysts. (1) flow is also part of the differential diagnoses. (1) Others include (2,5,6,9)

The prenatal diagnosis is crucial to stablish the delivery plan. It is important to establish if there is obstruction of the airway, to determine the need for an EXIT. (1,8)
 

Although epulis is a benign lesion, the covering mucosa can be erythematous or ulcerated. In addition, the prognosis depends on the size of the mass. If the lesion is large it can create problems with mouth closure, feeding, and/or breathing. Sometimes the lesion regresses spontaneously after birth. (1,9) Usually, epulis does not recur after surgical excision. (1)

Once the prenatal diagnosis is made, a multidisciplinary management is crucial (neonatologists, maternal fetal specialists, maxillofacial surgeons, otorhinolaryngologists). The type of delivery depends on the extent of the mass and the potential  involvement of the airway. (2) The treatment of the lesion is surgical resection. If the lesion is small, epulis can be managed conservatively. (1,9) When the lesion is substantial in size and it is obstructing the airway, an ex-utero intrapartum treatment procedure (EXIT) may be required. (1,3,4,10)
The resection of the mass can be made under general or local anesthesia. (1,3,4) Patients with major respiratory and digestive tract complications must be operated as soon as possible after birth. (2) 
Some authors have reported good outcomes with the use of electrocauterization or pulsed CO2 laser. (7)

1.    Torresani, E. et al. (2021) “Congenital granular cell epulis of newborn: Importance of prenatal diagnosis,” Pathologica, 113(4), pp. 280–284. Available at: https://doi.org/10.32074/1591-951x-135.
2.    Ye, Y. et al. (2021) “Prenatal diagnosis and multidisciplinary management: A case report of congenital granular cell epulis and literature review,” Journal of International Medical Research, 49(10), p. 030006052110537. Available at: https://doi.org/10.1177/03000605211053769.
3.    Mínguez Gómez, A. et al. (2020) “Épulis Congénito Gigante: Importancia del Diagnóstico prenatal,” Anales de Pediatría, 93(2), pp. 141–143. Available at: https://doi.org/10.1016/j.anpedi.2019.06.008.
4.    Johnson, K.M. et al. (2016) “Prenatal diagnosis of congenital Epulis: Implications for delivery,” Journal of Ultrasound in Medicine, 36(2), pp. 449–451. Available at: https://doi.org/10.7863/ultra.16.03055.
5.    Frisova, V. et al. (2013) “Prenatal diagnosis of congenital Epulis by 2D/3D ultrasound and Magnetic Resonance,” Fetal Diagnosis and Therapy, 33(4), pp. 272–276. Available at: https://doi.org/10.1159/000346811.
6.    Szlachetka, K., Lemcke-Berno, E. and Ozcan, T. (2012) “Prenatal Diagnosis of a Rare Gingival Granular Cell Tumor of the Fetal Mouth,” J Ultrasound Med, 31, pp. 123–129.
7.    Ben Hamouda, H. et al. (2010) “Obstructive congenital epulis,” European Annals of Otorhinolaryngology, Head and Neck Diseases, 127(2), pp. 86–89. Available at: https://doi.org/10.1016/j.anorl.2010.03.002.
8.    Jiang, L., Hu, B. and Guo, Q. (2011) “Prenatal Sonographic Diagnosis of Congenital Epulis,” Journal of Clinical Ultrasound, 39(4), pp. 217–220.
9.    Kim, Y.-D. et al. (2008) “Congenital epulis: Prenatal ultrasonographic and postnatal MR features with pathologic correlation,” Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology, 106(5), pp. 743–748. Available at: https://doi.org/10.1016/j.tripleo.2007.12.018.
10.    Kim, S.-K. et al. (2006) “Prenatal diagnosis of congenital epulis by three-dimensional ultrasound and magnetic resonance imaging,” Prenatal Diagnosis, 26(2), pp. 171–174. Available at: https://doi.org/10.1002/pd.1368.
11.    Nakata, M. et al. (2002) “Prenatal diagnosis of congenital epulis: a case report,” Ultrasound Obstet Gynecol, 20, pp. 627–629.

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