European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology · 2026
Characteristics and outcome of intra-abdominal desmoid-type fibromatosis.
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Penel Nicolas, Toulmonde Maud, Wallet Jennifer, LE Cesne Axel, Bonvalot Sylvie, Corradini Nadège, Valentin Thibaud, Chaigneau Loic, Bertucci François, Guillemet Cécile, Bay Jacques Olivier, Dubray-Longeras Pascale, Kurtz Jean-Emmanuel, Salas Sébastien, Bozec Laurence, Thery Julien, Le Deley Marie-Cécile, Blay Jean-Yves
Аннотация
INTRODUCTION: Data about intra-abdominal desmoid-type fibromatosis (IA-DTFs) are limited.
METHODS: We examined patients with IA-DTFs enrolled in the ALTITUDES study (NCT02867033). We compared their characteristics with those of other locations using chi-square and Wilcoxon tests, as appropriate, and assessed their outcomes using event-free survival (EFS). Association between primary location and EFS was determined using a Cox univariate model. Subgroup analysis was performed for patients initially managed with active surveillance (AS).
RESULTS: 95/610 tumors (15.5%) enrolled in ALTITUDES were intra-abdominal. Compared with other locations, patients with IA-DTFs were older (p < 0.001), more often men (p < 0.001), had more frequently a history of polyposis (p = 0.001), larger tumors (p = 0.003), different CTNNB1 mutation profiles (p = 0.004), and a diagnosis established more frequently on surgical specimens (p < 0.001). These patients reported less pain (p = 0.01) and fewer emotional difficulties (p = 0.001), but more constipation (p = 0.004). Surgery was the most common first-line approach (51.6%); AS accounted for the management of only 29.5%. Overall, we observed no significant difference between IA-DTFs and other locations in terms of EFS (hazard ratio, HR = 0.84; 95%CI, 0.56-1.26) and overall survival (HR = 1.84; 0.50-6.80). Among patients managed by AS, EFS was similar between both groups (HR = 1.05; 0.55-2.00).
CONCLUSION: One third of IA-DTFs patients were managed using AS, and their outcome was similar to those of patients with other locations. These observational data may help to discuss SA as a first-line approach in the management of IA-DTFs patients.
METHODS: We examined patients with IA-DTFs enrolled in the ALTITUDES study (NCT02867033). We compared their characteristics with those of other locations using chi-square and Wilcoxon tests, as appropriate, and assessed their outcomes using event-free survival (EFS). Association between primary location and EFS was determined using a Cox univariate model. Subgroup analysis was performed for patients initially managed with active surveillance (AS).
RESULTS: 95/610 tumors (15.5%) enrolled in ALTITUDES were intra-abdominal. Compared with other locations, patients with IA-DTFs were older (p < 0.001), more often men (p < 0.001), had more frequently a history of polyposis (p = 0.001), larger tumors (p = 0.003), different CTNNB1 mutation profiles (p = 0.004), and a diagnosis established more frequently on surgical specimens (p < 0.001). These patients reported less pain (p = 0.01) and fewer emotional difficulties (p = 0.001), but more constipation (p = 0.004). Surgery was the most common first-line approach (51.6%); AS accounted for the management of only 29.5%. Overall, we observed no significant difference between IA-DTFs and other locations in terms of EFS (hazard ratio, HR = 0.84; 95%CI, 0.56-1.26) and overall survival (HR = 1.84; 0.50-6.80). Among patients managed by AS, EFS was similar between both groups (HR = 1.05; 0.55-2.00).
CONCLUSION: One third of IA-DTFs patients were managed using AS, and their outcome was similar to those of patients with other locations. These observational data may help to discuss SA as a first-line approach in the management of IA-DTFs patients.