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Surgery of soft tissue sarcomas in children
Insights
Multidisciplinary treatment has improved pediatric soft tissue sarcoma management, reducing amputations. Surgery, radiation, and chemotherapy are key for embryonal rhabdomyosarcoma and synovial sarcoma, while fibrosarcoma requires surgery alone.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Soft tissue sarcomas in children necessitate specialized management.
- Historically, major amputations and exenterations were common.
- Modern approaches emphasize limb and organ preservation.
Purpose of the Study:
- To review current management strategies for pediatric soft tissue sarcomas.
- To highlight the impact of multidisciplinary treatment.
- To discuss specific approaches for common subtypes like rhabdomyosarcoma, fibrosarcoma, and synovial sarcoma.
Main Methods:
- Review of surgical techniques and adjuvant therapies.
- Analysis of treatment protocols for different sarcoma types.
- Emphasis on multidisciplinary team approach.
Main Results:
- Multidisciplinary treatment significantly reduces the need for major amputations.
- Embryonal rhabdomyosarcoma (RMS) achieves 80% 2-year survival with combined modality treatment.
- Fibrosarcoma management relies on surgery alone, with a 90% salvage rate via resection and follow-up.
- Synovial sarcoma responds well to surgery and chemotherapy protocols similar to osteosarcoma.
Conclusions:
- Pediatric soft tissue sarcoma treatment has evolved towards less invasive, function-sparing approaches.
- Tailored multidisciplinary strategies are crucial for optimizing outcomes in different sarcoma subtypes.
- Continued research is needed for rarer soft tissue sarcomas in children.
Abstract:
Surgery of soft tissue sarcomas in children has been modified by the introduction of multidisciplinary treatment so that major amputation and exenterations are now rare in the management of these tumors in children. Surgery must still be well planned for total removal of the tumor with preservation of limbs and, in most instances, the function of pelvic organs. The commonest soft tissue sarcomas in children are embryonal rhabdomyosarcoma (RMS), fibrosarcoma, and synovial sarcoma. Treatment of embryonal RMS can now achieve a 2-year survival of 80% at all sites. Surgery and irradiation are used to control the primary tumor and multidrug chemotherapy to control metastases or prevent dissemination of localized tumor. Fibrosarcoma in children, usually a low-grade, extra-abdominal desmoid lesion, does not respond to radiation treatment or chemotherapy, and management is by surgery alone. It shows a marked tendency to local recurrence, and multiple local resections may be necessary for cure. Ninety percent of the children with this tumor can be salvaged by surgery and careful follow-up. The management of synovial sarcoma is surgical and similar to that of RMS. Although not radiosensitive as is RMS, this tumor has responded well to multidisciplinary treatment. Adequate resection is now followed by a chemotherapy protocol similar to that used in osteogenic sarcoma. The smaller numbers of these tumors and their varied natural history make evaluation of treatment difficult. Other soft tissue sarcomas seen with extreme rarity in children are liposarcoma, angiosarcoma, and neurosarcoma. These tumors are treated with the same protocol as that of RMS.