Renal Primitive Neuroectodermal Tumor

Abstract

Primitive neuroectodermal tumor (PNET) is a malignant small round cell tumor and typically arises from bone or soft tissue in adolescents and young adults. Renal PNET is extraordinarily rare and exhibits highly aggressive biological behavior with poor prognosis.We present here a new case of renal PNET in a 31-year-old female. The patients were referred to our hospital because of left flank pain with nausea and vomiting for 1 week. A computed tomography scan revealed a 14.7 × 12.7 cm well-defined, unevenly mass lesion with both solid and cystic components and the tumor was not enhanced uniformly.A preoperative diagnosis of cystic renal cell carcinoma and urinary tract infection was made. The patient undergone anti-inflammatory therapy followed by a left radical nephrectomy. Taken with morphological pattern and immunohistochemical markers, a diagnosis of renal PNET was made. Two cycles of combined chemotherapy were executed. At the 14-month follow-up, no evidence of metastasis or recurrence was indicated.This case reminds clinicians that for adolescents and young adults with a suspicious renal mass, a diagnosis of renal PNET should be always considered. An initial surgery followed by radiotherapy and chemotherapy is suggested for the therapeutic management.

Primitive Neuroectodermal Tumor Presenting With a Large Subcapsular Hematoma in Pregnancy: A Case Report and Current Literature Review

“…The most common sites of metastases include the lung, liver, and abdominal lymph nodes [3]. The aggressive nature of this disease and the proclivity for advanced disease at presentation result in a 60-70% five-year survival for patients with non-metastatic disease and 20-30% five-year survival for patients with metastatic disease [5][6][7].…”

The prognostic factors of Ewing sarcoma/peripheral primitive neuroectodermal tumor: A retrospective analysis of 67 patients at a single center

“…Therefore, after review of the previous literature, we believe that ES/pPNET is most likely to occur in the soft tissue throughout the body, while bone and internal organs are the next most common sites. [ 5 , 11 ] The age of onset varies slightly from study to study. The median age of the 14 ES/pPNET patients described by Sun Pengtao et al was 34 years.…”