Sacrococcygeal teratomas (see also Chapter 22 by Malogolowkin and colleagues) are the most common congenital tumors, occurring in 1 of 40,000 neonates.49 These tumors occur four times more often in females than in males. The tumors show an age-dependent malignancy that increases with the age of the patients. Thus, 7% of tumors diagnosed at birth, 37% of tumors diagnosed by the age of 1 year, and 50% of tumors diagnosed at the age of 2 years are malignant.50
Congenital tumors present as masses protruding from the rump of the neonate (Figure 4-9). These tumors are classified into four groups according to the presacral and/or abdominal extension of neo-
plastic masses.49 Type I tumors have only a minimal presacral component; type II tumors are predominantly external but have a definitive intra-abdominal component; type III tumors are mostly in the abdomen, with a very small external component; and type IV tumors are entirely intra-abdominal and presacral, and there is no external tumor.
Overall, benign tumors predominate.51 Approximately 65% are classified as mature solid teratomas, 25% as immature teratomas, and 10% as malignant mixed GCTs. The latter tumors contain EC and yolk sac tumor components. Tumors treated in specialized surgical centers have an excellent prognosis.52
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