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CLEAR CELL SARCOMA OF SOFT TISSUES IN CHILDREN AND YOUNG ADULTS: The St. Jude Children's Research Hospital Experience
- Source :
- Pediatric Hematology and Oncology. 16:539-544
- Publication Year :
- 1999
- Publisher :
- Informa UK Limited, 1999.
-
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.
- Subjects :
- Adult
medicine.medical_specialty
Soft Tissue Neoplasm
Adolescent
medicine.medical_treatment
Soft Tissue Neoplasms
Disease-Free Survival
medicine
Humans
Child
Survival rate
Retrospective Studies
business.industry
Infant
Soft tissue
Retrospective cohort study
Hematology
medicine.disease
Combined Modality Therapy
Surgery
Survival Rate
Radiation therapy
Treatment Outcome
Oncology
Localized disease
Pediatrics, Perinatology and Child Health
Sarcoma, Clear Cell
Clear-cell sarcoma
Sarcoma
business
Subjects
Details
- ISSN :
- 15210669 and 08880018
- Volume :
- 16
- Database :
- OpenAIRE
- Journal :
- Pediatric Hematology and Oncology
- Accession number :
- edsair.doi.dedup.....b520df1a923d4989215f5db18d6c12fc