Jannasch O, Evert M, Rapp L, Lippert H, Meyer F
Universitätsklinikum Klinik für Allgemein-, Viszeralund Gefässchirurgie, Leipziger Strasse44, 39120 Magdeburg.
Zentralbl Chir. 2010 Feb;135(1):34-43. doi: 10.1055/s-0028-1098794. Epub 2009 Nov 11.
Based on a representative selection of relevant references, the aim of this study was to reflect the change of the algorithm in the surgical management of desmoid tumours (DT) in cases of accompanying familial adenomatous polyposis (FAP). Main focus is concerned with the basics of differential treatment, including additional considerations on epidemiology, diagnosis, outcome and follow-up. DT are rare benign tumours that do not metastasise but tend to invade locally. In contrast to the general population, DT in patients with FAP are more common, show a different pattern of tumour sites and cause considerable morbidity and mortality. Most DT occur in the abdominal cavity and account for the majority of serious problems. Genetic disposition and hormonal factors as well as prior surgical trauma are considered causative for the development of DT. Characteristic symptoms are abdominal pain, nausea and vomiting but DT may also present as acute abdomen. CT scan determines localisation and extension of the tumour. Treatment includes various strategies of medication, surgical resection and radiation. Data concerning diagnostic and therapeutic procedures are based on studies with small case series or case reports only. Therefore data from international multicentre studies are necessary for improving the prognosis and developing reliable and stringent guidelines.
基于对相关参考文献的代表性选取，本研究旨在反映在伴有家族性腺瘤性息肉病（FAP）的病例中，韧带样型纤维瘤病（DT）外科治疗算法的变化。主要关注点在于差异化治疗的基础，包括对流行病学、诊断、结果及随访的额外考量。DT是罕见的良性肿瘤，不会发生转移，但倾向于局部侵袭。与普通人群相比，FAP患者中的DT更为常见，呈现出不同的肿瘤部位模式，并导致相当高的发病率和死亡率。大多数DT发生在腹腔，是严重问题的主要原因。遗传易感性、激素因素以及既往手术创伤被认为是DT发生的病因。特征性症状为腹痛、恶心和呕吐，但DT也可能表现为急腹症。CT扫描可确定肿瘤的定位和范围。治疗包括多种药物治疗、手术切除和放疗策略。关于诊断和治疗程序的数据仅基于小病例系列研究或病例报告。因此，国际多中心研究的数据对于改善预后以及制定可靠且严格的指南是必要的。
