Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin

Abstract

A primitive neuroectodermal tumor (PNET) is a small round cell tumor that arises from the nerve crest. This tumor usually occurs in the central nervous system or soft tissue, but it can occur in the kidney in rare cases. Herein we report a case with severe multiple liver metastases after surgery for right renal PNET. The patient was a 21-year-old man with a chief complaint of right abdominal pain. Hemorrhage in a right renal malignant tumor was diagnosed, and radical nephrectomy was performed. Histopathology showed bare nuclear round atypical cells with a scarce cytoplasm proliferating like a seat and nest. Some of the cells formed a rosette structure and the tumor cells were positive for CD99, leading to diagnosis of PNET. Severe multiple liver metastases occurred 6 months after surgery, and six courses of chemotherapy with ifosfamide, etoposide and doxorubicin were performed. After this treatment, residual tumor was removed, but the tumor cells were absent histologically.

Primitive Neuroectodermal Tumors of the Kidney

“…Th e common presenting symptoms are fl ank or abdominal pain, mass in the abdomen, and hematuria (6). Patients are usually asymptomatic until the tumor reaches a large size; the maximum diameter of Primitive neuroectodermal tumors of the kidney Geetha Narayanan, MD, DM, Varun Rajan, MD, and T. R. Preethi, MD such tumors is often 10 cm (7,8). Systemic symptoms such as weight loss and fever may also occur (9).…”

Primary Ewing sarcoma of the kidney: a symptomatic presentation and review of the literature

“…These tumors are neural crest cells in origin belonging to a family of tumors called primitive neuroectoderm tumors (PNETs). The term peripheral primitive neuroectoderm tumors (pPNETs) was coined to encompass peripherally [Seemayer et al 1975;Ohgaki et al 2010].…”

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

“…It can occur in the trunk, extremities, brain, spinal cord and sympathetic nervous system as well as peripheral tissues, paraspinal region, and less commonly, genitourinary tract [1] . Renal PNET was first described by Seemayar et al [2,3] . Renal PNET usually occurs in children and young adults.…”

“…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.…”

“…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. Renal PNET is a highly aggressive tumour with high metastatic potential and almost 50% of patient present with distant metastases, most commonlyto regional lymph nodes, lungs liver and bones [1][2][3]11,12] . Prognostic factors are tumour stage, grade, patient's age, extent of surgery, surgical margin status and treatment beginning time [2,11] .…”