Department of General and Thoracic Surgery, National Institute of Oncology, Budapest, Hungary.
Med Sci Monit. 2012 Mar;18(3):CS17-25. doi: 10.12659/msm.882506.
Primary intra-thoracic desmoids are exceedingly rare borderline tumors, with 34 reported cases in the English-language literature. The characteristic localized infiltrative growth and the high rate of recurrence can result in life-threatening conditions. Radical surgical resection is considered to be the primary treatment. Achieving negative surgical margins is a challenge. Cases with positive surgical margins are associated with a high rate of local recurrence; therefore, other multimodal approaches play a large role in their therapy.
The authors reviewed the relevant literature and presented examples of long-term follow-up of 3 intra-thoracic desmoid tumour patients, multidisciplinarily treated between 2000 and 2008. All reports of intra-thoracic desmoid tumors that the authors could find on PubMed or in the reference sections of these PubMed located articles were included using the search terms: intra-thoracic, desmoid, aggressive fibromatoses.
Because of the rarity of the disease and the heterogeneity of the cases, it is difficult to assess the importance of the information for everyday clinical practice. It does however provide a useful guide for reference.
原发性胸内硬纤维瘤是一种极为罕见的交界性肿瘤，英文文献中有 34 例报道。其特征性的局限性浸润性生长和高复发率可导致危及生命的情况。根治性手术切除被认为是主要的治疗方法。达到阴性手术切缘是一个挑战。阳性手术切缘的病例与高局部复发率相关；因此，其他多模式方法在其治疗中起着重要作用。
作者回顾了相关文献，并介绍了 3 例胸内硬纤维瘤患者的长期随访情况，这些患者于 2000 年至 2008 年间接受了多学科治疗。作者使用以下检索词在 PubMed 或这些 PubMed 定位文章的参考文献部分搜索了所有能找到的胸内硬纤维瘤报告：胸内、硬纤维瘤、侵袭性纤维瘤病。
由于该病的罕见性和病例的异质性，难以评估这些信息对日常临床实践的重要性。但它确实为参考提供了有用的指南。
