A Female Infant Who Has a Pink, Smooth Soft-Tissue Mass

Abstract

A female newborn has a pink, fresh, smooth, soft tissue mass (2.0 cm x 1.5 cm x 1.0 cm) on the midline of the hard palate (Fig 1).Serum alpha feto protein was 61,575 ng/mL.Congenital epulisEpulis is a benign tumor arising from the gingival mucosa. It is also known as congenital gingival granular cell tumor or Neumann’s tumor after its first description in 1871.This gingival granular cell tumor grows only in utero, especially during the third trimester of gestation. No known cause of epulis has been identified though it has been theorized that intrauterine estrogen stimulates its development because of its female predominance and spontaneous regression once maternal estrogen is withdrawn.Epulis is rare. Only 186 published cases were found in a literature review by Yavuzer in 2001. It is more common in females, with female-to-male ratio from 3:2 to 10:1. The tumor occurs more commonly from the maxilla than the mandible, with 2:1 ratio. It involves both maxilla and mandible in 10% of cases. Our case is unusual in that it appeared on the palate.The lesion is typically a pink, smooth-surfaced, soft tissue mass attached to the gingiva by a broad base or by a pedicle. Its size is from 1 mm to 9 cm in diameter. The infant with epulis may have feeding problems and respiratory difficulties due to airway obstruction. Polyhydramnios secondary to a blockage of the fetal oral cavity and inability to swallow amniotic fluid can occur.The diagnosis of epulis can be made based on clinical findings and it is confirmed histologically. On histological examination, epulis has sheets of round cells with granular cytoplasm and small nonmitotic nuclei, which are surrounded by a delicate stroma of collagen and covered by the squamous epithelium with no pseudoepitheliomatous hyperplasia. It tends to have a prominent plexiform network of capillaries. Epulis may be diagnosed by prenatal ultrasonography.Simple surgical resection of the tumor is required. Our case reveals that some cases can spontaneously regress over time without resection. Radical excision is discouraged because of the damage to underlying structures. There are case reports that reveal the conservative approach to the small asymptomatic lesion can succeed in spontaneous regression. No recurrence or malignant transformation has been reported.Epignathus, a congenital oropharyngeal teratoma is one of the differential diagnoses. It originates from the craniopharyngeal canal and attaches to the base of the skull in the posterior region of the nasopharynx. Epignathus is a malignant tumor and is difficult to distinguish from an epulis on clinical grounds alone. Few cases with an intracranial extension along the craniopharyngeal canal can be fatal. The infant with epignathus may also have other malformations, such as cleft palate, bifid tongue, bifid nose, pituitary duplication, and hypoplasia of the corpus callosum.Because there were no feeding or respiratory problems, management was conservative, and the infant was discharged. The epulis underwent spontaneous regression by the time the infant was 2 months old.JoDee M. Anderson, MD, MEd, Division of Neonatal Medicine, Oregon Health & Science University, Portland, ORSpecial acknowledgements are given to Dr Art Cooper, Department of Pediatric Surgery; Dr Jason Moche, Department of ENT; and Dr Cesar Ong, Department of Radiology, Harlem Hospital Center, New York, NY.