Background/purpose The risk of malignant transformation of sacrococcygeal teratoma (SCT) and of presacral teratoma in Currarino syndrome (CS) may differ despite the similar position and appearance. Methods Malignant transformation and teratoma recurrence were assessed in a national retrospective comparative analysis of 205 SCT and 16 CS patients treated in one of the six pediatric surgical centers in the Netherlands between January 1981 and December 2010. Results The malignancy free survival of patients with SCT was lower than for patients with a presacral teratoma associated with CS (80% and 58% after one and two years in SCT versus 100% after two years in CS, p = 0.017) Conclusions In SCT, malignancy and recurrence risk are high. Therefore, early and complete resection is mandatory. Our data show that the risk of malignant transformation of a presacral teratoma in CS is small
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