Congenital Epulis in a Newborn

1. History: Infant was born at 39 weeks gestation by spontaneous vaginal delivery without complications. The infant was vigorous and pink at delivery with appropriate APGAR scores. The mother's pregnancy history, prenatal labs, and medical history were unremarkable. She had normal fetal ultrasound at 20 weeks gestation. Neonatology was called shortly after birth to assess an oral mass.
2. Physical Exam Findings: Infant presented as a healthy newborn baby with a solid beefy red mass deriving on a vascular stalk from the mandibular gum line, approximately 4 cm in diameter. He had a good suck reflex. A small natal tooth was palpable at the gum base, but oral cavity was otherwise unremarkable. He was not in respiratory distress.
3. Lab/Diagnostic Imaging/Procedures: Facial ultrasound showed arterial and venous flow within the oral mass, not characteristic for either hemangioma or AVM. The mass was excised with repair of mandibular alveolar ridge, and the natal tooth was removed. Histologic evaluation of the mass confirmed congenital granular cell epulis.
4. Final Diagnosis: congenital granular cell epulis
5. Discussion: 

    Congenital epulis (also known as congenital gingival granular cell tumor) is a rare gingival tumor that only grows in-utero. Although the mechanism remains unclear, it is hypothesized that growth may be stimulated by intrauterine estrogen. It is a rare condition with a strong female predominance (female to male ratio ranging from 3:2 to 10:1). 

    The epulis appears as a pink, smooth-surfaced, soft tissue mass that is attached to the gingiva by a vascular stalk. It is more common for the mass to arise from the maxilla than mandible, and even more uncommonly, can arise from both. It is typically discovered as the mass protrudes out of the newborn child's mouth, with mass size ranging from 1 mm to 9 cm in diameter. Due to the mass, the neonate may have difficulties with feeding, and if large enough, may have respiratory compromise due to airway obstruction.

    Diagnosis may be made clinically but is often confirmed histologically, especially to differentiate it from its differential diagnosis of epignathus, which is a malignant oropharyngeal tumor. Histologically, it is composed of eosinophilic spindle-shaped cells thought to be of mesenchymal origin. Diagnosis can be made by prenatal ultrasound, which may allow time to plan resuscitation efforts, such as the EXIT procedure. Although smaller masses may regress over time without intervention, most masses require surgical resection. No recurrence or malignant transformation has been documented in literature.

In this case, a healthy male newborn was born with an oral mass arising from the mandibular gingiva, confirmed to be congenital epulis by histology. The mass was not detected at the 20 week ultrasound, suggesting its development during the late second or third trimester.  Although already rare in occurrence, the presentation in this patient is special in that it occured in a male newborn with the mass arising from the mandible, rather than the maxilla. The infant was ultimately transferred to a tertiary children's hospital for surgical evaluation. While the mass was impressive in size, the infant did not have feeding nor respiratory difficulties and was able to feed by bottle around the epulis while awaiting surgery. Although there was no initial bleeding of the mass at the primary hospital, there was bleeding at the vascular stalk at the children's hospital, ultimately leading to its removal. Given current awareness of congenital epulis, it is anticipated that he will not experience recurrence, long-term sequelae nor changes in dentition.

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