Chemotherapy is Effective in Primitive Neuroectodermal Tumor (PNET) / Ewing Sarcoma (EWS) of The Kidney

Renal Ewing’s sarcoma/primitive neuroectodermal tumor (PNET): a case series of 7 patients and literature review

“…Risi et al summarized the clinical data of 116 reported renal PNET patients in 2012. For patients with distant metastasis, the median DFS was only 5 months, and the 18-month survival rate was only 60%, which was significantly lower than that of patients without metastasis (85%) (1).…”

“…The typical "renal cancer triad syndrome" (low back pain, hematuria and abdominal mass) can be observed in renal PNET. In addition, irregular fever and weight loss can be clinical manifestations (1). Renal PNET also has no specificity in imaging examinations; it often manifests as a single, large mass without obvious boundaries in CT, MRI or ultrasonography.…”

“…The 5-year survival rate of PNET is only 45-55%, and the median recurrence-free survival (RFS) is only 2 years for advanced PNET (1,20). Similar to other PNETs, renal PNETs are high-grade malignant.…”

“…Renal PNET is more common in young adults and older children (median age: 29 years old, 4-61 years old), and the male-to-female incidence ratio is 2:1-3:1. Renal PNET has a very poor prognosis; the 2-year survival rate of patients after radical nephrectomy was 80% and that of patients without radical nephrectomy was 30% (1). Forty percent of patients had complications Original Article Renal Ewing's sarcoma/primitive neuroectodermal tumor (PNET): a case series of 7 patients and literature review with tumor thrombosis and distant metastasis at diagnosis, with a median disease-free survival (DFS) time of 5 months and a median survival time of 24 months (2,3).…”

“…Surgery is currently the main treatment strategy, especially radical nephrectomy. Adjuvant radiotherapy is also required for patients with positive margins or late-stage disease, and neoadjuvant and adjuvant chemotherapy may prolong survival in patients with metastases (1,3,6).…”