Small cell carcinoma of the ovary of the hypercalcaemic type: an analysis of clinical and prognostic aspects of a rare disease on the basis of cases published in the literature
Small cell carcinoma of the ovary of the hypercalcaemic type: an analysis of clinical and prognostic aspects of a rare disease on the basis of cases published in the literature
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DOI：
10.1007/s00404-011-1846-5
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发表时间：
2011-11-01
影响因子：
2.6
通讯作者：
Muenstedt, Karsten
中科院分区：
文献类型：
--
作者：
Estel, Rebecca;Hackethal, Andreas;Muenstedt, Karsten
高钙血症型卵巢小细胞癌是一种非常罕见且高度侵袭性的恶性疾病，主要影响年轻女性。由于这种肿瘤实体的罕见性，不太可能进行前瞻性随机试验，唯一基于大型病例系列的回顾性分析是1994年的。由于诊断和治疗方式可能发生了变化，我们开始了这项分析，我们的研究目的是回顾和分析自1975年以来发表的病例，以验证以前的研究结果，并收集更多的信息，治疗方案，诊断和预后因素。对PubMed/Medline数据库进行了系统性文献检索，评估了截至2010年9月的所有文章。对所有检索到的文章进行了评价，并交叉检查了关于该主题的参考文献。总共有135个病例被纳入，这些病例是从62个病例报告和较小的病例研究中选出的。他们表现出非特异性症状，如腹痛或可触及的肿块，有时伴有钙或CA-125血清浓度升高。肿瘤几乎是单侧出现的，主要累及右侧卵巢。肿瘤分期是一个明确的预后因素。由依托泊苷、顺铂/卡铂或长春花生物碱组成的辅助化疗已显示出改善的生存率，而放疗则没有。尽管存在局限性，但该分析提供了新的见解，特别是在治疗方面。这篇综述强调了罕见肿瘤实体病例报告的重要性，以回答开放性问题。
Ovarian small cell carcinoma of the hypercalcaemic type is a very rare and highly aggressive malignant disease, mainly affecting young women. Due to the rarity of this tumour entity, prospective randomised trials are unlikely to be conducted, and the only retrospective analysis based on a large case series is from 1994. Since diagnostic and treatment modalities may have changed, we initiated this analysis.The aim of our study was to review and analyse cases published since 1975 to validate former findings and to gather more information about therapy options, diagnostic and prognostic factors. A systematic literature search of the PubMed/Medline database was performed assessing all articles until September 2010. All retrieved articles were evaluated and cross-checked for references on the topic. In total, 135 cases were included, selected from 62 case reports and smaller case studies.Small cell carcinoma mostly affects women with a mean age of 23.4 years. They present with unspecific symptoms like abdominal pain or palpable mass, sometimes accompanied by an elevated calcium or CA-125 serum concentration. The tumour appears nearly almost unilaterally, mostly affecting the right ovary. Tumour stage is a clearly prognostic factor. Adjuvant chemotherapy consisting of etoposide, cisplatinum/carboplatinum or vinca alkaloids has shown improved survival, whereas radiotherapy has not.In spite of limitations this analysis provides new insights especially with respect to therapeutic aspects. This review underlines the importance of case reports in rare tumour entities in order to answer open questions.