Background: Malignant peripheral neuroectodermal tumors (MPNETs) are primit
ive neuroblastic tumors that arise, unlike neuroblastomas, outside the auto
nomic nervous system. A renal origin has been described in very few cases.
Case report: We report the case of a young male patient with a large MPNET
of the right kidney, studied with ultrasound and computed tomography before
surgical resection. The main radiologic features, the microscopic appearan
ce and the typical immunohistochemical findings, are described and discusse
d.