Abstract
Simple SummaryThe Ewing sarcoma family of tumors (ESFT)s rarely originate in the kidneys and their treatment is significantly different from the other common kidney tumors. The standard treatment for ESFT of other sites includes multi-agent chemotherapy and local control with surgery and or radiation therapy. Limited information exists on the clinical behavior and management of ESFTs of the kidney. This study aims to describe our experience with these rare tumors over a period of 23 years at MD Anderson Cancer Center to identify potential prognostic factors and help develop management guidelines. We found that the 4-year overall survival for patients without metastasis was 85% compared to 47% for patients with metastasis. Patients with tumors confined to the kidney treated with nephrectomy and adjuvant chemotherapy have favorable outcomes. Local tumor extension beyond the kidney, tumor thrombus, and distant metastasis are unfavorable factors that warrant intensification or novel approaches of therapy.Limited information exists on the clinical behavior of the Ewing sarcoma family of tumors (ESFT) of the kidney. We reviewed the records of 30 patients (aged 8–69 years) with ESFT of the kidney seen at our institution between 1990 and 2013. We analyzed the event-free survival (EFS) and overall survival (OS) for associations with patient demographics, disease group, tumor size, tumor thrombus, and treatment. Six patients (20%) had tumors confined to the kidney (Group I), seven (23.3%) had local tumor extension beyond the kidney (Group II), and 17 (56.7%) had distant metastasis at diagnosis (Group III). Twenty-five (83.3%) patients underwent radical (19 upfront, five delayed) or partial (one upfront) nephrectomy, 25 (83.3%) chemotherapy and four (13.3%) radiotherapy. The 4-year EFS and OS were 43% (95% CI, 26–61%) and 63% (95% CI, 46–81%), respectively. EFS and OS were significantly associated with disease group and chemotherapy (p < 0.039). The presence of tumor thrombus in renal vein and/or inferior vena cava was associated with worse EFS (p = 0.053). Patients with disease confined to the kidney treated with nephrectomy and adjuvant chemotherapy have favorable outcomes. Local tumor extension beyond the kidney, tumor thrombus, and distant metastasis are unfavorable factors that warrant intensification or novel approaches of therapy.
Highlights
The Ewing sarcoma family of tumors (ESFT) represents a group of small round cell neoplasms including osseous and extra-osseous Ewing sarcoma (EWS), soft tissue primitive neuro-ectodermal tumors (PNET), and malignant small-cell tumor of the thoracopulmonary region (Askin’s tumor) [1].These highly aggressive sarcomas are poorly differentiated and commonly arise from the axial and appendicular skeleton of teenagers and young adults, but they develop from soft tissue [2].Extra-osseous EWS develops primarily in the trunk and extremities; rare sites including the retroperitoneum, head and neck, orbit, parameningeal, and genitourinary including bladder and prostate, have been described [3].ESFT of the kidney is a very rare entity representing less than 5% of renal tumors [4]
One patient was excluded from the analyses due to initial misdiagnosis and treatment as a renal cell carcinoma
In the 29 patients for whom data was available, tumor extension to the renal vein was present in two patients, to the inferior vena cava (IVC) in six, and to the IVC and right atrium in one
Summary
The Ewing sarcoma family of tumors (ESFT) represents a group of small round cell neoplasms including osseous and extra-osseous Ewing sarcoma (EWS), soft tissue primitive neuro-ectodermal tumors (PNET), and malignant small-cell tumor of the thoracopulmonary region (Askin’s tumor) [1].These highly aggressive sarcomas are poorly differentiated and commonly arise from the axial and appendicular skeleton of teenagers and young adults, but they develop from soft tissue [2].Extra-osseous EWS develops primarily in the trunk and extremities; rare sites including the retroperitoneum, head and neck, orbit, parameningeal, and genitourinary including bladder and prostate, have been described [3].ESFT of the kidney is a very rare entity representing less than 5% of renal tumors [4]. The Ewing sarcoma family of tumors (ESFT) represents a group of small round cell neoplasms including osseous and extra-osseous Ewing sarcoma (EWS), soft tissue primitive neuro-ectodermal tumors (PNET), and malignant small-cell tumor of the thoracopulmonary region (Askin’s tumor) [1]. These highly aggressive sarcomas are poorly differentiated and commonly arise from the axial and appendicular skeleton of teenagers and young adults, but they develop from soft tissue [2]. The most common chromosomal translocation results in an oncogenic EWS-FLI1 fusion protein involved in tumorigenesis. Presence of the fusion protein has been shown to recruit the BAF chromatin-remodeling complex ( known as SWI/SNF) to DNA binding sites, which dysregulates hundreds of genes and drives tumorigenesis [6,7]
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