NCCN Guidelines ® Updates: Soft Tissue Sarcoma .
Volume 14 //Number 6 // June 2016 .
The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Soft Tissue Sarcoma published in this issue (page 754) include the latest updates. To assist readers interested in noting how the guidelines were updated, highlights of major changes pertaining to the abbreviated version published in this issue are printed below. To view the complete list of updates and full version of the guidelines, visit the NCCN Web site at NCCN.org.
Updates in Version 2.2016 of the NCCN Guidelines for Soft Tissue Sarcoma from Version 1.2015 include:
SARC-B (1 of 3)
• The following translocation has been modified:
! Embryonal RMS:
♦ Aberration: Complex alterations; Gene(s) involved: Multiple, MYOD1 mutation
• Footnote 2 has been modified: “This table is not exhaustive for either sarcomas with characteristic genetic changes or the genes involved. For example, additional genetic aberrations found in alveolar RMS including PAX3- NCOA1, PAX3-NCOA2, and PAX3-INO80D. CIC-DUX4 fusion is present in primitive round or short spindle cell sarcomas, resulting from translocation of t(4;19)(q35;q13) or t(10;19)(q26;q13). It is not clear if this is an entirely new subtype of sarcoma or a new subtype of Ewing sarcoma. BCOR-CCNB3 fusion is considered Ewing-like sarcoma. NCOA2 gene rearrangements and MyoD mutation have recently been identified in spindle cell RMS. MIR143-NOTCH fusion has recently been identified in glomus tumor. Receptor tyrosine kinase/RAS/PIK3CA aberrations are found in 93% of RMS cases. Loss of TSC1 (9q34) or TSC2 (16p13.3) (mTOR pathway) or gene fusions of the TFE3 gene (microphthalmia-associated transcription factor family) have been identified in PEComa. MPNST is associated with loss of SUZ12/EED and alteration of NF1 and CDKN2A. Consultation with a pathologist who has expertise in sarcoma diagnosis and molecular diagnostic techniques should be obtained prior to testing.”
SARC-D (1 of 4)
• “These guidelines are intended to treat the adult population. For adolescent and young adult patients, refer to the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology” is a new footnote.
SARC-E (1 of 6)
• Trabectedin was added as a single agent for soft tissue sarcoma subtypes with non-specific histologies and rhabdomyosarcoma.
• “For Soft Tissue Ewings, see NCCN Guidelines for Bone Cancer” is new to the page.
• Footnote “f”: “Recommended only for palliative therapy” has been added to the following:
! Gemcitabine and vinorelbine .
! Vinorelbine and low-dose cyclophosphamide .
! Trabectedin .
SARC-E (2 of 6)
• Palbociclib was added for Well-differentiated/Dedifferentiated liposarcoma (WD-DDLS) for retroperitoneal sarcomas .