Adrenocortical cancer (ACC) - literature overview and own experience
Adrenocortical cancer (ACC) - literature overview and own experience
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DOI：
10.5603/ep.2014.0069
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发表时间：
2014-01-01
影响因子：
2.1
通讯作者：
Sworczak, Krzysztof
中科院分区：
文献类型：
--
作者：
Dworakowska, Dorota;Drabarek, Agata;Sworczak, Krzysztof
肾上腺皮质癌是一种恶性内分泌肿瘤。这种疾病的罕见性阻碍了治疗的发展。年龄分布显示出两个高峰：生命的第一个和第五个十年，儿童和妇女更经常受到影响。虽然60-70%的ACC在生物化学上被发现过度产生激素，但在许多情况下临床上并不明显。如果存在，内分泌症状包括高皮质醇血症、男性化或男性乳房发育的体征。ACC预后不良，只有通过完全手术切除才能治愈。米托坦既可用作辅助治疗，也可用于非手术患者。放疗和化疗的作用仍有争议。由于肿瘤复发率高，术后无病生存率低，在30%左右波动。诊断基于肿瘤组织学评估，使用韦斯评分，然而尿类固醇分析（如果可用）可用于区分ACC和其他肾上腺肿瘤。ACC的常规预后指标包括疾病的分期和分级，以及目前报道的高皮质醇血症的存在。分子分析对理解ACC发展的发病机制和评估预后和预测标志物产生了重大影响，其中IGF系统、Wnt通路、p53和参与癌细胞侵袭特性和血管生成的分子的改变似乎非常有希望。我们在这里总结了我们自己的经验，ACC的管理，并提出了一个文献综述。我们并不打算包括ACC中描述的分子改变生物学的详细总结，因为这已经在其他论文中讨论过了。
Adrenocortical carcinoma (ACC) is a malignant endocrine tumour. The rarity of the disease has stymied therapeutic development. Age distribution shows two peaks: the first and fifth decades of life, with children and women more frequently affected. Although 60-70% of ACCs are biochemically found to overproduce hormones, it is not clinically apparent in many cases. If present, endocrine symptoms include signs of hypercortisolaemia, virilisation or gynaecomastia. ACC carries a poor prognosis, and a cure can be achieved only by complete surgical resection. Mitotane is used both as an adjuvant treatment and also in non-operative patients. The role of radio-and chemotherapy is still controversial. The post-operative disease free survival is low and oscillates around 30% due to high tumour recurrence rate. The diagnosis is based on tumour histological assessment with the use of the Weiss score, however urinary steroid profiling (if available) can serve to differentiate between ACC and other adrenal tumours. Conventional prognostic markers in ACC include stage and grade of disease, and, as currently reported, the presence of hypercortisolaemia. Molecular analysis has had a significant impact on the understanding of the pathogenetic mechanism of ACC development and the evaluation of prognostic and predictive markers, among which alterations of the IGF system, the Wnt pathway, p53 and molecules involved in cancer cell invasion properties and angiogenesis seem to be very promising. We here summarise our own experience related to the management of ACC and present a literature overview. We have not aimed to include a detailed summary of the molecular alterations biology described in ACC, as this has already been addressed in other papers.