Pain in desmoid‐type fibromatosis: Prevalence, determinants and prognosis value

Abstract

The aim of this study is to evaluate the prevalence, determinants and prognostic value of pain at diagnosis in patients with desmoid‐type fibromatosis (DF). We selected patients from the ALTITUDES cohort (NCT02867033), managed by surgery, active surveillance or systemic treatments, with pain assessment at diagnosis. Patients were invited to fill QLQ‐C30 questionnaire and Hospital Anxiety Depression Scale. Determinants were identified using logistic models. Prognostic value on event‐free survival (EFS) was evaluated using the Cox model. Overall, 382 patients were included in the current study (median age: 40.2 years; 117 men). The prevalence of pain was 36%, without significant difference according to first‐line treatment (P = .18). In the multivariate analysis, pain was significantly associated with tumor size >50 mm (P = .013) and tumor site (P < .001); pain was more frequent in the neck and shoulder locations (odds ratio: 3.05 [1.27‐7.29]). Pain at baseline was significantly associated with poor quality of life (P < .001), depression (P = .02), lower performance status (P = .03) and functional impairment (P = .001); we also observed a nonsignificant association with anxiety (P = .10). In the univariate analysis, baseline pain was associated with poor EFS; the 3‐year EFS was 54% in patients with pain compared to 72% in those without pain. After adjustment for sex, age, size and line of treatment, pain was still associated with poor EFS (hazard ratio: 1.82 [1.23‐2.68], P = .003). One third of recently diagnosed patients with DF experienced pain, especially those with larger tumors and neck/shoulder locations. Pain was associated with unfavorable EFS after adjustment for the confounders.

Case report: Rapidly progressive desmoid tumor after surgery for esophagogastric junction cancer and slowly progressive primary desmoid tumor: a report of two cases and literature review

“…Pain is a common symptom of desmoid tumor and is often related to the size and location of the tumor. Studies have shown that one-third of confirmed desmoid tumor patients experience pain, particularly those with tumors larger than 5 cm or located in the neck or shoulder region (7). In these current cases, neither patient felt significant pain, probably because the affected area was non-invasively located in the mesentery.…”

Desmoid Tumors: A Comprehensive Review

“…The presence of pain was associated with lower performance status ( P = 0.024) and functional impairment ( P = 0.001). The 3-year event-free survival probability in the absence of baseline pain was 72.2% (95% CI, 65.1–78.1) and in the presence of baseline pain 53.9% (95% CI, 43.3–63.4) [ 43 ]. In a phase 3 trial of an investigational agent, nirogacestat (DeFi trial, n = 142), 41% of patients with progressing DT had uncontrolled pain at baseline, defined as average worst pain intensity score of > 4 as measured using the Brief Pain Inventory-Short Form (range 0–10, with higher scores indicating worse pain) [ 44 ].…”

“…In a phase 3 trial of an investigational agent, nirogacestat (DeFi trial, n = 142), 41% of patients with progressing DT had uncontrolled pain at baseline, defined as average worst pain intensity score of > 4 as measured using the Brief Pain Inventory-Short Form (range 0–10, with higher scores indicating worse pain) [ 44 ]. The pain severity and associated burden of disease can lead to anxiety and depression in many patients [ 4 , 43 , 45 ].…”

A Case of Painful Growing Abdominal Wall Mass during Pregnancy Requiring Resection in the Second Trimester

“…There are rare cases reported of desmoid tumors in extra-abdominal sites such as the vulva, larynx, neck, and popliteal fossa [ 13 , 14 ]. Furthermore, a recent study involving 382 patients with desmoid-type fibromatosis found that the prevalence of pain was only 36%, thereby highlighting the relatively uncommon nature of this symptom in the condition [ 15 ].…”