Epignathi Teratoma Co-Existing with Tongue Choristoma and Cleft Palate in a Neonate: Report of a Rare Case and Review of Literature

Abstract

Background: Oral epignathus teratoma is an extremely rare neoplasm that occurs exclusively in neonates. The tumor can grow to a large size obstructing airways and it may prevent fusion of the nasal septum with the palatine processes, which may result in cleft palate formation. The rare tumor may also co-exist with other congenital malformations such as hamartoma and choristoma causing airway obstruction and death. Case report: We present a case of a one-day-old male neonate who presented with oral mass associated with difficulty in breathing and inability to suck breast milk. Computed Tomography (CT) scan revealed multiple masses involving the alveolar, palate, and tongue along with cleft palate. Emergency surgery was done to relieve the airway and histology confirmed epignathi teratoma with tongue choristoma. Conclusion: Epignathus teratoma is a rare condition that requires prenatal prompt diagnosis. However, in countries with underprivileged hospital facilities, early surgical intervention during the neonatal period is what is required to save the patient from airway obstruction. Association of the tumor with midline anomalies is mostly the norms, however, 5% of cases with malignant transformation have been reported and the tumor is commonly associated with endodermal sinus tumor. Therefore long term follow-up of patients with serum tumor markers is highly recommended.