Malignant peripheral neuroectodermal tumor (MPNET) of the kidney

Abstract

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 appearance and the typical immunohistochemical findings, are described and discussed.

Peripheral Primitive Neuro-Ectodermal Tumor - A Case Report

“…Peripheral primitive neuroectodermal tumours (pPNETs) are uncommon tumours constituting 1% of all soft tissue sarcomas [1]. They constitute a group of embryonic malignant tumours collectively designated as small round cell tumours.…”

“…This tumour can occur at any age, although the peak age incidence is during adolescence and young adulthood. [1,2] In general, PNETs often exhibits aggressive behaviour with a poor prognosis, 5-year disease-free survival rate being 45%-55%. [2] The most common locations of peripheral PNETs are the thoracopulmonary region, followed by the retroperitoneal, paravertebral soft tissues, and the head and neck region.…”

“…The onset of the tumour is insidious and patients often have no specific clinical symptoms. [1,2] The diagnosis of a pPNET requires the combination of clinical symptoms, pathological characteristics, immunohistochemical features and cytogenetic analysis. This tumour can occur at any age, although the peak age incidence is during adolescence and young adulthood.…”

Renal primitive neuroectodermal tumour in childhood: Case report and review of literature

“…Other helpful markers are neuron-specific enolase and vimentin, synaptophysin and S100 protein. [20][21][22][23][24][25] 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 tumours; it is also characteristic of PNET and Ewing's sarcoma. Fluorescence in-situ hybridization (FISH) allows confirmation of the PNET/Ewing sarcoma specific t (11:22) and t (21:22).…”

“…Radiographic features of renal PNET are nonspecific and are usually presented as a huge renal mass with or without calcification. 20 Histologically, PNET consists of sheets and nests of primitive small round blue cells and presence of Homer-Wright rosettes (Fig. 3, Fig.…”