Desmoid-type fibromatosis and pregnancy: a multi-institutional analysis of recurrence and obstetric risk.
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.


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.
*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.
Marco
M
Fiore
Sara
S
Coppola
Amanda J
AJ
Cannell
Chiara
C
Colombo
Monica M
MM
Bertagnolli
Suzanne
S
George
Axel
A
Le Cesne
Rebecca A
RA
Gladdy
Paolo G
PG
Casali
Carol J
CJ
Swallow
Alessandro
A
Gronchi
Sylvie
S
Bonvalot
Chandrajit P
CP
Raut
D000293:Adolescent; D000328:Adult; D036861:Delivery, Obstetric / Q000706:statistics & numerical data*; D005260:Female; D018222:Desmoid Tumors / Q000175:diagnosis / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000453:epidemiology*; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic; D011256:Pregnancy Outcome; D011379:Prognosis; D011446:Prospective Studies; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D055815:Young Adult