Primary ewing sarcoma/primitive neuroectodermal tumor of the kidney: The md anderson cancer center experience

Nidale Tarek*, Rabih Said, Clark R. Andersen, Tina S. Suki, Jessica Foglesong, Cynthia E. Herzog, Nizar M. Tannir, Shreyaskumar Patel, Ravin Ratan, Joseph A. Ludwig, Najat C. Daw

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

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.

Original languageEnglish (US)
Article number2927
Pages (from-to)1-15
Number of pages15
JournalCancers
Volume12
Issue number10
DOIs
StatePublished - Oct 2 2020
Externally publishedYes

Keywords

  • Chemotherapy
  • Ewing sarcoma
  • Kidney
  • Outcome
  • Renal
  • Survival
  • Treatment
  • Tumor thrombus

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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