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Abstract
Background/Purpose: Sacrococcygeal teratomas (SCT) are a relatively uncommon tumor affecting neonates,
infants, and children. This study was designed to determine the effect of therapy
on the long-term outcome of neonates and children with sacrococcygeal teratomas (SCT).
Methods: The authors conducted a retrospective review of children with SCT treated at 15
Childrens Cancer Group institutions from 1972 to 1994.
Results: One hundred twenty-six children presented with SCT diagnosed prenatally (n = 32),
at birth (n = 79), or later in infancy (n = 15). For neonates, complete resection
was performed except in two babies with lethal associated defects. All others (n =
15) underwent resection at the age of diagnosis. Six had a sacral mass identified
at birth but had delayed surgery (1.5 to 11 months) and of these, two were malignant.
Resection was via sacral (n = 96) or abdominosacral (n = 28) approach. Histology showed
mature teratoma (MT, 69%), immature teratoma (IT, 20%), or endodermal sinus tumor
(EST, 11%) at presentation. Seven neonates (5.6%) died of perioperative complications,
whereas the remaining 117 were available for long-term follow-up. Between 6 and 34
months postresection, recurrent disease developed in 9 of 80 MT patients (11%) followed-up
for a mean of 5 years. Recurrent disease was MT (n = 2) and EST (n = 7). The recurrent
EST patients were treated with adjuvant chemotherapy. Six are alive with mean follow-up
of 114 months, whereas one with metastatic disease was lost to follow-up. Recurrence
(MT) developed in only 1 of 24 IT patients, and all are alive and well at mean follow-up
of 39 months. Patients presenting with EST (n = 13) underwent excision, with two dying
from non-EST causes. Six EST patients received no chemotherapy, with two of the six
(33%) experiencing recurrence within 11 months and both disease free after salvage
chemotherapy. The remaining five EST patients received adjuvant chemotherapy; four
are alive and one died of metastatic disease. Of the 18 EST patients followed-up after
resection (presentation, 11, recurrent teratoma, 7), 16 (89%) are free of disease
with a mean follow-up of 91 months.
Conclusions: (1) Benign teratomas have a significant recurrence rate mandating close follow-up
for more than 3 years. (2) Surgical resection alone is adequate therapy for nonmetastatic
malignant tumors. (3) Survival for malignant lesions with metastases is excellent
with modern chemotherapy.
Keywords
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Article info
Footnotes
☆Presented at the 28th Annual Meeting of the American Pediatric Surgical Association, Naples, Florida, May 18–21, 1997.
Identification
Copyright
© 1998 Published by Elsevier Inc.
