Primitive neuroectodermal kidney tumour

Abstract

Renal primitive neuroectodermal tumour (PNET) is a rare malignant tumour of paediatric patients. We describe the important imaging findings, including the ultrasound, CT and MRI of a case of renal PNET in a teenager. To our knowledge, some findings have not been described previously. Some features that have been considered as characteristic are not present in our case, and it is, to our knowledge, the first documented case with such extensive tumour thrombus. We also demonstrate the importance of including PNET as a differential diagnosis because combined chemotherapy is thought to be the best first-line treatment in most cases. Histological diagnosis can be determined by a minimally invasive procedure instead of laparotomy or open biopsy.

Renal Peripheral Neuroectodermal Tumor Presenting at Age 78: Case Report

“…Bright heterogenous signal intensity is noted on T2-weighted MRI [8]. It may appear hyperechoic or isoechoic on ultrasound [9].…”

Metastatic Primitive Neuroectodermal Tumor to the Kidney

“…In these cases, the probability of a second primary renal malignancy is 4.5 times higher than that of a renal metastasis [13] . The most likely differential diagnoses, especially in the presence of renal vein thrombosis, are renal cell carcinoma and Wilms' tumor [7] , depending on the age group. Primary PNET of the kidney have also been associated with renal vein invasion [4,5,7,10] .…”

“…A primary PNET of the kidney is a highly aggressive malignant neoplasm, often presenting locally advanced disease or metastasis at initial diagnosis [3,7,9] .…”

“…PNET were fi rst described in 1918 by Arthur P. Stout [8] and, in the early 1980s, were associated into a group of tumors called Ewing's sarcoma family because of their common features [2][3][4][5][6][7] . PNET are rare small round cell malignancies of presumed neural crest origin that arise from outside the central and sympathetic nervous system [2,3] .…”

“…They belong to the Ewing sarcoma family of tumors which usually affect young individuals and bear a highly aggressive course [2][3][4][5][6][7] .…”