Neupane Durga, Khadka Sarada, Upadhyaya Paricha, Sah Suresh P, Dulal Soniya, Shah Siddhartha K, Shah Ujjwal K
Departments of Surgery.
Surgery (Breast, Endocrine and General Surgery).
Ann Med Surg (Lond). 2023 Mar 27;85(5):1834-1838. doi: 10.1097/MS9.0000000000000097. eCollection 2023 May.
Adrenal tumors are very common, affecting 3-10% of the human population, and most are small, benign, nonfunctional adrenocortical adenomas. Adrenocortical carcinoma (ACC), in contrast, is a very rare disease. The median age of diagnosis is in the fifth to sixth decade. There is a predilection for the female gender (the ratio of female to male ranges from 1.5 to 2.5 : 1) the adult.
A 28-year-old man who had no prior history of systemic hypertension or diabetes mellitus presented with bilateral limb swelling for 2 months and facial puffiness for 1 month. He had an episode of hypertensive emergencies. A radiological and hormonal work-up established the diagnosis of primary ACC. One cycle of chemotherapy was given until he lost follow-up and succumbed to death due to financial constraints.
Adrenocortical carcinoma is an extremely uncommon tumor of the adrenal gland, and it is even more uncommon when it manifests without any symptoms. If patients exhibit signs of rapid and multiple adrenocortical hormone excess, such as weakness, hypokalaemia, or hypertension, ACC may be suspected. Recently developed gynecomastia in men may be brought on by an ACC producing too much sex hormone. To accurately diagnose the condition and give the patient a fair prognosis, a multidisciplinary approach involving endocrine surgeons, oncologists, radiologists, and internists is advised. Proper genetic counseling is recommended. It is critical to know whether the adrenal mass is malignant or not, and to get this ascertained by a computed tomography finding and biopsy.
肾上腺肿瘤非常常见，影响3%至10%的人群，且大多数是小的、良性的、无功能的肾上腺皮质腺瘤。相比之下，肾上腺皮质癌（ACC）是一种非常罕见的疾病。诊断的中位年龄在五到六十岁。成年女性更易患病（女性与男性的比例为1.5至2.5:1）。
一名28岁男性，既往无系统性高血压或糖尿病病史，出现双侧肢体肿胀2个月，面部浮肿1个月。他曾发生过高血压急症。经影像学和激素检查确诊为原发性肾上腺皮质癌。给予一个周期的化疗，直到他失去随访，因经济困难死亡。
肾上腺皮质癌是一种极其罕见的肾上腺肿瘤，无症状表现时更为罕见。如果患者出现快速和多种肾上腺皮质激素过多的迹象，如虚弱、低钾血症或高血压，则可能怀疑为肾上腺皮质癌。男性近期出现的男性乳房发育可能是由分泌过多性激素的肾上腺皮质癌引起的。为准确诊断病情并给患者一个合理的预后，建议采用内分泌外科医生、肿瘤学家、放射科医生和内科医生参与的多学科方法。建议进行适当的遗传咨询。关键是要知道肾上腺肿块是否为恶性，并通过计算机断层扫描结果和活检来确定。
