Primitive neuroectodermal tumor of the kidney: a rare case

Abstract

Background Primitive neuroectodermal tumor commonly occurs in bones and is equivalent to Ewing’s sarcoma. Very few cases have been reported in the literature and they had a very different presentation and very aggressive behavior. Case presentation We present a case of a young 23-year-old male who presented with complaints of pain in left lumbar region of abdomen since 8 months and hematuria off and on. CT scan of the abdomen was done which revealed a large heterogeneously enhancing mass lesion in the left lumbar region arising from the superior and mid-pole of left kidney showing multiple non-enhancing necrotic areas. A diagnosis of left renal cell carcinoma was kept. Through the transperitoneal approach, the left kidney was approached and a left radical nephrectomy was done. The histopathology report revealed the tumor cells to be positive for CD99 and focally positive for Vimentin and negative for cytokeratin thereby making a diagnosis of primary neuroectodermal tumor (PNET). Conclusions Renal PNET is a rare renal malignancy that should be kept in the differential diagnosis of a renal SOL especially when it is a presenting feature in adolescent and young adult. It has a very aggressive course and multimodal therapy has to be considered in its treatment

Renal Ewing sarcoma with extensive neuroectodermal differentiation: Case report and literature review

“…EWS with extensive neuroectodermal differentiation usually occurs in adolescents and young adults, often between 20 to 30 years, with a male: female ratio of 3:1 [3,6,9,[15][16][17][18]. Renal localization of this tumor is sporadic and, as the neural markers are expressed, a neuroectodermal origin has been proposed [2,5].…”

“…As regards histogenesis, tumor cells derived from the neural tube can migrate into kidney and therefore undergo tumorigenesis. Its development from neural ramifications from celiac plexus that invest the kidney was also supposed [2,3,5,18].…”

“…Despite the multimodal treatment, the overall survival of patients with renal EWS with extensive neuroectodermal differentiation is dismal [3]. Factors with prognostic value are: primary tumor site and size, patient's age, stage of the disease, tumor dissemination, and the response or resistance to therapy [2,17].…”

“…EWS with extensive neuroectodermal differentiation was first described by Stout, as a PNET, in 1918, and was considered a variant of EWS, sharing the same pathologic features microscopically, immunohistochemically (IHC) and genetically [3,4]. It is described as a fast-growing small round cell tumor, which derives from the neural tube and shares ectodermal or neuronal differentiation abilities [5,6].…”