Primary primitive neuroectodermal tumor of kidney: A rare case report with diagnostic challenge

Abstract

Primary primitive neuroectodermal tumors (PNETs) of the kidney are quite rare and can be mistaken for a wide variety of other small round blue cell tumors which includes rhabdomyosarcoma, Wilm's tumor, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma etc. Renal Ewings/PNET can occur in the age group from 4 to 61 years. Approximately, 90% of Ewing sarcoma (ES)/PNET have a specific t(11;22) which results in a chimeric EWS-FLI-1 fusion protein. Immunohistochemical for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. Herein, we have an interesting presentation in a 23-year-old male who came with neck pain and progressive quadriparesis and was diagnosed as a case of poorly differentiated malignant tumor with a differential of lymphoma versus metastatic renal cell carcinoma. The patient's condition deteriorated fast and he had a rapid downhill course. The final diagnosis of Ewings/PNET was confirmed at autopsy.

Primitive neuroectodermal tumor (PNET)/Ewing sarcoma of kidney: A rare case report of three cases

“…5 Rafael et al in study of 11 cases observed that most common age of diagnosis was between 18 to 45 years. 4 Other studies also showed mean age of 27 for the diagnosis of these cases. 1,2,4 In our study, the age of two patients was 25 years and the third one was 35 years.…”

“…2 They are commonly confused with other small round cell tumors.Also clinical findings are very similar to other renal malignancies, hence histopathology and immunohistochemistry remains the gold standard for confirming the diagnosis. 3,4 We report three cases of PNET / Ewing sarcoma .Two patients were males 25 and 35 years and third patient was25 year old female. Histopathology showed malignant small round cells in sheets and diagnosis was confirmed on immunohistochemistry.…”

The CT and US features of Ewing’s sarcoma/primary neuroectodermal tumor of the kidney: two case reports and review of literature

“…EWS/PNET of the kidney is an exceedingly rare entity, and only ~126 cases have been reported in literature. 4 – 13 Due to the poor prognosis of primary renal EWS/PNET, accurate and timely diagnosis is demanded in patient diagnosis.…”

“…However, EWS/PNET of the kidney is an extraordinally rare tumor that can be found in both children and adults. 1 , 2 It was first described in 1974 by Seemayer et al 3 To date, only ~126 cases have been reported 4 – 13 with very few detailed radiological descriptions. EWS/PNET of the kidney is not well recognized by either clinicians or radiologists, and the clinical and imaging features of EWS/PNET have not been well illustrated.…”

Successful Radical Pneumonectomy for a Primitive Neuroendodermal Tumor in the Lung: A Case Report and Review of the Literature

“…PNETs arise more frequently in the bone and soft tissue and are very rare in other parts of the body. However, there have been reports of PNETs in the kidney, liver, urinary bladder, myocardium, adrenal glands, pancreas, and female genital tract (3)(4)(5)(6)(7)(8)(9). In 1979, Askin et al (10) first reported a PNET that occurred in the chest, which is currently referred to as Askin's tumor.…”