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[肾上腺皮质癌]

[ADRENOCORTICAL CARCINOMA].

作者信息

Kranjčević K

出版信息

Acta Med Croatica. 2016 Dec;70(4-5):315-8.

PMID:29087170
Abstract

Adrenocortical carcinoma is a rare entity. However, it is the most common type of cancerous adrenal gland tumor with poor prognosis. Approximately 4 to 12 per 1 million people develop this type of tumor, which begins in the adrenal cortex. Adrenocortical carcinoma can occur at any age. Women tend to be diagnosed slightly more often than men. In most cases, the cause of cancerous adrenal gland tumor remains unknown. However, people with certain hereditary conditions have a higher risk. Adrenocortical carcinoma can be a functional or nonfunctional tumor. If the tumor is functional, it may produce hormones causing symptoms such as high blood pressure, low potassium level, heart palpitations, nervousness, feelings of anxiety or panic attacks, excessive perspiration, diabetes, Cushing syndrome, unexplained weight gain or weight loss, weakness, abdominal stretch marks, excessive hair growth, changes in genitalia, change in libido, etc. If the tumor is nonfunctional, symptoms occur because the tumor has grown so large that it exerts pressure on the nearby organs, causing abdominal pain or a feeling of fullness. To diagnose adrenocortical carcinoma, in addition to thorough physical examination, the following tests are used: blood and urine tests to help determine whether the tumor is functional or nonfunctional, and imaging tests (computed tomography scan or magnetic resonance imaging). The treatment depends on cancer stage. Two major staging systems are used: the American Joint Committee on Cancer (AJCC) TNM staging system and the ENSAT (European Network for the Study of Adrenal Tumors) staging system. Both are based on the same TNM categories. The main types of treatment for adrenal cancer are surgery (the main treatment), chemotherapy and targeted therapy. Radiation therapy is not used often as the main initial treatment for adrenal cancer because the cancer cells are not easy to kill with x-rays. Radiation may be used as adjuvant therapy. By definition, adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctional adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g., adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma, or metastasis).

摘要

肾上腺皮质癌是一种罕见的疾病。然而,它是肾上腺肿瘤中最常见的癌症类型,预后较差。每100万人中约有4至12人会患上这种起源于肾上腺皮质的肿瘤。肾上腺皮质癌可发生于任何年龄。女性被诊断出该病的几率略高于男性。在大多数情况下,肾上腺癌性肿瘤的病因尚不清楚。然而,患有某些遗传性疾病的人风险更高。肾上腺皮质癌可以是功能性肿瘤或非功能性肿瘤。如果肿瘤是功能性的,它可能会产生激素,导致诸如高血压、低钾水平、心悸、紧张、焦虑感或惊恐发作、多汗、糖尿病、库欣综合征、不明原因的体重增加或减轻、虚弱、腹部妊娠纹、毛发过度生长、生殖器变化、性欲改变等症状。如果肿瘤是非功能性的,症状的出现是因为肿瘤长得太大,对附近器官施加压力,导致腹痛或饱腹感。为了诊断肾上腺皮质癌,除了进行全面的体格检查外,还会使用以下检查:血液和尿液检查,以帮助确定肿瘤是功能性还是非功能性的,以及影像学检查(计算机断层扫描或磁共振成像)。治疗方法取决于癌症分期。使用两种主要的分期系统:美国癌症联合委员会(AJCC)TNM分期系统和ENSAT(欧洲肾上腺肿瘤研究网络)分期系统。两者都基于相同的TNM分类。肾上腺癌的主要治疗类型是手术(主要治疗方法)、化疗和靶向治疗。放射治疗通常不作为肾上腺癌的主要初始治疗方法,因为癌细胞不容易被X射线杀死。放射治疗可作为辅助治疗。根据定义,肾上腺偶发瘤是在并非因疑似肾上腺疾病而进行的影像学检查中发现的无症状肾上腺肿块。在大多数情况下,肾上腺偶发瘤是非功能性肾上腺皮质腺瘤,但也可能代表需要治疗干预的情况(例如,肾上腺皮质癌、嗜铬细胞瘤、产生激素的腺瘤或转移瘤)。

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