*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.
Ann Surg. 2014 May;259(5):973-8. doi: 10.1097/SLA.0000000000000224.
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.
To investigate the possible relationship between DF and pregnancy.
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.
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.
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.
许多患有韧带样纤维瘤病 (DF) 的女性都有近期妊娠史。关于妊娠期间和妊娠后疾病行为的长期数据尚缺乏。
探讨 DF 与妊娠之间的可能关系。
从 4 个肉瘤中心确定了一组患有 DF 和妊娠的女性患者。确定了 4 个组：诊断于妊娠期间 (A)；产后诊断 (B)；妊娠期间临床明显的 DF (C)；妊娠前切除的 DF (D)。分析了进展/消退率、切除后的复发率和产科结局。
共纳入 92 名女性。44 名女性（48%）存在与妊娠相关的 DF（A + B），而 48 名（52%）在受孕前有 DF 病史（C + D）。初始治疗为切除的占 52%，药物治疗的占 4%，观察等待的占 43%。A + B 中的手术后复发率为 13%，尽管观察等待中的进展率为 63%。C + D 中的复发/进展率为 42%。妊娠后，46%对 DF 进行了治疗，而 54%进行了观察等待。最终，仅有 17%在治疗后出现进一步进展。14%出现自发消退。进一步妊娠后，仅 27%出现进展。唯一相关的产科事件是剖宫产。
与妊娠相关的 DF 结局良好。妊娠期间进展风险高，但可安全管理。DF 不会增加产科风险，不应成为未来妊娠的禁忌证。
