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Current management of pediatric soft tissue sarcomas
#MMPMID26566481
Sangkhathat S
World J Clin Pediatr
2015[Nov]; 4
(4
): 94-105
PMID26566481
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Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within
embryonic mesenchymal tissues during the process of differentiation into muscle,
fascia and fat. The tumors have a biphasic peak for age of incidence.
Rhabdomyosarcoma (RMS) is diagnosed more frequently in younger children, whereas
adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents.
The latter group comprises a variety of rare tumors for which diagnosis can be
difficult and typically requires special studies, including immunohistochemistry
and molecular genetic analysis. Current management for the majority of pediatric
sarcomas is based on the data from large multi-institutional trials, which has
led to great improvements in outcomes over recent decades. Although surgery
remains the mainstay of treatment, the curative aim cannot be achieved without
adjuvant treatment. Pre-treatment staging and risk classification are of prime
importance in selecting an effective treatment protocol. Tumor resectability, the
response to induction chemotherapy, and radiation generally determine the
risk-group, and these factors are functions of tumor site, size and biology.
Surgery provides the best choice of local control of small resectable tumors in a
favorable site. Radiation therapy is added when surgery leaves residual disease
or there is evidence of regional spread. Chemotherapy aims to reduce the risk of
relapse and improve overall survival. In addition, upfront chemotherapy reduces
the aggressiveness of the required surgery and helps preserve organ function in a
number of cases. Long-term survival in low-risk sarcomas is feasible, and the
intensity of treatment can be reduced. In high-risk sarcoma, current research is
allowing more effective disease control.
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