Primitive neuroectodermal tumour of kidney with thrombosis of the inferior vena cava and good responsive to surgical and medical treatment: description of a case and revision of literature.

Abstract

Primitive neuroectodermal tumour (PNET) of kidney is a rare cancer typical of young adults with few cases described in the literature. We report a case of renal PNET in a 31-year-old man who presented to our department with a computed tomographic (CT)-scan revealing a large renal mass of 20 cm, massive thrombosis of the inferior vena cava (IVC). The patient underwent radical nephrectomy with contextual retroperitoneal lymphadenectomy and resection of IVC needing Dacron prosthesis substitution. Definitive histopathological examination showed PNET of kidney infiltrating ipsilateral adrenal gland, massive cava thrombosis with infiltration of vena cava wall and one lymph nodal metastasis. Postoperative PET-scan showed metastatic lesions in bilateral adrenal glands and pancreas. The patient received chemotherapy, and currently, he is in follow-up after 26 months from first diagnosis without any sign of recurrence of disease. Kidney PNET usually is associated with poor prognosis, so, it needs an early identification and differentiation from other similar small cells tumours in order to obtain a good response to the treatments.