CORR Insights®: PROMIS Function Scores Are Lower in Patients Who Underwent More Aggressive Local Treatment for Desmoid Tumors.

Abstract

During the last several decades, treatment for desmoid tumors has evolved away from surgery and toward fewer and lessinvasive operations. I believe this movement started when a study on Gardner’s syndrome (familial adenomatous polyposis) found that sulindac and indomethacin plus high-dose vitamin C caused regression of intestinal polyps, resulting in fewer colorectal cancers, as well as a decrease in desmoid tumors [10]. While selective estrogen-receptor inhibitors or of lowdose chemotherapy (methotrexate and vinblastine) are seeing wider use [11], the pendulum swung further away from surgery when a study found that negative margins did not predict remaining recurrence free, nor were positive margins routinely associated with local recurrence [2]. More-sophisticated, targeted therapies have achieved high response rates [4]. One study found an 87% lower risk of progression or death in a group treated with sorafenib than in the placebo group, although 12% still progressed while on the active drug [4]. Responses can be monitored by assessing the relative cellularity of the tumor, since it is the cellular component that can grow and shrink far more dramatically than the relatively stable fibrous component [4]. This approach has become the first-line treatment for desmoid tumors. However, the responses to targeted agents are timedependent, and can take many months; as a result, patients often are treated for 1 to 2 years. Despite prolonged therapy, patients had partial response rates of 33% by RESIST 1.1 criteria. The favorable news is that disease rarely progressed while on these targeted therapies. However, the toxicity of treatment can be severe. Palmar-plantar erythrodysesthesia (painful redness, swelling and sometimes blistering, often referred to as hand-foot syndrome) occurs in about 20% of patients and hypertension in 9.4% to 18.9% of patients [8]. In the current study, Newman and colleagues [7] use the Patient-Reported Outcomes Measurement Information System (PROMIS) to assess the quality of life (QOL) of patients treated for desmoid tumors. Because desmoid tumors are a local disease, where the treatment can be worse than the disease, QOL and patient satisfaction are very important outcomes to consider.