Clear cell sarcoma of soft tissues in children and young adults: The St. Jude Children's Research Hospital experience

Sudha Parasuraman, Bhaskar N. Rao, Sara Bodner, Alvida Cain, Charles B. Pratt, Thomas E. Merchant, Alberto S. Pappo

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

Clear cell sarcoma is a rare soft tissue neoplasm whose clinical behavior and outcome has not been previously characterized. This study reviewed the clinical characteristics and outcome of all children with clear cell sarcoma of the soft tissues who were treated at St. Jude Children's Research Hospital from March 1962 through August 1998. Of 225 children with nonrhabdomyosarcomatous soft tissue sarcomas, 5 (2.2%) were diagnosed with clear cell sarcoma. Median age at diagnosis was 15 years 3 months. Primary sites included the extremities (n = 3), chest wall (n = 1), and abdomen (n = 1). At diagnosis 3 patients had localized disease. Following surgical resection (n = 3), radiotherapy (n = 2), and chemotherapy (n = 1) all three survive disease-free 10, 11, and 90 months after diagnosis, respectively. The remaining two patients with metastatic disease at diagnosis died 21 days and 9 months after diagnosis. Clear cell sarcoma of the soft tissues is rare in pediatrics. Complete surgical resection with negative margins is the most effective treatment for this disease. Patients with metastatic disease are candidates for multiinstitutional chemotherapy trials.

Original languageEnglish (US)
Pages (from-to)539-544
Number of pages6
JournalPediatric Hematology and Oncology
Volume16
Issue number6
DOIs
StatePublished - Nov 1999

Keywords

  • Clear cell sarcoma
  • Pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology
  • Cancer Research
  • Management of Technology and Innovation

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