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Sacrococcygeal Teratoma

 

 

Related narrative: Sacrococcygeal Teratoma

Sacrococcygeal teratoma is the most common tumor in the newborn with a heavy preponderance in females. It is increasingly diagnosed in utero when ultrasound is done for inappropriate uterine size due to tumor mass or polyhydramnios. Children delivered with this lesion prior to 30 weeks have a high mortality from pulmonary immaturity. Delivery after 30 weeks is usually by planned Cesarean section to avoid dystocia, tumor rupture and hemorrhage. Placentamegaly and hydrops carry a poor prognosis. Associated serious congenital anomalies are rare.

Half these lesions contain only mature tissues, 20% contain immature tissue and 30% have malignant elements. Malignant behavior increases rapidly with age, reaching about 50% at 2 months. The higher the teratoma extends into the pelvis, the greater the chance of malignancy. A low type I (Altman classification) tumor such as this one has about an 8% chance of being malignant, while a high lesion may have a rate as high as 50% or more. Higher, intrapelvic lesions may be occult until after 2 months, allowing malignant elements to grow and spread. In the past 25 years, combination chemotherapy (Einhorn) has dramatically increased survival in malignant lesions.

Early complete excision, including the coccyx (Gross) has a high cure rate. CEA is a good marker for recurrence. While naturally high in newborns, the level should decrease after complete excision. A subsequent rising level may indicate recurrence. Work is now being done on in utero surgery for sacrococcygeal teratoma.

Because the coccygeal margin was positive in this case, a postoperative MRI scan was done. There was no radiological evidence of residual pelvic heterotopic tissue. No further surgery was done and the patient will be observed closely.

References:

Altman RP et al. Sacrococcygeal teratoma, J Ped Sur, 9:389-398, 1974.

Donnellen WA & Swenson O. Benign and malignant sacrococcygeal teratoma, Sur 64:834-846, 1968.

Flake AW et al. Fetal sacrococcygeal teratoma, J Ped Sur 21:563-566, 1986.

Gross RE et al. Sacrococcygeal teratomas in infants and children, SG&O 92:341-354, 1951.

Grossfeld JL et al. Benign and malignant teratomas in children: analysis of 85 patients, Sur 80:297-305, 1976.

Hickey RC & Layton JM. Sacrococcygeal teratoma, Cancer 7:1031-1043, 1954.

Schropp KP et al. Sacrococcygeal teratoma: the experience of four decades, J Ped Sur 27:1075-1079, 1992.


This page was last modified on 14-Sep-2000.