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The Desmoid Tumor Research Foundation

November 2022

Pregnancy-associated desmoid fibromatosis: A Dutch multi-centre retrospective study

Author Information

C Drabbe C (1), van der Graaf WTA (1), Husson O (2), Bonenkamp JJ (3), Verhoef C (4), van Houdt WJ (5).

(1) Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands; Department of Medical Oncology, ErasmusMC Cancer Institute, Erasmus Medical Center, Rotterdam, the Netherlands. (2) Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands; Department of Surgical Oncology, ErasmusMC Cancer Institute, Erasmus Medical Center Rotterdam, the Netherlands; Division of Clinical Studies, Institute of Cancer Research, London, United Kingdom. (3) Department of Surgical Oncology, Radboud University Medical Center, Nijmegen, the Netherlands. (4) Department of Surgical Oncology, ErasmusMC Cancer Institute, Erasmus Medical Center Rotterdam, the Netherlands. (5) Department of Surgical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands. Electronic address: w.v.houdt@nki.nl.

Publication

Eur J Surg Oncol

Summary

Introduction: The development of desmoid fibromatosis (DF) is associated with pregnancy. The current treatment consensus recommends active surveillance (AS). However, data in pregnancy-associated DF is scarce and it is uncertain whether AS is the best management strategy for this DF-subgroup. The aim of this study was to describe demographic, tumor, obstetric, treatment characteristics and treatment outcome in pregnancy-associated DF. Methods: Female DF patients who were 18-50 years old at time of diagnosis (2000-2020) and had a history (≤5 years) of pregnancy at time of diagnosis were included. Results: Overall, 62 patients were included. The most common locations were abdominal wall (74%), pelvis (10%) and extremities (10%). Mutational analysis was conducted in 31 patients of which 94% had CTNNB1-mutations. Ten patients (16%) were diagnosed during pregnancy, while the remainder were diagnosed after pregnancy with a median time from delivery to diagnosis of 19 months (1-60). The frontline management was AS in 38 patients (61%) of whom 12 (33%) developed progressive disease and surgery in 23 patients (37%). In total, 30 patients underwent surgery and five had local recurrence (17%). Positive resection margins were no prognostic factor. Nine patients received systemic treatment in second- or third-line. Conclusions: Pregnancy-associated DF generally has an indolent behavior, where our results underscore the difficulty of establishing a clear definition of this entity. This study shows that AS should be the frontline management strategy for pregnancy-associated DF. When active treatment is indicated, surgery is a good option with low recurrence rates, even with positive (R1) resection margins.

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