Immature teratoma within an omphalocele: A unique finding

Introduction: Teratomas are the only true umbilical cord neoplasms. Antenatal diagnosis of an umbilical cord tumor in the presence of an exomphalos is difficult because of the rarity and pleomorphic presentation. We report a case of omphalocele containing an immature umbilical cord teratoma. Case presentation: A 3-day old girl with an antenatally diagnosed omphalocele was found to have, other than intestinal loops and a part of the liver, two solid masses at the caudal aspect near the top of the omphalocele sac. On exploration, a cystic structure with solid components was seen within the sac and excised. Microscopic examination revealed a teratoma with immature neural tissue represented by primitive neural tubes and rosettes. Discussion: Seen in 4000-5000 live births, 70-80% of omphaloceles are associated with morphologic abnormalities of other systems. Prenatal diagnosis of umbilical cord teratomas is challenging when associated with an exomphalos but should be suspected when a mixed solid and cystic mass is documented attached to the umbilical cord in the midline. Till date, there are 19 reported cases of umbilical teratomas of which only five (26.3%) contained immature tissue. Surgical excision with close follow-up is the recommended treatment. Outcome depends on the presence of associated anomalies, extent of tumor resection, occurrence of surgical complications and the presence of foci of yolk sac tumor in the neoplasm. Conclusion: Immature teratoma within an omphalocele is a rarity. However, the malformation is surgically correctable and prognosis is largely determined by the presence of associated abnormalities.