Desmoid Tumors: Clinical Features and Treatment Options for Advanced Disease
Desmoid Tumors: Clinical Features and Treatment Options for Advanced Disease
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DOI：
10.1634/theoncologist.2010-0281
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发表时间：
2011-01-01
期刊：
影响因子：
5.8
通讯作者：
Hohenberger, Peter
中科院分区：
文献类型：
--
作者：
Kasper, Bernd;Stroebel, Philipp;Hohenberger, Peter
硬纤维瘤描述了一种罕见的单克隆，成纤维细胞增殖的特点是一个变量，往往不可预测的临床过程。虽然组织学上是良性的，但硬纤维瘤是局部浸润性的，并与高局部复发率相关，但缺乏转移潜力。在分子水平上，韧带样瘤的特征在于β-连环蛋白基因CTNNB 1或腺瘤性结肠息肉病基因APC的突变。CTNNB 1突变的证据可能是有用的，当病理鉴别诊断是困难的，位置可能是预测疾病reproduction.Many问题关于硬纤维瘤患者的最佳治疗仍然存在争议;然而，手术是治疗的主要手段，除非mutilating和相关的相当大的功能丧失。术后放疗可降低手术切缘受累病例的局部复发率。由于韧带样瘤生物学行为的异质性，包括长时间的疾病稳定甚至自发消退，治疗需要个体化，以优化局部肿瘤控制并保持患者的生活质量。因此，应用多学科评估与多模式治疗形成了对这些患者的护理基础。对于选定的无症状患者，观察等待可能是最合适的管理方法。位于肠系膜或头颈部的韧带样瘤患者可能出现危及生命的并发症，通常需要更积极的治疗。本文综述了硬纤维瘤患者的治疗选择和管理策略，重点是晚期疾病。肿瘤学家2011;16：682-693
Desmoid tumors describe a rare monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Although histologically benign, desmoids are locally invasive and associated with a high local recurrence rate, but lack metastatic potential. On the molecular level, desmoids are characterized by mutations in the beta-catenin gene, CTNNB1, or the adenomatous polyposis coli gene, APC. Proof of a CTNNB1 mutation may be useful when the pathological differential diagnosis is difficult and location might be predictive for disease recurrence.Many issues regarding the optimal treatment of patients with desmoids remain controversial; however, surgery is the therapeutic mainstay, except if mutilating and associated with considerable function loss. Postoperative radiotherapy reduces the local recurrence rate, in cases of involved surgical margins. Because of the heterogeneity of the biological behavior of desmoids, including long periods of stable disease or even spontaneous regression, treatment needs to be individualized to optimize local tumor control and preserve patients' quality of life. Therefore, the application of a multidisciplinary assessment with multimodality treatment forms the basis of care for these patients. Watchful waiting may be the most appropriate management in selected asymptomatic patients. Patients with desmoids located at the mesentery or in the head and neck region could present with life-threatening complications and often need more aggressive treatment. This review describes treatment options and management strategies for patients with desmoid tumors with a focus on advanced disease. The Oncologist 2011;16:682-693