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Published on: October 14, 2016
Pediatric soft tissue sarcomas
David M Loeb1, Katherine Thornton, Ori Shokek
1Oncology and Pediatrics, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University, Bunting-Blaustein Cancer Research Building, Baltimore, MD 21231, USA. loebda@jhmi.edu
Insights
Pediatric soft tissue sarcomas, including rhabdomyosarcoma (RMS) and non-RMS soft tissue sarcomas (NRSTS), are rare cancers. This review covers their causes, biology, and treatment options.
Area of Science:
- Pediatric Oncology
- Cancer Biology
- Surgical Oncology
Background:
- Soft tissue sarcomas (STS) are rare in children, with approximately 850-900 annual diagnoses in the US.
- Rhabdomyosarcoma (RMS) predominantly affects younger children (≤14 years), while non-RMS STS (NRSTS) are more common in adolescents and young adults.
- Infants with NRSTS present unique histological features.
Purpose of the Study:
- To provide a comprehensive overview of pediatric soft tissue sarcomas.
- To discuss the etiology, biology, and current treatment modalities for these rare childhood cancers.
Main Methods:
- This is a review article.
- Information was gathered from existing literature on pediatric soft tissue sarcomas.
Main Results:
- Surgery is a primary treatment for pediatric STS.
- Radiation therapy is also a significant component of treatment.
- Adjuvant chemotherapy is standard for RMS, while chemotherapy for NRSTS is reserved for high-grade or unresectable cases.
Conclusions:
- Pediatric soft tissue sarcomas require a multidisciplinary approach involving surgery, radiation, and chemotherapy.
- Understanding the distinct biology of RMS and NRSTS is crucial for effective treatment stratification.
Abstract:
Soft tissue sarcomas in children are rare. Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or a non-RMS soft tissue sarcoma (NRSTS). RMS is more common in children 14 years old and younger and NRSTS in adolescents and young adults. Infants get NRSTS, but their tumors constitute a distinctive set of histologies. Surgery is a major therapeutic modality and radiation plays a role. RMS is treated with adjuvant chemotherapy, whereas chemotherapy is reserved for the NRSTS that are high grade or unresectable. This review discusses the etiology, biology, and treatment of pediatric soft tissue sarcomas.
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