General Surgery Department, Hospital Ángeles Lomas, Mexico City, Mexico.
Gac Med Mex. 2020;156(5):439-445. doi: 10.24875/GMM.M20000440.
A literature review on desmoid tumors was carried out, which are tumors that affect soft tissues with a locally aggressive behavior and are unable to metastasize. Sporadic cases are located on the extremities and chest wall; hereditary cases have an intra-abdominal predilection, and those associated with pregnancy occur on the abdominal wall. Imaging techniques assess disease extension. Trucut biopsy is the study of choice for diagnosis. Mutations in the CTNNB1 or APC genes cause an abnormal accumulation of b-catenin within the cell. In this review, an emphasis is made on therapeutic strategies' evolution and change, and current tools for decision making are analyzed, as well as clinical outcomes. Radiation therapy can play a therapeutic or adjuvant role. Advances in the understanding of the disease have allowed establishing better targeted treatments with lower morbidity; however, there are still unanswered questions regarding the choice of the ideal candidate for surveillance and/or early treatment. Data related to quality of life are also presented, as well as the uncertainty generated by this diagnosis for both doctor and patient.
进行了关于硬纤维瘤的文献回顾,硬纤维瘤是一种影响软组织的肿瘤,具有局部侵袭性,不会转移。散发性病例位于四肢和胸壁;遗传性病例倾向于腹腔内,与妊娠相关的病例发生在腹壁。影像学技术评估疾病的扩展。Trucut 活检是诊断的首选研究。CTNNB1 或 APC 基因突变导致细胞内 b-连环蛋白异常积聚。在这篇综述中,重点介绍了治疗策略的演变和变化,分析了当前的决策工具以及临床结果。放射治疗可以发挥治疗或辅助作用。对疾病的认识的进步使得能够建立具有更低发病率的更好的靶向治疗;然而,对于监测和/或早期治疗的理想候选者的选择,仍有一些悬而未决的问题。还介绍了与生活质量相关的数据,以及该诊断对医生和患者带来的不确定性。