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Definition: This tumor, derived from the three germinal layers is found frequently
in the sacroccocygeal region. In
fact, this is the most frequent site of
teratomas of the fetus.
Prevalence: The incidence is 1 in 35,000 to 1 in 40,000 live births. Females are
four times more likely to be affected than males, but malignant
change is seen more frequently in males (23).
Etiology: The etiology is unknown,
but has been suggested that it is derived from
totipotential cells in Hensen’s node. A theory of “twinning accident” with
incomplete separation during embryogenesis has also been proposed. Some cases of sacrococcygeal teratoma are
familial, with autosomal dominant inheritance.
Ultrasound features: Most sacrococcygeal teratomas appear solid or mixed solid
and cystic, containing randomly arranged, irregularly shaped
cysts. Occasionally the tumor displays a solid, or more often a
completely cystic variant. Most teratomas are extremely vascular, which
is easily shown using color Doppler technique. Polyhydramnios
is frequent, and its mechanism is unclear. Secondary to high output cardiac failure, hepatomegaly, placentomegaly
and hydrops fetalis can occur. High output failure
is the result of tumor hemorrhage and/or arteriovenous shunting.
Prognosis: The mortality rate of fetal sacrococcygeal teratoma approaches 50%,
while the mortality in newborns is
5%. Most tumors are benign, but malignant change is a definite
risk. The risk rises from 10% before 2 months of age to 65 to 90
percent if the diagnosis and surgical excision are delayed beyond 4
months of age. Other factors that may influence prognosis include tumor size,
and presence of tumor hemorrhage. It
should be remembered that this tumor tends to become large,
and thus may cause obstetric complication such as dystocia and
malpresentations.
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fig. 19 fig. 20 |
| 23. | Altman,
R.P., Randolph, J.G., Lilley, J.R. Sacroccocygeal teratoma: American Academy of
Pediatrics Surgical Section Survey. J Pediatr Surg 1974;9:389-398. |
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