Abstract
•Presacral ependymoblastoma can present as inguinal lymph node metastasis.•Clinical and radiographic findings are not sufficient for diagnosis.•LIN28 staining can reliably classify this exceedingly rare tumor.
Ependymoblastoma presenting outside the brain is rare in children. The overwhelming majority of presacral ependymal tumors are of the benign myxpapillary type. We present a case of ependymoblastoma in the presacral region of a four-year old child. The patient presented with a presacral mass and ipsilateral inguinal lymph node metastasis. Both masses revealed histologic and immunophenotypic features of ependymoblastoma associated with myxoid areas and high Ki-67 proliferation index. The masses were excised and the patient remained well for a year after diagnosis with no other therapy. This report emphasizes ependymoblastoma as rare entity in the differential diagnosis of presacral tumors in children and, up to our knowledge, the first report of inguinal lymph node metastasis of ependymoblastoma.