Rhabdomyosarcoma

Excerpt

Rhabdomyosarcoma (RMS) is a malignant soft tissue sarcoma that is believed to originate from primitive mesenchymal cells that typically differentiate into skeletal tissue. However, these tumors can also arise in other types of tissue and any anatomic area. RMS is the most common soft tissue sarcoma diagnosed in children; only 1% of these cancers are found in adults. The etiology and risk factors remain largely unknown. Most cases of rhabdomyosarcoma are sporadic; however, the disease can be associated with familial syndromes.

Rhabdomyosarcoma is classified into histologic subtypes: embryonal, alveolar, pleomorphic, spindle, and mixed-type. Embryonal is the most commonly occurring subtype and also has the best prognosis. The management approach is tailored to a patient's risk stratification. Two systems are used to characterize risk stratification for RMS: the Clinical Group or the Stage. Each system uses different factors to categorize patients according to risk, and most authorities use both to determine the most appropriate therapeutic approach. Currently, treatment is multimodal, combining surgery, when feasible, to completely remove the primary tumor and chemotherapy to control disease spread, even if no evidence of metastasis is present on imaging because most patients have been found to have micrometastasis in studies. Radiotherapy is used to treat most high and intermediate-risk patients as well. The survival of rhabdomyosarcoma patients has improved due to interprofessional collaboration, leading to advancements in diagnosis and management approaches. Healthcare professionals should seek to enhance their competence when managing rhabdomyosarcoma. Furthermore, they should have updated knowledge, skills, and strategies for timely diagnosis and effective interventions to improve patient outcomes and reduce morbidity.