Pediatric soft tissue sarcomas

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.

Fig. 1

CT appearance of RMS. (A) Chest CT from a 16-year-old-boy who presented with a “lump on the back.” (B) Abdominal CT with intravenous and oral contrast from a 5-year-old girl who presented with abdominal pain and constipation.

Fig. 2

Survival IRS-I through IRS-IV. Improvement in survival with successive clinical trials. The overall survival curves for each IRS study are shown. (Courtesy of W. Meyer, University of Oklahoma, Oklahoma City, OK; with permission.)

Fig. 3

Postoperative nomogram for 12-year sarcoma-specific death. Points are added up for size (second line), depth (third line), site (fourth line), histology (fifth line), and patient age (sixth line). A line drawn down from the point total to the low-grade or high-grade line reveals the likelihood of sarcoma-specific death in 12 years. Fibro, fibrosarcoma; GR, grade; Lipo, liposarcoma; leiomyo, leiomyosarcoma; MFH, malignant fibrous histiocytoma; MPNT, malignant peripheral-nerve tumor; SSD, sarcoma-specific death. (From Kattan MW, Leung DH, Brennan MF. Postoperative nomogram for 12-year sarcoma-specific death. J Clin Oncol 2002;20(3):791–6; with permission.)