Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival? Academic Article uri icon

Overview

abstract

  • BACKGROUND: Ewing sarcoma (ES) is the second most common primary osseous malignancy in childhood and adolescence. The improvement in survival is primarily associated with the combination of surgery and chemotherapy. HYPOTHESIS: Little is known about the outcome of adults with soft tissue ES or primitive neuroectodermal tumors (PNET). Certain prognostic factors from soft tissue sarcomas (tumor size, tumor location, margin status, and initial presentation) in adults (>16 years) with ES/PNET will help to identify factors associated with outcome. METHODS: Between July 1, 1982, and June 30, 2000, we identified 59 adult patients with primary soft tissue ES/PNET. Clinicopathologic factors were correlated with the end points studied: patient factors, tumor factors, pathologic factors, status of surgical margins, adjuvant chemotherapy, and radiation therapy. RESULTS: There were 41 male and 18 female patients, with a median age of 27 years (range, 16-72 years). Median tumor size was 8 cm, with all lesions being high grade. The most common site was the trunk (n = 22), with an even distribution of retroperitoneal, pelvis, buttock, and lower extremity (all n = 5). The median follow-up was 29 months (range, 6-222 months), with local recurrence identified in 13 patients (22%), with a median time to recurrence of 15 months (range, 5-200 months). Overall 5-year survival was 60%. Initial presentation was the only predictor of long-term survival, with primary tumor-only presentation having a 5-year survival of 60% (median not reached) compared with primary tumor plus metastatic disease having a 5-year survival of 33% (median, 17 months) (P =.02). CONCLUSION: Initial presentation of disease represents the only predictor of survival identified in this small group of adult patients with ES/PNET.

publication date

  • March 1, 2003

Research

keywords

  • Neuroectodermal Tumors, Primitive, Peripheral
  • Sarcoma, Ewing
  • Soft Tissue Neoplasms

Identity

Scopus Document Identifier

  • 0037336178

PubMed ID

  • 12611575

Additional Document Info

volume

  • 138

issue

  • 3