Rhabdomyosarcoma (RMS) is a highly malignant tumor which is thought to originate from the pluripotent mesenchyme. It is the most common soft-tissue sarcoma of childhood. This review article summarizes the recent and older published literature and gives an overview of management of RMS in children. RMS can arise in a wide variety of primary sites, some of which are associated with specific patterns of local invasion, regional lymph nodal spread, therapeutic response and long term outcome, hence requiring physicians to be familiar with site-specific staging and treatment details. Most common primary sites include the head and neck region, genitourinary tract, and extremities. Prognosis for children and adolescents with RMS has recently improved substantially, especially for patients with local or locally extensive disease because of the development of multi-modal therapy incorporating surgery, dose-intensive combination chemotherapy, and radiation therapy. Despite aggressive approaches the outcome for patients who present with metastatic disease remains unsatisfactory. Clinical trials are ongoing to reduce toxicity and improve outcomes of such patients; newer agents in combination are being investigated.
Dramatic advancement has been made in the management of children with hepatoblastoma (HB) over the past 3 decades owing to the improvement in diagnostic imaging, new chemotherapeutic agents, better surgical care and availability of liver transplantation. These advances are the end results of contributions from 4 major study groups across the globe including International Society of Pediatric Oncology - Liver Tumor Strategy Group (SIOPEL), Children's Oncology Group (COG), German Pediatric Hematology Oncology Group (GPOH) and Japanese Pediatric Liver Tumor Study Group (JPLT). The current manuscript is written with the objective of developing a consensus guideline for practitioners at a National level. Based on literature and personal experience over last 3 decades, the Indian Council of Medical Research (ICMR) Expert group has made recommendations for management of children with HB in resource-challenged nations including India.
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