Adrenocortical carcinoma

Adrenocortical carcinoma
Other namesAdrenal cortical carcinoma, adrenocorticocarcinoma, adrenal cortical cancer, adrenal cortex cancer
Micrograph of an adrenocortical carcinoma (left of image – dark blue) and the adrenal cortex it arose from (right-top of image – pink/light blue). Benign adrenal medulla is present (right-middle of image – gray/blue). H&E stain.
SpecialtyOncology

Adrenocortical carcinoma (ACC) is an aggressive cancer originating in the cortex (steroid hormone-producing tissue) of the adrenal gland.

Adrenocortical carcinoma is remarkable for the many hormonal syndromes that can occur in patients with steroid hormone-producing ("functional") tumors, including Cushing's syndrome, Conn syndrome, virilization, and feminization. Adrenocortical carcinoma has often invaded nearby tissues or metastasized to distant organs at the time of diagnosis, and the overall 5-year survival rate is about 50%.[1]

Adrenocortical carcinoma is a rare tumor, with incidence of one to two per million population annually.[2][3][4][5][6] It has a bimodal distribution by age, with cases clustering in children under 5 and in adults 30–40 years old.[5] The widely used angiotensin-II-responsive steroid-producing cell line H295R was originally isolated from a tumor diagnosed as adrenocortical carcinoma.[7][8]

  1. ^ "Adrenal Gland Tumor: Statistics". Cancer.net. 25 June 2012. Retrieved 2020-07-01.
  2. ^ Cite error: The named reference WangSun2014 was invoked but never defined (see the help page).
  3. ^ Fassnacht M, Dekkers OM, Else T, Baudin E, Berruti A, de Krijger R, et al. (October 2018). "European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors". European Journal of Endocrinology. 179 (4): G1–G46. doi:10.1530/EJE-18-0608. hdl:2318/1650497. PMID 30299884.
  4. ^ Fassnacht M, Terzolo M, Allolio B, Baudin E, Haak H, Berruti A, et al. (June 2012). "Combination chemotherapy in advanced adrenocortical carcinoma". The New England Journal of Medicine. 366 (23): 2189–2197. doi:10.1056/NEJMoa1200966. hdl:2318/102217. PMID 22551107.
  5. ^ a b DeVita VT, Hellman S, Rosenberg SA, eds. (2005). Cancer: principles & practice of oncology. Philadelphia: Lippincott-Raven. ISBN 978-0-7817-4865-0.
  6. ^ Savarese DM, Nieman LK (2006-08-08). "Clinical presentation and evaluation of adrenocortical tumors". UpToDate Online v. 15.1. UpToDate. Archived from the original on 2007-09-29. Retrieved 2007-06-05.
  7. ^ Wang T, Rainey WE (March 2012). "Human adrenocortical carcinoma cell lines". Molecular and Cellular Endocrinology. 351 (1): 58–65. doi:10.1016/j.mce.2011.08.041. PMC 3288152. PMID 21924324.
  8. ^ Gazdar AF, Oie HK, Shackleton CH, Chen TR, Triche TJ, Myers CE, et al. (September 1990). "Establishment and characterization of a human adrenocortical carcinoma cell line that expresses multiple pathways of steroid biosynthesis". Cancer Research. 50 (17): 5488–5496. PMID 2386954.