First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece.
First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece.
Surg Oncol. 2022 May;41:101724. doi: 10.1016/j.suronc.2022.101724. Epub 2022 Feb 19.
Desmoid-type fibromatosis (DF) is a distinctly rare condition, mostly of younger adults, characterized by the development of locally aggressive tumors of mesenchymal origin. Desmoid tumors (DT) arise either sporadically or in association with FAP (familial adenomatous polyposis), although certain risk factors have also been identified, including pregnancy and antecedent surgical trauma. They can emerge from any connective tissue including muscle, fascia and aponeurosis and are therefore classified, according to location, as intra-abdominal, of the abdominal wall and extra-abdominal. Despite the lack of metastasizing potential, the course can be unpredictable. Various mutations of APC and β-catenin genes, among others, play a catalytic role in the pathogenesis of this neoplastic entity. Surgery has lost its traditional role as first line treatment of the disease and several other treatment methods are being considered. Cytotoxic chemotherapy, non-cytotoxic systemic therapy and targeted therapy have been revealed as part of different treatment regimens. Recent progress regarding DT biology and molecular pathways has led to the development of promising novel biological agents. In any case, a multidisciplinary approach is required and is gradually employed, espe-cially in intra-abdominal DTs. In this review, we aim to present current knowledge on DF and summarize current treatment regimens as well as their effectiveness, with emphasis on the intraperitoneal type of DT.
韧带样纤维瘤病（DF）是一种罕见的疾病，主要发生在年轻人，其特征是间叶来源的局部侵袭性肿瘤的发展。韧带样瘤（DT）要么是散发性的，要么与家族性腺瘤性息肉病（FAP）有关，尽管已经确定了某些危险因素，包括妊娠和先前的手术创伤。它们可以出现在任何结缔组织中，包括肌肉、筋膜和腱膜，因此根据位置分为腹腔内、腹壁和腹腔外。尽管缺乏转移潜能，但病程可能是不可预测的。APC 和 β-连环蛋白等多种基因突变在这种肿瘤实体的发病机制中发挥催化作用。手术已失去作为疾病一线治疗的传统作用，正在考虑其他几种治疗方法。细胞毒性化疗、非细胞毒性全身治疗和靶向治疗已被证明是不同治疗方案的一部分。最近关于 DT 生物学和分子途径的进展导致了有前途的新型生物制剂的开发。在任何情况下，都需要多学科的方法，并逐渐采用，特别是在腹腔内 DTs 中。在这篇综述中，我们旨在介绍 DF 的现有知识，并总结目前的治疗方案及其疗效，重点是腹腔内 DT。
