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Precursor Lesions of the Adrenal Gland

机译:肾上腺前体病变

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Objective: To review the existing literature for evidence that adrenocortical and adrenomedullary tumours develop through a multistep process of carcinogenesis. Results: In the adrenal cortex hyperplasia and adenomas are frequently observed tumours or tumour-like conditions. In contrast, adrenocortical carcinomas are rare. Based on well-validated histopathological scoring systems, benign and malignant adrenocortical tumours can be separated, although a small subset of tumours remains hard to classify. Although extensive follow-up studies might argue against multistep carcinogenesis, analysis of chromosomal imbalances and gene expression profiling studies in these tumours are inconclusive and could give support for both multistep pathogenesis or de novo genesis of carcinomas. A major limit to most of these studies is the small sample size and the lack of extensive clinical (follow-up) data. In the adrenal medulla, pheo-chromocytomas (PCC) are the most frequent tumours in adults, with an incidence of 8 per million. They can be divided into benign and malignant PCC, but the distinction can only be made when metastases are present. Arbitrarily, lesions of less than 1 cm in diameter are called hyperplastic, but it should be expected that the majority of these are early lesions and if left in situ would grow to classify as PCC. In contrast to cortical tumours, the frequent 1p and 3q loss as an early event in tumourigenesis of benign PCC is verified in multiple studies. However, studies in malignant PCC yield divergent results, due to the small numbers analysed. Conclusion: Taken together, there appears to be a relationship between cortical and medullary hyperplasia on the one hand and cortical adenomas and PCC on the other. However, whether there is a transition from benign to malignant tumours, both cortical and medullary, remains to be determined.
机译:目的:回顾现有文献,以证明肾上腺皮质和肾上腺髓质肿瘤是通过多步致癌过程发展而来的。结果:在肾上腺皮质增生和腺瘤中常观察到肿瘤或肿瘤样情况。相反,肾上腺皮质癌是罕见的。基于经过良好验证的组织病理学评分系统,可以区分良性和恶性肾上腺皮质肿瘤,尽管一小部分肿瘤仍然难以分类。尽管广泛的随访研究可能反对多步癌变,但这些肿瘤中染色体失衡的分析和基因表达谱研究尚无定论,可以为癌症的多步发病机理或从头开始提供支持。这些研究大多数的主要局限性是样本量小且缺乏广泛的临床(随访)数据。在肾上腺髓质中,嗜铬细胞瘤(PCC)是成人中最常见的肿瘤,发病率为百万分之八。它们可以分为良性和恶性PCC,但只有在存在转移时才能进行区分。直径小于1 cm的病变被任意称为增生性病变,但是应该预期其中大多数是早期病变,如果留在原位,将会发展为PCC分类。与皮质肿瘤相比,良性PCC肿瘤发生中频繁发生的1p和3q丢失是早期事件,已在多项研究中得到证实。但是,由于分析的数量少,因此对恶性PCC的研究产生了分歧的结果。结论:两者合计,似乎一方面是皮质和髓样增生,另一方面是皮质腺瘤和PCC。然而,是否存在从良性到恶性肿瘤的转变,无论是皮质还是髓样,都有待确定。

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