Sex Cord-Gonadal Stromal Tumors

性索性腺间质瘤
  • 文章类型: Letter
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  • 文章类型: Journal Article
    目的:我们制定了第一个基于德国证据和共识的诊断临床指南,治疗,和成年患者睾丸生殖细胞肿瘤(GCT)的随访。我们在两个单独的出版物中介绍了指南内容。第二部分总结了对晚期疾病阶段的治疗以及随访和后期效果的管理的建议。
    方法:由包括1名患者代表在内的42名专家组成的跨学科小组制定了指南内容。临床建议和陈述基于科学证据和专家共识。为此,几个复习问题的证据表,这是基于系统的文献检索(最后一次检索是在2018年3月),提供了。31位专家,有权投票的人,对最终临床建议和声明进行评级。
    结果:在这里,我们分别提出转移性精原细胞瘤和非精原细胞瘤GCT(IIA/B和IIC/III期)患者的治疗建议。用于残余肿块的重新分类和治疗,以及复发和难治性疾病阶段。这些建议还涵盖性腺外和性索/基质肿瘤,随访和毒性的管理,生活质量方面,姑息治疗,和支持性治疗。
    结论:参与诊断的医师和其他医疗服务提供者,治疗,以及GCT的随访(所有阶段,门诊和住院护理以及康复)是本指南的使用者。该指南还包括质量指标,用于衡量常规临床护理中指南建议的实施情况;这些数据将在未来的出版物中提供。
    OBJECTIVE: We developed the first German evidence- and consensus-based clinical guideline on diagnosis, treatment, and follow-up of germ cell tumours (GCT) of the testes in adult patients. We present the guideline content in 2 separate publications. The present second part summarizes therecommendations for the treatment of advanced disease stages and for the management of follow-up and late effects.
    METHODS: An interdisciplinary panel of 42 experts including 1 patient representative developed the guideline content. Clinical recommendations and statements were based on scientific evidence and expert consensus. For this purpose, evidence tables for several review questions, which were based on systematic literature searches (last search in March 2018), were provided. Thirty-one experts, who were entitled to vote, rated the final clinical recommendations and statements.
    RESULTS: Here we present the treatment recommendations separately for patients with metastatic seminoma and non-seminomatous GCT (stages IIA/B and IIC/III), for restaging and treatment of residual masses, and for relapsed and refractory disease stages. The recommendations also cover extragonadal and sex cord/stromal tumours, the management of follow-up and toxicity, quality-of-life aspects, palliative care, and supportive therapy.
    CONCLUSIONS: Physicians and other medical service providers who are involved in the diagnostics, treatment, and follow-up of GCT (all stages, outpatient and inpatient care as well as rehabilitation) are the users of the present guideline. The guideline also comprises quality indicators for measuring the implementation of the guideline recommendations in routine clinical care; these data will be presented in a future publication.
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  • 文章类型: Journal Article
    性索间质肿瘤(SCST)是成人卵巢区域的罕见癌症。它们构成了一组异质性肿瘤,从性索和卵巢基质发展而来。这些肿瘤通常在早期被发现,它们可能会在最初治疗后30年内复发。因为70%的患者出现I期肿瘤,手术是最重要的治疗手段。没有数据支持IA或IB期SCST患者的任何类型的术后辅助治疗,鉴于这些肿瘤的惰性性质和总体预后良好。如果复发,该疾病的长期自然史可能导致重复的外科手术。基于铂的化疗目前用于晚期SCSTs或复发性疾病的患者,总反应率为63%至80%。SCSTs的惰性性质具有晚期复发的趋势,需要长期随访。
    Sex cord stromal tumors (SCST) are rare cancers of the ovarian area in adults. They constitute a heterogeneous group of tumors that develop from the sex cords and the ovarian stroma. These tumors are detected typically at an early stage, and they may recur as late as 30 years after the initial treatment. Because 70% of the patients present with stage I tumors, surgery represents the most important therapeutic arm. There are no data to support any kind of postoperative adjuvant treatment for patients with stage IA or IB SCSTs, given the indolent nature of these neoplasms and the overall good prognosis. The long natural history of the disease may lead to repeated surgical procedure should a relapse occurs. Platinum-based chemotherapy is currently used for patients with advanced stage SCSTs or recurrent disease, with an overall response rate of 63% to 80%. The indolent nature of SCSTs with the tendency for late recurrence requires long-term follow-up.
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  • 文章类型: Consensus Development Conference
    背景:代表欧洲泌尿外科协会(EAU),诊断指南,治疗,并建立了睾丸癌的随访。
    目的:本文是EAU睾丸癌指南的简短版本,总结了有关睾丸癌治疗指南的主要结论。
    方法:指南由一个多学科指南工作组编制。使用Medline和Embase进行了系统评价,还考虑了Cochrane证据和欧洲生殖细胞癌症共识小组的数据。一个专家小组对参考文献进行了加权,并分配了证据水平和推荐等级.
    结果:文献很少,特别是关于长期随访的文献,目前还在等待一些正在进行的试验的结果.治疗中心的选择至关重要,和临床试验中参考中心的治疗,尤其是预后不良的非精原细胞生殖细胞肿瘤,提供更好的结果。对于临床I期精原细胞瘤的患者,根据最近公布的长期毒性数据,辅助放疗不再推荐作为一线辅助治疗.建议使用2009年TNM分类。
    结论:这些指南包含了基于最新科学见解的睾丸癌患者标准化管理信息。治愈率一般都很好,但是因为睾丸癌主要影响男性的第三或第四个十年,对生育能力的治疗效果需要对患者进行仔细的咨询,治疗必须根据个人情况和患者偏好进行调整。
    结论:虽然睾丸癌有很好的治愈率,治疗中心的选择至关重要。专家中心在早期睾丸癌(较低的复发率)和总体生存率(临床试验中的较高阶段)方面都取得了更好的结果。对于临床I期精原细胞瘤的患者,辅助放疗不再推荐作为一线辅助治疗.
    BACKGROUND: On behalf of the European Association of Urology (EAU), guidelines for the diagnosis, therapy, and follow-up of testicular cancer were established.
    OBJECTIVE: This article is a short version of the EAU testicular cancer guidelines and summarises the main conclusions from the guidelines on the management of testicular cancer.
    METHODS: Guidelines were compiled by a multidisciplinary guidelines working group. A systematic review was carried out using Medline and Embase, also taking Cochrane evidence and data from the European Germ Cell Cancer Consensus Group into consideration. A panel of experts weighted the references, and a level of evidence and grade of recommendation were assigned.
    RESULTS: There is a paucity of literature especially regarding longer term follow-up, and results from a number of ongoing trials are awaited. The choice of treatment centre is of the utmost importance, and treatment in reference centres within clinical trials, especially for poor-prognosis nonseminomatous germ cell tumours, provides better outcomes. For patients with clinical stage I seminoma, based on recently published data on long-term toxicity, adjuvant radiotherapy is no longer recommended as first-line adjuvant treatment. The TNM classification 2009 is recommended.
    CONCLUSIONS: These guidelines contain information for the standardised management of patients with testicular cancer based on the latest scientific insights. Cure rates are generally excellent, but because testicular cancer mainly affects men in their third or fourth decade of life, treatment effects on fertility require careful counselling of patients, and treatment must be tailored taking individual circumstances and patient preferences into account.
    CONCLUSIONS: Although testicular cancer has excellent cure rates, the choice of treatment centre is of the utmost importance. Expert centres achieve better results for both early stage testicular cancer (lower relapse rates) and overall survival (higher stages within clinical trials). For patients with clinical stage I seminoma, adjuvant radiotherapy is no longer recommended as first-line adjuvant treatment.
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