Small round cell sarcomas.
Small round cell sarcomas are the most aggressive of the tumors morphologically and clinically encountered in children and adults, and in some ways the most leukemia- or lymphoma-like of sarcomas. Small round cell sarcomas often are associated with chromosomal translocations, like hematologic malignancies, and are usually more sensitive to chemotherapy than other sarcoma subtypes. They have a high risk of mortality, but chemotherapy (in addition to surgery and often radiation therapy) provides a good cure rate, although treatment is often long and intensive. The biology of these tumors is very telling in terms of some of the mechanisms of cancer cell survival and proliferation. Although there is some overlap of the discussion below with the section on translocation associated sarcomas, we have highlighted some of the key issues with these sarcomas below, with some ideas that may bear fruit both in terms of the management of these, other sarcomas, and other cancers alike.
Lessnick
Stephen L
SL
Huntsman Cancer Institute, Salt Lake City, UT, USA.
Dei Tos
A Paolo
AP
Sorensen
Poul H B
PH
Dileo
Palma
P
Baker
Laurence H
LH
Ferrari
Stefano
S
Hall
Kirsten Sundby
KS
D050939:Gene Fusion; D000090322:Homeobox Protein Nkx-2.2; D018398:Homeodomain Proteins / Q000502:physiology; D006801:Humans; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D017526:Receptor, IGF Type 1 / Q000037:antagonists & inhibitors; D012509:Sarcoma / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D012512:Sarcoma, Ewing / Q000235:genetics / Q000473:pathology; D013584:Sarcoma, Synovial / Q000235:genetics / Q000473:pathology; D014157:Transcription Factors / Q000235:genetics / Q000502:physiology; D029961:Zebrafish Proteins
C106031:EWS-FLI fusion protein; D000090322:Homeobox Protein Nkx-2.2; D018398:Homeodomain Proteins; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors; D029961:Zebrafish Proteins; D017526:Receptor, IGF Type 1