Updates in Desmoid Tumors for the Community Oncologist:
Integrating Recent Guidelines, Novel Mechanisms, and Multidisciplinary Management
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Which of the following regimens are NCCN Category 1 recommended systemic therapies for the
treatment of desmoid tumors? (Choose all that apply)
Liposomal doxorubicin
Pazopanib
Nirogacestat
Sorafenib
Methotrexate-vinblastine/vinorelbine
Imatinib
Doxorubicin +/-dacarbazine
You are considering systemic therapy in a 22-year-old pregnant woman, currently in her 2nd trimester,
with a large, symptomatic intraabdominal desmoid tumor, at the root of the mesentery, for whom
surgery is not currently an option. You make the patient aware of the risks to her pregnancy if she takes
systemic therapy, but she is likely to lose the baby if the tumor continues to grow. As such, which one of
the following treatment options would you prescribe, while closely monitoring the pregnancy?
Nirogacestat
Doxorubicin
Pazopanib
Sorafenib
A 63-year-old woman with a recurrent desmoid tumor in her right thigh and lower limb has had several
surgeries to reduce symptoms associated with compression of nearby nerves. The most recent growth is
not amenable to surgery and has progressed by >20% as measured by RECIST v1.1 since the most recent
scan 5 months ago, along with an ECOG performance status of ≤2. You begin treating her with 150 mg
nirogacestat, twice daily. In this patient, which of the following side effects would concern you the most?
Hepatotoxicity
Ovarian toxicity
Upper respiratory tract infections
Headaches
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