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Desmoid-Type Fibromatosis and Pregnancy: A Multi-institutional Analysis of Recurrence and Obstetric Risk

Fiore, Marco MD*; Coppola, Sara MD; Cannell, Amanda J. BScH; Colombo, Chiara MD*; Bertagnolli, Monica M. MD§; George, Suzanne MD; Le Cesne, Axel MD; Gladdy, Rebecca A. MD, PhD; Casali, Paolo G. MD**; Swallow, Carol J. MD, PhD; Gronchi, Alessandro MD*; Bonvalot, Sylvie MD; Raut, Chandrajit P. MD, MSc§

Annals of Surgery:
doi: 10.1097/SLA.0000000000000224
Original Articles
Abstract

Background: Many women who present with desmoid-type fibromatosis (DF) have had a recent pregnancy. Long-term data about disease behavior during and after pregnancy are lacking.

Objective: To investigate the possible relationship between DF and pregnancy.

Patients and Methods: A cohort of women with DF and pregnancy was identified from 4 sarcoma centers. Four groups were identified: diagnosis during pregnancy (A); diagnosis after delivery (B); DF clinically evident during pregnancy (C); and DF resected before pregnancy (D). Progression/regression rates, recurrence rates after resection, and obstetric outcomes were analyzed.

Results: Ninety-two women were included. Forty-four women (48%) had pregnancy-related DF (A + B), whereas 48 (52%) had a history of DF before conception (C + D). Initial treatment was resection in 52%, medical therapy in 4%, and watchful waiting in 43%. Postsurgical relapse rate in A + B was 13%, although progression during watchful waiting was 63%. Relapse/progression in C + D was 42%. After pregnancy, 46% underwent treatment of DF, whereas 54% were managed with watchful waiting. Eventually, only 17% experienced further progression after treatment. Spontaneous regression occurred in 14%. After further pregnancies, only 27% progressed. The only related obstetric event was a cesarean delivery.

Conclusions: Pregnancy-related DF has good outcomes. Progression risk during pregnancy is high, but it can be safely managed. DF does not increase obstetric risk, and it should not be a contraindication to future pregnancy.

In Brief

The relation between desmoid-type fibromatosis and pregnancy is analyzed in a large international series. Pregnancy-related fibromatosis showed good outcomes, and conservative nonsurgical management was also feasible. Although disease progression was frequent during and after pregnancy, it could be safely managed in referral centers. No obstetric risks were described.

Author Information

*Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

Department of Surgery, Institut Gustave Roussy, Villejuif, France

Division of General Surgery, Mount Sinai Hospital; Department of Surgical Oncology, Princess Margaret Hospital; and Department of Surgery, University of Toronto, Toronto, Ontario, Canada

§Department of Surgery, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA

Department of Medical Oncology, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA

Department of Medical Oncology, Institut Gustave Roussy, Villejuif, France

**Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

Reprints: Marco Fiore, MD, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, Milan 20133, Italy. E-mail: marco.fiore@istitutotumori.mi.it or Chandrajit P. Raut, MD, MSc, Department of Surgery, Brigham and Women's Hospital, 75 Francis St, Boston, MA 02115. E-mail: craut@partners.org.

Disclosure: The authors declare no conflicts of interest.

© 2014 by Lippincott Williams & Wilkins.