Division of Endocrinology, Metabolism and Diabetes, Department of Medicine, University of Colorado School of Medicine at Colorado Anschutz Medical Campus, Aurora, Colorado.
Division of Endocrinology, Mayo Clinic, Rochester, Minnesota.
Endocr Pract. 2020 Nov;26(11):1366-1383. doi: 10.4158/DSCR-2020-0567. Epub 2020 Dec 14.
The aim of this Disease State Clinical Review is to provide a practical approach to patients with newly diagnosed adrenocortical carcinoma, as well as to follow-up and management of patients with persistent or recurrent disease.
This is a case-based clinical review. The provided recommendations are based on evidence available from randomized prospective clinical studies, cohort studies, cross-sectional and case-based studies, and expert opinions.
Adrenocortical carcinoma is a rare malignancy, often with poor outcomes. For any patient with an adrenal mass suspicious for adrenocortical carcinoma, the approach should include prompt evaluation with detailed history and physical exam, imaging, and biochemical adrenal hormone assessment. In addition to adrenal-focused imaging, patients should be evaluated with chest-abdomen-pelvis cross-sectional imaging to define the initial therapy plan. Patients with potentially resectable disease limited to the adrenal gland should undergo en bloc open surgery by an expert surgeon. For patients presenting with advanced or recurrent disease, a multidisciplinary approach considering curative repeat surgery, local control with surgery, radiation therapy or radiofrequency ablation, or systemic therapy with mitotane and/or cytotoxic chemotherapy is recommended.
As most health care providers will rarely encounter a patient with adrenocortical carcinoma, we recommend that patients with suspected adrenocortical carcinoma be evaluated by an expert multidisciplinary team which includes clinicians with expertise in adrenal tumors, including endocrinologists, oncologists, surgeons, radiation oncologists, pathologists, geneticists, and radiologists. We recommend that patients in remote locations be followed by the local health care provider in collaboration with a multidisciplinary team at an expert adrenal tumor program.
ACC = adrenocortical carcinoma; ACTH = adrenocorticotropic hormone; BRACC = borderline resectable adrenocortical carcinoma; CT = computed tomography; DHEAS = dehydroepiandrosterone sulfate; EDP = etoposide, doxorubicin, cisplatin; FDG = F-fluorodeoxyglucose; FNA = fine-needle aspiration; HU = Hounsfield units; IVC = inferior vena cava; LFS = Li-Fraumeni syndrome; MEN1 = multiple endocrine neoplasia type 1; MRI = magnetic resonance imaging; OAC = oncocytic adrenocortical carcinoma; PC = palliative care; PET = positron emission tomography.
本疾病临床综述旨在为新诊断的肾上腺皮质癌患者提供实用的治疗方法，以及为持续性或复发性疾病患者提供随访和管理建议。
这是一项基于病例的临床综述。提供的建议是基于随机前瞻性临床研究、队列研究、横断面研究和病例研究以及专家意见的现有证据得出的。
肾上腺皮质癌是一种罕见的恶性肿瘤，预后通常较差。对于任何有疑似肾上腺皮质癌的肾上腺肿块的患者，应包括详细的病史和体格检查、影像学和生化肾上腺激素评估。除了肾上腺聚焦成像外，患者还应进行胸部-腹部-骨盆横断面成像，以制定初始治疗计划。对于局限于肾上腺且有潜在可切除性的患者，应由专家外科医生进行整块开放式手术。对于有进展或复发性疾病的患者，建议采用多学科方法考虑再次手术治愈、手术、放疗或射频消融的局部控制、以及米托坦和/或细胞毒性化疗的全身治疗。
由于大多数医疗保健提供者很少遇到肾上腺皮质癌患者，我们建议疑似肾上腺皮质癌的患者由包括肾上腺肿瘤专家（内分泌学家、肿瘤学家、外科医生、放射肿瘤学家、病理学家、遗传学家和放射科医生）在内的多学科专家团队进行评估。我们建议位于偏远地区的患者由当地医疗保健提供者与专家肾上腺肿瘤项目的多学科团队合作进行随访。
ACC = 肾上腺皮质癌；ACTH = 促肾上腺皮质激素；BRACC = 交界性可切除肾上腺皮质癌；CT = 计算机断层扫描；DHEAS = 脱氢表雄酮硫酸酯；EDP = 依托泊苷、多柔比星、顺铂；FDG = F-氟脱氧葡萄糖；FNA = 细针抽吸；HU = Hounsfield 单位；IVC = 下腔静脉；LFS = Li-Fraumeni 综合征；MEN1 = 多发性内分泌肿瘤 1 型；MRI = 磁共振成像；OAC = 嗜酸细胞性肾上腺皮质癌；PC = 姑息治疗；PET = 正电子发射断层扫描。
