PDF(Pubmed)
Abstract：
Desmoid fibromatosis of the breast (also known as desmoid tumor of the breast) is a rare entity infrequently encountered by oncologists and surgeons caring for patients with breast disease. The current body of literature is highly reliant on case series and extrapolations from other sites of desmoid tumor-related disease. Much remains unclear regarding the pathological origins, natural history, and response to treatment of this condition. Traditional treatment strategies have centered on surgical resection, which may result in significantly disfiguring cosmetic and functional outcomes, frequent need for re-operation, and associated morbidity. There are limited data to support the superiority of upfront surgical resection when compared to medical therapy or watchful waiting strategies. Current treatment guidelines for desmoid tumors do not focus on the breast as a site of disease and are purposefully ambiguous due to the paucity of evidence available. We aim to review the literature concerning desmoid fibromatosis of the breast and propose an algorithm for current evidence-based management of this rare disease in the context of our experience with this pathology at a high-volume quaternary referral center.
摘要：
乳腺纤维瘤病（也称为乳腺纤维瘤）是肿瘤学家和外科医生护理乳腺疾病患者的罕见实体。当前的文献高度依赖于病例系列和来自硬纤维瘤相关疾病其他部位的外推。关于病理起源仍不清楚，自然史,以及对这种情况的治疗反应。传统的治疗策略以手术切除为中心，这可能会导致显著毁容的外观和功能结果，经常需要重新手术，和相关的发病率。与药物治疗或观察等待策略相比，支持前期手术切除的优越性的数据有限。当前针对韧带样肿瘤的治疗指南并未将乳房作为疾病部位，并且由于缺乏可用的证据而有目的地模棱两可。我们的目标是回顾有关乳腺纤维瘤病的文献，并根据我们在高容量四元转诊中心的病理学经验，提出一种当前对这种罕见疾病进行循证治疗的算法。
