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The Desmoid Tumor Research Foundation

July 2026

Characteristics and outcome of intra-abdominal desmoid-type fibromatosis

Author Information

Nicolas Penel 1, Maud Toulmonde 2, Jennifer Wallet 3, Axel LE Cesne 4, Sylvie Bonvalot 5, Nadège Corradini 6, Thibaud Valentin 7, Loic Chaigneau 8, François Bertucci 9, Cécile Guillemet 10, Jacques Olivier Bay 11, Pascale Dubray-Longeras 12, Jean-Emmanuel Kurtz 13, Sébastien Salas 14, Laurence Bozec 15, Julien Thery 3, Marie-Cécile Le Deley 3, Jean-Yves Blay 16

1Department of Medical Oncology, Centre Oscar Lambret, Lille, France; Univ. Lille, CHU Lille, ULR 2694 - Metrics: Evaluation des technologies de santé et des pratiques médicales, F-59000, Lille, France. Electronic address: n.penel@bordeaux.unicancer.fr. 2Department of Medical Oncology, Institut Bergonié, Bordeaux, France. 3Department of Clinical Research and innovation, Centre Oscar-Lambret, Lille, France. 4Department of Medical Oncology, Gustave Roussy, Villejuif, France. 5Department of Surgical Oncology, Institut Curie, Paris, France. 6Institute of Pediatric Hematology and Oncology (IHOPE), Centre Leon Berard Center, Lyon, France. 7Department of Oncology, Toulouse Cancer Center (IUCT-Oncopole), OncoSarc, INSERM U1037, Toulouse Cancer Research Center (CRCT), France. 8Department of Medical Oncology, CHU Besançon, Besançon, France. 9Department of Medical Oncology, Institut Paoli-Calmettes, Aix-Marseille Université, Marseille, France. 10Department of Medical Oncology, Centre Henri Becquerel, Rouen, France. 11Hematology Department, Clermont-Ferrand University Hospital, Clermont-Ferrand, France. 12Department of Medical Oncology, Centre Jean Perrin, Clermont-Ferrand, France. 13Department of Medical Oncology, Institut de Cancérologie Strasbourg Europe, Strasbourg, France. 14Department of Medical Oncology, Assistance Publique des Hôpitaux de Marseille, Hôpital La Timone, Marseille, France. 15Department of Medical Oncology, Institut Curie, Saint-Cloud, France. 16Department of Medical Oncology, University Claude Bernard Lyon I, Department of Medicine, Léon Bérard Centre, Unicancer, Lyon, France.

Publication

Eur J Surg Oncol

Summary

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.

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