Giant congenital cervical Teratoma: A case report

Abstract

Introduction

Congenital teratomas affecting the head and neck region are rare, accounting for only 2–5% of all germ cell tumors. Congenital cervical teratomas occur in 1 per 20,000 to 40,000 live births. Due to their location, giant neck teratomas obstruct the airway and esophagus, leading to polyhydramnios and requiring an ex-utero intrapartum therapy (EXIT) procedure to allow survival.

Case presentation

A large fetal neck mass resulting in fetal neck extension and polyhydramnios was noted at 24 weeks' gestation by ultrasound and further characterized by fetal MRI. The infant was delivered by classical c-section for non-reassuring fetal heart rate status at 33 weeks’ gestational age. The mass was overlying both mandibles, extending down the neck, leading to extensive neck extension and obscuring anatomic landmarks. Resuscitation required puncturing vocal cord adhesions with a rigid bronchoscope and suctioning of airway fluid prior to intubation. The extensively vascularized mass was successfully resected at one week of age, when it weighed 1088 g and was 21cm × 12cm x 8 cm in dimension. Pathology was consistent with a grade 3, stage II mixed immature and mature teratoma including tissue derivatives of three germ layers.

Conclusion

Despite the unusually large size of this giant congenital neck teratoma, management by a multidisciplinary team with prenatal planning of resuscitation and postnatal treatment allowed for a positive outcome for the infant.