Nicole Petrykowski, DNP, FNP-C, OCN , focuses on desmoid tumor treatment and management, emphasizing shared decision-making with patients. Initial management often involves observation with scans every 3-6 months. Treatment, using sorafenib or nirogacestat, is considered if pain impacts daily life or tumor progression exceeds 20% on imaging. Side effects, such as diarrhea, nausea, and skin rash, can occur with treatment. Patient education about the tumor and treatment is crucial. NCCN guidelines are used to inform treatment and surveillance.

Alexis Spence, PharmD, BCOP, shares how clinic pharmacists play a vital role in the care of sarcoma patients, especially those with rare desmoid tumors. They provide an extra layer of support, reviewing medications for potential interactions and ensuring renal and liver function are assessed before starting therapies. The pharmacist also recommends appropriate doses, manages side effects, and provides patient counseling on medications like nirogacestat and sorafenib. These medications require close monitoring for liver enzymes, electrolytes, blood pressure, and other side effects.

Anne Mailhot, PA-C, discusses how her clinic focuses on managing symptoms, primarily pain and quality of life, rather than solely the size of the desmoid tumor. Treatment begins when symptoms interfere with daily life, and its duration is guided by ongoing symptom evaluation and monitoring of tumor size through scans. Treatment may be paused if the tumor response plateaus, or symptoms are well-managed and resumed if they recur. Medication can be continued indefinitely if tolerated well, with a focus on discussing treatment breaks to reserve medication effectiveness. Testosterone levels in male patients on nirogacestat are monitored, as low levels are a potential side effect and may require replacement therapy.

Julie Messina, PA-C, explores desmoid tumors treatments and how they often use agents that can affect fertility. Treatment options include nirogacestat and sorafenib, but data on the effects of tyrosine kinase inhibitors on fertility is conflicting. For women, egg quantity declines with age, and treatment could cause earlier menopause. Men can make sperm if stem cells are intact. Fertility preservation options should be discussed prior to treatment and include freezing eggs, embryos, sperm, and testicular or ovarian tissue. Baseline evaluations of hormone levels can help assess fertility changes from nirogacestat.