Nonrhabdomyosarcoma Soft Tissue Sarcoma

Shahab Abdessalam, MD, Israel Fernandez-Pineda, MD
Nonrhabdomyosarcoma Soft Tissue Sarcoma is a topic covered in the Pediatric Surgery NaT.

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Soft tissue sarcomas constitute a broad group of tumors derived from mesenchymal tissues compromised of structural and connective tissue including muscle, endothelium, synovium, fat, lymphatics and fascia. The tumors generally acquire their name from the tissue they most closely resemble. There are up to fifty histologic subtype variants with their names changing as more information is gained at a cellular and molecular level [1]. With the tissue of origin located thorughout the body, these tumors can occur throughout the body and in all age groups. As a group they constitute the fifth most common malignancy in childhood and roughly five to seven percent of pediatric cancers.

They are divided into rhabdomyosarcoma (RMS) and nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) with each representing approximately 50% of the total number of sarcomas [2]. A primary differentiation is that RMS is relatively sensitive to radiation and chemotherapy while NRSTS are less sensitive and similar to their adult sarcoma counterparts. The exceptions are infantile fibrosarcoma and synovial cell sarcoma which are sensitive to standard chemotherapy.

These tumors are very rare and attempts at trying to study them have necessitated such grouping in order to gain valuable information about prognosis and different potential treatment strategies. As the genetics and biology of the various subtypes of sarcomas are elucidated, the term NRSTS most likely will fall out of favor. Evidence for the optimal treatment of these rare tumors is in evolution and successful treatment of patients with NRSTS depends on a multidisciplinary approach to therapy including oncology, surgery, radiation oncology, radiology and pathology.

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Last updated: December 27, 2017