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Neck Teratoma
Introduction
Fetal tumors are rare; teratomas are the most common histologic type. The neck is the most common location after the sacrococcygeal area for teratomas. The tissues found in fetal and infant teratomas are essentially the same regardless of the site of origin. A neck teratoma may be associated with neonatal mortality in 80% to 100% of cases if delivery is not managed properly. For large masses, adequate prenatal diagnosis allowing planned delivery with ex utero intrapartum treatment (EXIT) and intensive neonatal care are essential to improve the management.
Disease
Definition
A normally benign tumor in the neck composed of multiple tissues derived from all three germ layers of the embryonic disk—ectoderm, mesoderm, and endoderm—with a high potential of growing in excess.
Prevalence and Epidemiology
The prevalence of fetal tumors is difficult to establish; however, it is estimated to be from 1.7 : 100,000 to 13.5 : 100,000 live births. Cervical teratomas are found in about 1 : 20,000 to 1 : 40,000 live births, accounting for 3% to 6% of all neonatal teratomas.
The presentation is sporadic, without an apparent relationship to race, maternal age, parity, or fetal sex. Some cases of familial recurrence have been reported.
Etiology and Pathophysiology
The development of fetal tumors does not match the same processes as tumors observed in adults. In fetuses, tumors may result from failure of developing tissues to undergo normal cytodifferentiation and maturation.
Cervical teratomas may originate from the palate, nasopharynx, or thyrocervical area. They are usually closely related to, but do not arise from, the thyroid gland. Mature or immature neuroglial tissues are the most frequent component, but cartilage, respiratory epithelium, and ependyma-lined cysts are common. Malignancy is extremely rare. Immature elements present do not express the biologic behavior.
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