Renal primitive neuroectodermal tumor: An immunohistochemical and cytogenetic analysis

Abstract

The cytogenetic and morphologic characteristics of a case with a primitive neuroectodermal tumor (PNET) arising from the left kidney in a 22 year old man are presented. The patient was detected as having a left renal mass with a tumor embolus in the inferior vena cava and multiple pulmonary metastases. A radical nephrectomy with tumor embolectomy of the inferior vena cava, along with a resection of the pulmonary nodules were performed. Histologic examination revealed a dense proliferation of small round cells with many Homer-Wright type rosettes and perivascular pseudorosettes. Immunohistochemically, the tumor cells stained strongly positive for HBA71(p30/32MIC2), a surface glycoprotein specific to PNET and Ewing's sarcoma. In addition, the tumor cells expressed several neural markers (neuron specific enolase, neurofilament, synaptophysin, and Leu-7) and vimentin, while the epithelial, muscular, and lymphocytic markers were negative in the tumor cells. Cytogenetic analysis of cultured tumor cells showed a reciprocal translocation t(11;22)(q24;q12) that is considered to be specific to PNET and Ewing's sarcoma. In conclusion, this case suggested that a karyotyping analysis is a useful diagnostic tool for renal PNET, and it may therefore be utilized to help distinguish between difficult cases of small round cell tumors and Wilms' tumor of the kidney.

Primitive Neuroectodermal Tumor of the Kidney: A Single-Institute Series of Six Patients from China

“…Renal PNET is positive for CD99 in more than 90-95% of the cases [5]. Other helpful markers are neuron-specific enolase and vimentin, synaptophysin and S100 protein [4,15]. Furthermore, cytogenetic studies showed that renal PNET is consistently associated with translocation of the long arms of chromosome 11 and 22, t(11:22) (q24:q12) in more than 90% of the tumors, it is also characteristic of PNET and Ewing's sarcoma.…”

Malignant peripheral neuroectodermal tumor (MPNET) of the kidney

“…3B), which recognizes a cell membrane protein that is the product of MIC2 (p30-32 MIC2 ) [9,10]. To a minor extent but always positive were the reactions for the antibodies directed to neuron-specific enolase, Leu-7, and vimentin.…”

Cerebral Primitive Neuroectodermal Tumor in an Adult Male

“…Neuronal differentiation was observed in six of eight cases, and nine died. None of these reports documented a positive PAS reaction and CD99 (MIC 2), the characteristics of peripheral PNET, 10,11 or the cytologic findings. In 1971, Rubinstein outlined the concept of PNETs.…”

“…Immunohisto- chemically, the tumor cells weakly expressed CD99 (MIC-2) ( Figure 5). 10,11 In addition, the cells stained for several neural markers, with an especially strong positive reaction for NSE and synaptophysin and a weak reaction for GFAP. Neurofilament and S-100 protein were negative, but other neural markers, including chromogranin A, were positive in the tumor cells ( Figure 5).…”