NCCN Guidelines for Soft Tissue Sarcoma V 1.
2021 – Annual on 08/25/20
Guideline Page
and Request
SARC-F (1 of 9)
Internal request:
Preferred regimens, subsequent lines of therapy we
specifically say 2A for eribulin for other subtypes, what
about trabectedin as a category 2A for other subtypes.
SARC-F (1 of 9)
Internal request:
Consider adding trabectedin (for myxoid liposarcoma)
under useful in certain circumstances.
SARC-F (1 of 9)
Internal request:
Consider moving the regimen MAID (mesna,
doxorubicin, ifosfamide, dacarbazine) from preferred,
first-line therapy advanced/metastatic to useful in
certain circumstances.
SARC-F (1 of 9)
Internal request:
Suggest moving pembrolizumab from other
recommended regimens for UPS to useful in certain
circumstances for subsequent lines of therapy for
advanced/metastatic STS with non-specific histologies.
SARC-F (1 of 9)
External request:
Foundation Medicine (6/23/20). Add pembrolizumab as
a treatment option useful in certain circumstances for
patients with unresectable or metastatic tumors with
tissue tumor mutational burden-high (TMB-H) ≥10
mutations/megabase, as determined by an FDA-
approved test, who have progressed following prior
treatment and who have no satisfactory alternative
treatment options. (SARC-F 1 of 9) and add a footnote
referencing the validation standards published in
Merino DM, et al. J Immunother Cancer
2020;8:e000147.
Institution Vote
Panel Discussion/References
YES NO ABSTAIN ABSENT
The panel consensus was to include a category 2A 20 0 0 7
preferred recommendation for trabectedin in other
subtypes.
The panel consensus was to include trabectedin (for 20 0 0 7
myxoid liposarcoma) under useful in certain
circumstances for neoadjuvant/adjuvant therapy for
soft tissue sarcoma subtypes with non-specific
histologies. This is a category 2A, useful in certain
circumstances recommendation.
The panel consensus was to move the regimen MAID 20 0 0 7
(mesna, doxorubicin, ifosfamide, dacarbazine) for
first-line therapy advanced/metastatic. This is a
category 2A, useful in certain circumstances
recommendation
The panel consensus was to move pembrolizumab 20 0 0 7
from other recommended regimens for UPS to useful
in certain circumstances for subsequent lines of
therapy for advanced/metastatic STS with non-
specific histologies. This is a category 2A, useful in
certain circumstances recommendation.
Based on a review of the data and discussion, the 0 20 0 7
panel consensus was not to include pembrolizumab
as a treatment option under useful in certain
circumstances for patients with unresectable or
metastatic tumors with tissue tumor mutational
burden-high (TMB-H) ≥10 mutations/megabase, as
determined by an FDA-approved test, who have
progressed following prior treatment and who have
no satisfactory alternative treatment options. (SARC-
F 1 of 9) and add a footnote referencing the
validation standards published in Merino DM, et al. J
Immunother Cancer 2020;8:e000147.
NCCN Guidelines for Soft Tissue Sarcoma V 1.2021 – Annual on 08/25/20

Guideline Page Panel Discussion/References Institution Vote

and Request YES NO ABSTAIN ABSENT
SARC-F (1 of 9) Based on a review of the data and discussion, the 0 20 0 7
External request: panel consensus was not to include pembrolizumab
Foundation Medicine (6/23/20). Add pembrolizumab as as a treatment option under useful in certain
a treatment option useful in certain circumstances for circumstances for patients with microsatellite
patients with microsatellite instability-high (MSI-H) instability-high (MSI-H) cancer for the treatment of
cancer for the treatment of adult and pediatric patients adult and pediatric patients with unresectable or
with unresectable or metastatic, microsatellite metastatic, microsatellite instability-high (MSI-H) or
instability-high (MSI-H) or mismatch repair deficient mismatch repair deficient tumors that have
tumors that have progressed following prior treatment progressed following prior treatment and who have
and who have no satisfactory alternative treatment no satisfactory alternative treatment options.
options.
SARC-F (2 of 9) Based on a review of the data and discussion, the 20 0 0 7
Internal request: panel consensus was to include extraskeletal
Include extraskeletal osteosarcoma with ifosfamide or osteosarcoma as a subtype.
platinum-based therapy (cisplatin/doxorubicin). ifosfamide or platinum-based therapy
(cisplatin/doxorubicin) are included as an option for
extraskeletal osteosarcoma. This is a category 2A
preferred recommendation.
SARC-F (2 of 9) Based on a review of the data and discussion, the 20 0 0 7
Internal request: panel consensus was to include pazopanib as a
Consider including pazopanib as a treatment option for treatment option for desmoid tumors. This is a
desmoid tumors. category 2A preferred regimen for Time to response
more critical recommendation.
• Toulmonde M, et al. Pazopanib or
methotrexate-vinblastine combination
chemotherapy in adult patients with
progressive desmoid tumors (DESMOPAZ):
a non-comparative, randomize, open-label,
multicentre, phase 2 study. Lancet Oncol.
2019;20:1263-1272.
SARC-F (2 of 9) The panel consensus was to move the requested 20 0 0 7
Internal request: regimens from preferred to useful in certain
Consider moving the following regimens for desmoid circumstances as follows:
tumors: • Sulindac or other nonsteroidal anti-
• Tamoxifen ± Sulindac inflammatory drugs (NSAIDs), including
• Toremifene celecoxib (for pain)
This is a category 2A recommendation
NCCN Guidelines for Soft Tissue Sarcoma V 1.2021 – Annual on 08/25/20

Guideline Page Panel Discussion/References Institution Vote

and Request YES NO ABSTAIN ABSENT
SARC-F (3 of 9) The panel consensus was to include alternating 20 0 0 7
Internal request: ifosfamide and etoposide with vincristine, doxorubicin
Consider adding ifosfamide and etoposide to and cyclophosphamide.
vincristine, doxorubicin and cyclophosphamide for Non-
Pleomorphic Rhabdomyosarcoma.
SARC-F (3 of 9) The panel consensus was to remove the following 20 0 0 7
Internal request: regimens:
Under Non-Pleomorphic Rhabdomyosarcoma consider Vincristine and dactinomycin
removing the following regimens: Doxorubicin
Vincristine and dactinomycin High-dose methotrexate
Doxorubicin Trabectedin
High-dose methotrexate
Trabectedin
SARC-F (4 of 9) Based on a review of the data and discussion, the 20 0 0 7
External request: panel consensus was to include brigatinib for
Please consider the following update: Under Inflammatory Myofibroblastic tumor (IMT) with ALK
Inflammatory Myofibroblastic tumor (IMT) with ALK Translocation. This is a category 2A preferred
Translocation, Preferred regimens, ALK inhibitors: add recommendation.
Brigatinib.
SARC-F (5 of 9) Based on a review of the data and discussion, the 0 20 0 7
Internal request: panel consensus was not to include abemaciclib.
Add abemaciclib to the useful under certain
circumstances for Well-Differentiated/Dedifferentiated
Liposarcoma.
SARC-F (5 of 9) Based on a review of the data and discussion, the 0 20 0 7
External request: panel consensus was not to include pembrolizumab,
Submission from Merck & Co., Inc. (4/29/20). We either 200 mg every 3 weeks or 400 mg every 6
request the inclusion of the updated dosing weeks administered as a 30-minute intravenous (IV)
recommendations for pembrolizumab, either 200 mg infusion until disease progression, unacceptable
every 3 weeks or 400 mg every 6 weeks administered toxicity, or up to 24 months for the treatment of adult
as a 30-minute intravenous (IV) infusion until disease patients with microsatellite instability-high (MSI-H)
progression, unacceptable toxicity, or up to 24 months soft tissue sarcoma.
for the treatment of adult patients with microsatellite
instability-high (MSI-H) soft tissue sarcoma.
SARC-F (5 of 9) Based on a review of the data and discussion, the 0 20 0 7
External request: panel consensus was not to include pembrolizumab
Submission from Merck & Co., Inc. (3/20/20). We in the treatment of advanced MSI-H soft tissue
request that the recommendation for pembrolizumab in sarcoma be added as a category 2A based on the
the treatment of advanced MSI-H soft tissue sarcoma efficacy data from the publication by Marabelle et al.
NCCN Guidelines for Soft Tissue Sarcoma V 1.2021 – Annual on 08/25/20

be added as a category 2A based on the efficacy data

from the publication by Marabelle et al.
NCCN Guidelines for Soft Tissue Sarcoma V 1.2021 – Annual on 08/25/20

Guideline Page Panel Discussion/References Institution Vote

and Request YES NO ABSTAIN ABSENT
SARC-F (5 of 9) Based on a review of the data and discussion, the 0 20 0 7
External request: panel consensus was not to include the updated
Submission from Merck & Co., Inc. (3/11/20). We publication by Marabelle et al, be used as a reference
request that the updated publication by Marabelle et al, in the NCCN Guidelines for MSI-H sarcoma and
be used as a reference in the NCCN Guidelines for recommendation for pembrolizumab be a category
MSI-H sarcoma and recommendation for 2A.
pembrolizumab be a category 2A.