Adrenocortical CarcinomaSymptoms, Doctors, Treatments, Advances & More
Learn About Adrenocortical Carcinoma
Adrenocortical carcinoma (ACC) is a cancer of the adrenal glands. The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney.
Tumor - adrenal; ACC - adrenal
ACC is most common in children younger than 5 years old and adults in their 40s and 50s.
The condition may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor.
ACC can produce the hormones cortisol, aldosterone, estrogen, or testosterone, as well as other hormones. In women the tumor often releases these hormones, which can lead to male characteristics.
ACC is very rare. The cause is unknown.
Symptoms of increased cortisol or other adrenal gland hormones may include:
- Fatty, rounded hump high on the back just below the neck (buffalo hump)
- Flushed, rounded face with pudgy cheeks (moon face)
- Obesity
- Stunted growth (short stature)
- Virilization -- the appearance of male characteristics, including increased body hair (especially on the face), pubic hair, acne, deepening of the voice, and enlarged clitoris (females)
Symptoms of increased aldosterone are the same as symptoms of low potassium, and include:
- Muscle cramps
- Weakness
- Pain in the abdomen
The primary treatment is surgery to remove the tumor. ACC may not improve with chemotherapy. Medicines may be given to reduce production of cortisol, which causes many of the symptoms.
Rogel Cancer Center
Gary D. Hammer, M.D., Ph.D. is Professor in Departments of InternalMedicine (MEND), Cell & Developmental Biology, and Molecular &Integrative Physiology. He currently serves as Director of EndocrineOncology Program in Rogel Cancer Center where he holds the MillieSchembechler Professorship in Adrenal Cancer. He is past Director ofCenter for Organogenesis: Stem Cells & Regenerative Medicine. He hasbrokered the recent renaissance of the current Michigan team of adrenalscientists that includes a who’s who in the study of adrenal disease.He received the UofM Jerome Conn Award for Outstanding Research inInternal Medicine, the Endocrine Society Edwin B. Astwood Award forOutstanding Research and is a member of ASCI and AAP. He has served innumerous leadership roles at UofM and in the public and private sectorsincluding recent tenure on Board of Scientific Advisors for NCI. He isthe editor of four textbooks including Pathophysiology of Disease: AnIntroduction to Clinical Medicine (McGraw Hilll), and the upcomingAdrenal Section Editor for Degroot’s Endocrinology (Elsevier). He hasserved in numerous capacities for the world’s largest endocrineorganization, the Endocrine Society including his most recent role asPresident of the Society (2020-2021). An Advisor to the CongressionalH.E.L.P. committee - he co-authored the 21st Century Cancer ALERT(Access to Life-Saving Early detection, Research and Treatment) Act’’ lintroduced in Congress in 2009. He has been catalyst for the formationof two international cooperative groups, most recently the A5 (AmericanAustralian Asian Adrenal Alliance) that leverages collaboration tosecure projects from Pharma and funding agencies across the spectrum ofadrenal diseases. He is a cofounder of two companies - MILLENDO andSLING THERAPEUTICS that focus on rare endocrine diseases. Dr. Hammer is rated as an Elite provider by MediFind in the treatment of Adrenocortical Carcinoma. He is also highly rated in 12 other conditions, according to our data. His clinical expertise encompasses Adrenal Cancer, Adrenocortical Carcinoma, Hereditary Neuroblastoma, and Neuroblastoma. Dr. Hammer is board certified in Endocrinology, Diabetes & Metabolism.
Rogel Cancer Center
Dr. Else’s primary appointment is with the Division of Metabolism, Endocrinology and Diabetes. He received his MD degree from the University of Hamburg, Germany. He pursued his clinical training at the University of Hamburg (Residency: Internal Medicine) and the University of Michigan (Residency: Internal Medicine, Fellowship: Endocrinology). His main research interests are the genetics of endocrine tumors. Dr. Else’s clinical practice focuses on the care for patients with benign and malignant endocrine tumors, particularly pheochromocytoma, adrenocortical tumors and neuroendocrine tumors as well as accompanying hormone excess syndromes (e.g Cushing syndrome, primary aldosteronism). Dr. Else has a particular interest in taking care of patients with hereditary syndromes that predispose to the development of endocrine tumors (e.g. Multiple Endocrine Neoplasia (MEN) type 1, MEN2A & MEN2B, Hereditary Paraganglioma Syndrome). He attends the Multidisciplinary Endocrine Oncology Clinic, where patients with tumors receive diagnostic procedures and treatment. In addition he is part of the Cancer Genetics Clinic, which evaluates patients for hereditary syndromes and also facilitates regular exams and surveillance for patients at risk for endocrine tumors. Dr. Else is rated as an Elite provider by MediFind in the treatment of Adrenocortical Carcinoma. He is also highly rated in 105 other conditions, according to our data. His clinical expertise encompasses Adrenal Cancer, Adrenocortical Carcinoma, Adrenal Gland Adenoma, Neuroendocrine Tumor, and Thyroidectomy. Dr. Else is board certified in Internal Medicine and Endocrinology, Diabetes & Metabolism.
University Of Colorado Hospital Authority
Margaret Wierman is an Endocrinologist practicing medicine in Aurora, Colorado. Dr. Wierman is rated as an Elite provider by MediFind in the treatment of Adrenocortical Carcinoma. She is also highly rated in 32 other conditions, according to our data. Her clinical expertise encompasses Adrenocortical Carcinoma, Pituitary Tumor, Adrenal Cancer, Hypothalamic Tumor, and Hormone Replacement Therapy (HRT).
The outcome depends on how early the diagnosis is made and whether the tumor has spread (metastasized). Tumors that have spread usually lead to death within 1 to 3 years.
The tumor can spread to the liver, bone, lung, or other areas.
Contact your provider if you or your child has symptoms of ACC, Cushing syndrome, or failure to grow.
Background: Adrenocortical carcinoma (ACC) is a rare cancer of the adrenal glands. ACC often returns after tumors are removed with surgery. Less than 35% of people with ACC survive 5 years after diagnosis.
Summary: NOTE: This is a research study and is not meant to be a substitute for clinical genetic testing. Families may never receive results from the study or may receive results many years from the time they enroll. If you are interested in clinical testing please consider seeing a local genetic counselor or other genetics professional. If you have already had clinical genetic testing and meet eligibility...
Published Date: August 21, 2024
Published By: Warren Brenner, MD, Oncologist, Lynn Cancer Institute, Boca Raton, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Habra M, Else T, Fassnacht M. Adrenocortical carcinoma. In: Robertson RP, ed. DeGroot's Endocrinology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 98.
National Cancer Institute website. Adrenocortical carcinoma treatment (PDQ) - health professional version. www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-pdq. Updated August 25, 2022. Accessed August 27, 2024.
Yeh MW, Livhits M, Duh Q-Y. The adrenal glands. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, et al, eds. Sabiston Textbook of Surgery. 21st ed. St Louis, MO: Elsevier; 2022:chap 40.