What is pediatric rhabdomyosarcoma (RMS)?

Updated: Mar 28, 2019
  • Author: Roshni Dasgupta, MD, MPH; Chief Editor: Eugene S Kim, MD, FACS, FAAP  more...
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Answer

Rhabdomyosarcoma (RMS) is a malignancy that arises from embryonic mesenchymal cells and is the most common sarcoma in the pediatric population, accounting for 4.5% of all childhood malignancies. It was first described in the English literature in 1937. In 1946, Stout described rhabdomyosarcoma as a tumor with rhabdomyoblasts of round, strap, racquet, and spider forms. [1]

Before the evolution of multimodal treatment for malignancies and the development and recognition of effective chemotherapeutic agents, the primary therapy for pediatric RMS was surgical excision. Unfortunately, this purely surgical approach was often unsuccessful and, even when curative, often proved to be quite morbid.

Formal investigations of the treatment of pediatric rhabdomyosarcoma have subsequently been carried out through the Intergroup Rhabdomyosarcoma Study (IRS) Group (IRSG) and have consisted of five studies: IRS-I (1972-1978), IRS-II (1978-1984), IRS-III (984-1991), IRS-IV (1991-1997), and IRS V (1997-2003). [2, 3, 4, 5, 6, 7, 8] Studies have been initiated by the Soft Tissue Sarcoma Committee of the Children's Oncology Group, which attempts to improve outcome and decrease treatment-related morbidity. [9, 10]

Treatment of RMS, once purely surgical, is now multimodal, involving surgical resection, biopsy only, or surgical staging, combined with chemotherapy and radiation therapy, when necessary.

The prognosis of RMS depends on tumor location, patient age, metastasis, histology, tumor biology, and adequacy of tumor resection. Lymph node evaluation is essential in determining the extent of disease and guiding therapy. With adequate treatment, the 5-year survival rate is higher than 70-80%.


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