NCCN Guidelines for Soft Tissue Sarcoma V.1.

2019 – Web teleconference on 06/22/18

Guideline Page Panel Discussion/References Institution Vote

and Request
YES NO ABSTAIN ABSENT

DESMOID TUMORS Category change not made 0 16 1 10

External request: Based on a review of data and discussion, the panel consensus did not support
the designation of sorafenib as a category 1 option for both first-line and
Submission from Novartis subsequent therapy of patients with desmoid tumors due to insufficient data.
Pharmaceutical
Corporation to consider
including sorafenib as a
single agent to category 1a
for both first-line and
subsequent therapy of
patients with desmoid
tumors based on the results
of phase III randomized
double-blind study.

DESM-2 Addition at call

Internal request: Based on the discussion, the panel consensus was to include adjuvant RT as an 9 7 1 10
option following R1 resection, with the following footnote: “Consider RT for
Institutional Review lesions where recurrence would be technically challenging to resect and would
comment to consider the lead to significant morbidity.” This is a category 2B recommendation.
inclusion of adjuvant
radiation as a treatment
possibility for an R1
resection

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NCCN Guidelines for Soft Tissue Sarcoma V.1.2019 – Web teleconference on 06/22/18

SARC-C (1 of 3) Change made 16 0 1 10

External request: Based on a review of data and discussion, the panel consensus supported the
inclusion of NGS as another method of molecular genetic testing in the diagnosis
Submission from Illumina of sarcomas as follows, “Most molecular testing utilizes fluorescence in situ
Inc. to include the following hybridization (FISH) approaches or polymerase chain reaction (PCR)-based
statement for NGS, methods and next-generation sequencing (NGS)-based methods.”
“Molecular testing methods
include fluorescence in situ
hybridization (FISH),
polymerase chain reaction
(PCR) and next-generation
sequencing (NGS)-based
methods. NGS technology
allows simultaneous
detection of multiple
genetic aberration and
detects variant allele
frequencies (VAF) as low
as 5%.”

SARC-F (1 of 6) Addition at call 16 0 1 10

Internal request:
Institutional Review
comment to consider the
addition of regorafenib for
non-adipocytic sarcomas.
Based on the discussion, the panel consensus was to include regorafenib as a
single-agent option for treating soft tissue sarcoma subtypes with non-specific
histologies. A corresponding footnote designates the recommendation for use in
non-adipocytic sarcomas. This is a category 2A recommendation.
Reference:
Berry V, Basson L, Bogart E, et al. REGOSARC: Regorafenib versus placebo in
doxorubicin-refractory soft-tissue sarcoma-A quality-adjusted time without
symptoms of progression or toxicity analysis. Cancer 2017;123:2294-2302.

SARC-F (1 of 6) Change not made 3 11 3 10

Internal request: Based on a review of data and discussion, the panel consensus did not support
the inclusion of ipilimumab/nivolumab for UPS due to insufficient available data.
Institutional Review
comment to consider if
there is enough evidence to
include ipi/nivo for UPS.
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NCCN Guidelines for Soft Tissue Sarcoma V.1.2019 – Web teleconference on 06/22/18
SARC-F (1 of 6)
Internal request:
Institutional Review
comment to consider the
addition of pembrolizumab
as a potential treatment
option based upon the
results of SARC028.
SARC-F (1 of 6)
External request:
Submission from Novartis
Pharmaceutical
Corporation to consider
including pazopanib as a
systemic therapy agent in
soft tissue sarcoma
subtypes with non-specific
histologies for patients with
liposarcoma and removal of
footnote that pazopanib
should not be used for
lipogenic sarcomas.
Internal request:
Institutional review
comment to add pazopanib
as a systemic therapy
agent for SFT/HPC and
ASPS.
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Addition at call 7 5 5 10
Based on the discussion, the panel consensus was to include pembrolizumab for
the following:
 Alveolar Soft Part Sarcoma. This is a category 2B recommendation.
 Undifferentiated Pleomorphic Sarcoma (UPS). This is a category 2B
recommendation.
References:
Burgess MA, Bolejack V, Van Tine BA, et al. Multicenter phase II study of
pembrolizumab (P) in advanced soft tissue sarcoma (STS) and bone sarcomas
(BS): Final results of SARC028 and biomarker analyses. J Clin Oncol 2017;35
(Supplement; Abstract 11008).
Groisberg R, Hong DS, Behrang A, et al. Characteristics and outcomes of
patients with advanced sarcoma enrolled in early phase immunotherapy trials. J
Immunother Cancer 2017;5(1):100.
Addition at call 7 5 5 10
Based on the discussion, the panel consensus was to include pazopanib for the
following:
 Solitary Fibrous Tumor/Hemangiopericytoma. This is a category 2A
recommendation.
 Alveolar Soft Part Sarcoma. This is a category 2A recommendation.
 Removed footnote, “Pazopanib should not be used for lipogenic
sarcomas.
References:
Ebata T, Shimoi T, Bun S, et al. Efficacy and safety of pazopanib for recurrent or
metastatic solitary fibrous tumor. Oncology 2018;94:340-344.
Stacchiotti S, Mir O, Le Cesne A, et al. Activity of pazopanib and trabectedin in
advanced alveolar soft part sarcoma. Oncologist 2018; 23(1):62-70.
Samuels B, Chawla S, Somaiah N, et al. Results of a prospective phase 2 study
of pazopanib in patients with advanced intermediate-grade or high-grade
liposarcoma. Cancer. 2017; 123:4640-4647
Gilbert J. Pazopanib for advanced liposarcoma. Lancet Oncol. 2017;
18(10):e564.
NCCN Guidelines for Soft Tissue Sarcoma V.1.2019 – Web teleconference on 06/22/18

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