Recent advances in understanding and managing rhabdomyosarcoma

Abstract

Rhabdomyosarcoma is the most common childhood soft tissue sarcoma and the fourth most common pediatric solid tumor. For most patients, treatment consists of a multimodality approach, including chemotherapy, surgery, and/or radiotherapy. To guide treatment, patients with rhabdomyosarcoma are risk stratified based on a number of factors. These factors include clinical group, which depends largely on the extent of resection and nodal involvement, and stage, which takes into account tumor size, invasion, nodal involvement, and disease site. Histology of the tumor and age at diagnosis are also factored into risk stratification. Recent advances in understanding the biology of the disease have allowed for the further sub-classification of rhabdomyosarcoma. In addition, elucidation of additional clinical features associated with poor prognosis has allowed for better understanding of risk and provides more clarity regarding those patients who require more intensive therapy. Many areas of active investigation are ongoing, including the following: further delineation of the biological underpinnings of the various disease subtypes with the possibility of molecularly targeted therapy; a better understanding of clinical risk factors, including the evaluation and management of potentially involved lymph nodes; determination of the appropriate role of post-treatment imaging and assessment of response to therapy; and incorporation of advanced radiotherapeutic techniques, including conformal intensity-modulated photon and proton therapy.

Clinicopathologic traits and prognostic factors associated with pediatric sinonasal rhabdomyosarcoma

“…The IRSG was developed with the purpose of pooling international data to effectively study this childhood tumor, and, since its establishment in 1972, the prognosis of this disease has improved gradually . It developed both a pretreatment staging classification and a postsurgical clinical group classification based on residual disease to predict prognosis as well as for use in clinical trials .…”

Soft tissue sarcoma nomograms and their incorporation into practice

“…Moreover, large tumor size, the presence of local invasion, parameningeal site, age <3 years or >10 years, tumor stage, and lack of response to induction therapies all have emerged as negative prognostic factors. Furthermore, the use of surgery and the administration of radiotherapy were found to be associated with oncological outcome …”

Incidence and Histomorphological Evaluation of Paediatric Non-Haematological Malignant Tumours in a Tertiary Care Hospital

“…13,14,15 are malignant neoplasm, which show morphologic, immunohistochemical, and ultrastructural or molecular genetic evidence of primary skeletal muscle differentiation usually in the absence of any other pattern of differentiation.…”