BOB Ped Surg 2023 - Lieke Josephine Van Heurn, EUPSA - Presentation

Sacrococcygeal teratoma is the most common germ cell tumor in neonates with an estimated incidence of one per 14,000 to 35,000 live births.

The likelihood of malignant transformation increases with age, with malignancy rates up to 70% if SCCT is diagnosed at the age of one year.

More than 150 centers from 65 countries participated in the SCCT study.

Data was collected for patients treated for SCCT between 1980 and 2020.

Data of patients where SCCT was associated with Currarino syndrome was not analyzed because it is unknown whether SCCT associated with Currarino has the same risk of malignant transformation as SCCT.

All participating centers included 3,614 patients with SCCT, with 205 patients with Currarino syndrome excluded, leaving 3,409 SCCT patients included in analysis.

During the first six years of life, the probability of malignant transformation increases strongly, and after six years of age, the probability remains stable at 60%.

After six years of age, almost no malignancies have been found.

Malignancy-free survival stabilized at the age of five years with a malignancy-free survival rate of 81%.

Late malignancies up to 15 years of age have been found.

Most recurrences were found four years after initial resection, with probability of recurrence-free survival at 85% four years after initial resection.

Incidental late recurrences up to 20 years after primary resection have been found in this study.

Altman type 2 and 3 were significant risk factors for recurrent SCCT in multivariate analysis.

Malignant histology of the primary tumor increases the risk of recurrent SCCT with an odds ratio of 4.7.

Income country was not a risk factor for recurrent SCCT.