Primary malignant tumors of the adrenal glands
Primary malignant tumors of the adrenal glands
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DOI：
10.6061/clinics/2018/e756s
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发表时间：
2018-01-01
期刊：
影响因子：
2.7
通讯作者：
Fragoso, Maria Candida B.V.
中科院分区：
文献类型：
--
作者：
Almeida, Madson Q.;Bezerra-Neto, Joao Evangelista;Fragoso, Maria Candida B.V.
恶性肿瘤必须考虑在管理肾上腺病变，包括那些偶然发现的影像学研究。肾上腺皮质癌（ACCs）是一种罕见的肿瘤，估计每年发病率为0.7-2例，全球患病率为每百万人/年4-12例。然而，这些肿瘤在巴西南部和东南部的发病率要高得多（415倍）。大多数acc引起类固醇的高分泌，包括糖皮质激素和雄激素。ACC患者预后非常差，大多数系列患者的5年总生存率（OS）低于30%。嗜铬细胞瘤或副神经节瘤（PPGL）是一种起源于肾上腺髓质的嗜铬细胞的代谢活性肿瘤。PPGL的发病率为每年每10万人0.2至0.9例。大约4-7%的肾上腺偶发瘤患者存在嗜铬细胞瘤。典型地，PPGL表现为以下4种症状的阵发性发作：头痛、出汗、心悸和严重高血压发作。恶性PPGL的诊断依赖于是否存在局部侵袭或转移。在这篇综述中，我们介绍了影响人类肾上腺皮质和髓质的恶性原发性病变的临床、生化特征和发病机制。
Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. However, a much higher incidence of these tumors (415 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.