Principles and Management of Adrenal Cancer
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Adrenals | CURRENT Diagnosis & Treatment: Surgery, 14e | AccessSurgery | McGraw-Hill Medical
Cancer of the endocrine system. Abeloff's Clinical Oncology. Philadelphia, PA: Elsevier Saunders; chap The adrenal cortex. Williams Textbook of Endocrinology. Philadelphia, PA: Elsevier; chap Editorial team. Cushing syndrome due to adrenal tumor. Adrenal tumors are rare. They can be noncancerous benign or cancerous malignant. Noncancerous tumors that can cause Cushing syndrome include: Adrenal adenomas, a common tumor that rarely makes excess cortisol Macronodular hyperplasia , which causes the adrenal glands to enlarge and make excess cortisol Cancerous tumors that may cause Cushing syndrome include an adrenal carcinoma.
Exams and Tests. The health care provider will perform a physical exam and ask about your symptoms. The B-Cell Lymphoma Moon Shot is revolutionizing the conventional medical research approach to rapidly translate findings into patient treatment options and develop personalized therapeutic strategies. Adrenal tumors can be malignant cancer or benign not cancerous. Even benign adrenal tumors can be dangerous or cause uncomfortable symptoms. The adrenal glands are part of the endocrine system, which releases hormones into the blood system.
Hormones are important in many body processes, including metabolism, sexual development and puberty, and stress. There are two adrenal glands, one on the top of each kidney. Each gland has two parts. The medulla is the inner part of the adrenal gland. It makes hormones called catecholamines, which include adrenaline and noradrenaline. They also affect heart rate, blood pressure and sweating. The outer part of the adrenal gland is called the cortex.
It makes hormones that impact blood pressure, metabolism and how the body uses fats, carbohydrates and proteins. There are two main types of adrenocortical cancer:. It is extremely rare, with only about cases are diagnosed each year in the United States. About two to four new cases per 1 million people are diagnosed in this country each year.
Hyperaldosteronism: This condition is caused by a small tumor in the adrenal gland that makes too much aldosterone or an enlargement hyperplasia of the adrenal glands. Not everyone with these syndromes develops an adrenal tumor. However, if you or anyone in your family has one of these syndromes, your doctor may recommend genetic testing. Adrenal tumors may or may not cause symptoms, which depend on the type of hormone made by the tumor. If you have an adrenal tumor, your symptoms may include:. These symptoms do not always mean you have an adrenal tumor.
However, it is important to discuss any symptoms with your doctor, since they may signal other health problems. Adrenal tumors are diverse and can be challenging to diagnose. It is essential that an endocrinologist familiar with adrenal tumor recommends which tests you should have and analyzes your test results. They use the latest techniques and technology to give you the most accurate and concise diagnosis possible, including radiographic scanning, adreno-venous sampling and biochemical testing. Comprehensive genetic testing and counseling are available if your family members have certain inherited disorders.
If you have symptoms that might signal an adrenal tumor, your doctor will examine you and ask you questions about your health and your medical history. One or more of the following tests may be used to find out if you have an adrenal gland tumor or if treatment is working. The diagnostic work-up differs between ACC and pheochromocytoma. Guided biopsies of potentially resectable primary adrenal tumors are not informative in most cases, but are potentially harmful and should be avoided. Histological diagnosis should be done by an experienced pathologist and should rely on morphological, mitotic, and immunohistochemical parameters.
Complete surgical extirpation of localized and locally advanced ACC or pheochromocytoma R0 resection is the mainstay of potentially curative approaches. Additionally, a locoregional lymphadenectomy is suggested for ACC. In pheochromocytoma cytoreductive surgery might be considered. In advanced ACC, this approach is only reasonable for patients with severe hormone excess. R1 and Rx ACC resections may be followed by additional adjuvant radiotherapy to the tumor bed. Fit patients with inoperable ACC, high tumor volume and rapid disease progression should be treated with combination cytotoxic chemotherapy plus mitotane EDP-M.
Wait and see policy is recommended in low tumor burden and asymptomatic malignant pheochromocytoma and paraganglioma. Patients with resected ACC or pheochromocytoma should be followed at regular intervals with clinical, imaging and biochemical screens for at least 10 years. Berruti has reported: advisory board honorarium from Astellas. Oxford University Press is a department of the University of Oxford. It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide.
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Close mobile search navigation Article Navigation. Volume Article Contents. Oxford Academic. Google Scholar. Cite Citation. Permissions Icon Permissions. Open in new tab. Thyroid hormone replacement is recommended in patients with clinical symptoms of hypothyroidism Testosterone, free testosterone, and sexual hormone binding globulin SHBG should be tested in male patients with symptoms of hypogonadism Renin every 3 months.
If renin increases in the presence of symptoms suggestive of mineralocorticoid deficiency, fludrocortisones should be added Cholesterol High-Density Lipoprotein HDL , Low-Density Lipoprotein LDL , triglycerides every 3—4 months in an adjuvant setting.
Open in new tab Download slide. Search ADS. International symposium on pheochromocytoma. Pheochromocytoma: recommendations for clinical practice from the first international symposium.
October Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Urine steroid metabolomics as a biomarker tool for detecting malignancy in adrenal tumors.
Superiority of fluorodeoxyglucose positron emission tomography to other functional imaging techniques in the evaluation of metastatic SDHB-associated pheochromocytoma and paraganglioma. High diagnostic and prognostic value of steroidogenic factor-1 expression in adrenal tumors. Pathologic features of prognostic significance in adrenocortical carcinoma. Therapeutic management of advanced adrenocortical carcinoma: what do we know in ? Gene expression profiling reveals a new classification of adrenocortical tumors and identifies molecular predictors of malignancy and survival.
Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. Succinate dehydrogenase B gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas.
Retrospective evaluation of the outcome of open versus laparoscopic adrenalectomy for stage I and II adrenocortical cancer. Adrenocortical carcinomas: surgical trends and results of a patient series from the French Association of Endocrine Surgeons study group. Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma. Management of patients with adrenal cancer: recommendations of an international consensus conference. Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma.
Adjuvant therapy in patients with adrenocortical carcinoma: a position of an international panel. Optimal treatment of adrenocortical carcinoma with mitotane: results in a consecutive series of 96 patients. Phase II trial of mitotane and cisplatin in patients with adrenal carcinoma: a Southwest Oncology Group study. Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: insights from the largest single-institutional experience. Drug interactions with mitotane by induction of CYP3A4 metabolism in the clinical management of adrenocortical carcinoma.
Phase II study of high-dose [I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma.
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