Teratoma of the neonatal head and neck: A 41-year experience.

OBJECTIVE: To review our institution's experience with the presentation, evaluation, and management of teratoma of the head and neck in the neonatal population.

DESIGN: Retrospective case series (November 1970 through September 2011).

SETTING: Tertiary care children's hospital.

PATIENTS: 14 patients (12 boys and 2 girls).

INTERVENTION: Detailed review of presentation, diagnostic approaches, surgical management, and outcomes.

MAIN OUTCOME MEASURES: Anatomic sites, use of pre and post-natal imaging, use of EXIT (ex utero intrapartum treatment) procedure, presenting symptoms, surgical approaches, additional therapeutic modalities, and outcomes are reviewed.

RESULTS: Seven patients were diagnosed prenatally, while the remaining 7 patients were diagnosed at birth or shortly thereafter. The tumor emanated from the neck in 9 patients, the nasopharynx/oropharynx in 3 patients, the external nose in 1 patient and the face in 1 patient. Nine patients had associated upper airway obstruction. Four underwent an EXIT procedure, with 3 requiring intubation and 1 requiring tracheostomy. All patients underwent surgical resection. One patient demonstrated recurrence at follow-up.

CONCLUSIONS: Teratoma of the head and neck, though rare, is an important part of the differential diagnosis of neck masses in children, particularly in the perinatal period. The ability to make this diagnosis prenatally with high-resolution fetal ultrasound and MRI (magnetic resonance imaging) permits planning for airway and tumor management prior to delivery. An EXIT procedure should be considered when airway compromise by tumor compression is suspected. Early surgical excision is the treatment of choice and recurrence is rare when a complete resection is achieved.