Desmoid-type fibromatosis in the puerperium: a case requiring pelvic exenteration

Abstract

Desmoid-type fibromatosis (DF) is a rare, locally aggressive soft tissue tumor with no metastatic potential but a high recurrence rate. We present the case of a 28-year-old postpartum woman who developed a rapidly enlarging pelvic mass, which was later confirmed as DF via imaging and biopsy. The initial management with chemotherapy and radiotherapy was unsuccessful, necessitating radical surgical intervention with pelvic exenteration. Given its association with hormonal changes, particularly during pregnancy and the postpartum period, DF poses significant diagnostic and therapeutic challenges. This case underscores the importance of early recognition, multidisciplinary management, and individualized treatment strategies to optimize patient outcomes.

Keywords

• Desmoid fibromatosis
• postpartum period
• pelvic exenteration

Doğum sonrası dönemde desmoid tip fibromatozis: pelvik ekzenterasyon gerektiren bir olgu

Abstract

Desmoid tipi fibromatozis (DF), metastaz yapmayan ancak yüksek nüks oranına sahip, nadir görülen yerel agresif bir yumuşak doku tümörüdür. Bu olgu sunumunda, doğum sonrası dönemde hızla büyüyen bir pelvik kitle gelişen 28 yaşındaki bir kadının süreci ele alınmaktadır. Görüntüleme ve biyopsi ile DF tanısı doğrulandı. İlk tedavi olarak kemoterapi ve radyoterapi uygulanmasına rağmen başarılı olunamadı ve hastaya pelvik ekzenterasyon yapıldı. DF’nin özellikle gebelik ve doğum sonrası dönemde hormonal değişikliklerle ilişkili olması, tanı ve tedaviyi zorlaştırmaktadır. Bu olgu, erken tanı, multidisipliner yönetim ve bireyselleştirilmiş tedavi stratejilerinin hasta sonuçlarını iyileştirmedeki kritik rolünü vurgulamaktadır.

Keywords

• Desmoid fibromatosis
• postpartum period
• pelvic exenteration

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