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2015 ; 7
(ä): 59
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Recent advances in understanding and managing rhabdomyosarcoma
#MMPMID26097732
Hiniker SM
; Donaldson SS
F1000Prime Rep
2015[]; 7
(ä): 59
PMID26097732
show ga
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.