Primitive neuroectodermal tumor of the kidney: case report and review of literature

Abstract

BackgroundRenal primitive neuroectodermal tumor (rPNET) as a member of Ewing’s sarcoma family is extremely rare and usually occurs in children and young adults. Most literature about rPNET was isolated case reports.Case presentationWe reported a case of 45-year-old man with the complaint of right flank pain. Computerized tomography (CT) scan demonstrated a large substantive tumor involving the lower pole of the right kidney. Then the patient underwent radical nephrectomy. Pathologic characteristics and immunohistochemical analysis confirmed the diagnosis of rPNET. Additionally, the patient received three cycles of chemotherapy, and was still alive without metastasis at 15-months follow-up.ConclusionrPNET is rare and presents aggressive clinical behavior and worse prognosis. We expect that further awareness and study of this rare tumor can be had by presenting our case.

Unilateral Renal Primitive Neuroectodermal Tumor (PNET)

“…Imaging findings, such as huge renal mass (more than 10 cm in diameter) is prevalent (6,7,11). As the tumor is highly aggressive, it is often diagnosed at an advanced stage with distant metastasis, with lung being one of the most common sites (4,12,13). However, some studies, such as Thyavihally et al (2008) showed that patients mostly had localized disease at presentation (10).…”

Renal Primitive Neuroectodermal Tumor

“…Molecular testing is useful in situations with a confusing immunohistochemical profile. The diagnosis of renal PNET always needs to include tumor morphology, immunostaining profile and sometimes genetic mutations (10).…”

A Rare Case of Primitive Neuroectodermal Tumour (Pnet) of Kidney with Review of Literature

“…There is a slight male predominance seen in the literature for PNET in general as well as renal PNET. The tumours tend to be very large and maximum diameter of renal PNET is more than 10 cm (average 12.4 cm) with size ranging from 4 to 24 cm [4,5] . This difference in size may be a differentiating feature from renal carcinoma as the tumours of renal cell carcinoma can be smaller than 3cm and can be multi-focal in the setting of Von hippel Lindau syndrome.…”

“…PET-CT demonstrated intense2-[18F]flouro-2-deoxy-glucose (FDG) uptake (standardised uptake value[SUV] maximum 11.8) similar to other renal tumours [4] . Pain, palpable mass and hematuria are the most common symptoms [5] .…”

“…In 85 to 90% cases of Ewings family tumours, a recurrent chromosomal translocation, t (11; 22) (q24; q12), fuses the 5' portion of the EWSR1 gene on chromosome 22 to the 3' portion of the FLI1 (friend leukemia integration locus-1) gene on chromosome 11wich can be detected by flouresceneinsitu hybridization. Using a large panel of immunohistochemical markers is often essential [cytokeratin, EMA, WT1 (Wilms tumour 1), synaptophysin, muscle specific actin, desmin, myogenin, leukocyte common antigen (LCA) and CD45] to exclude other round cell tumours because of their important similarities [1][2][3][4][5][6][8][9][10][11][12][13][14] . Differential diagnosis of renal PNET includes wilms tumour, neuroblastoma, clear cell carcinoma, lymphoma, rhabdomyosarcoma, small cell variant of osteosarcoma and small cell anaplastic neuroendocrine carcinoma.…”