It is a cancer that is mainly originating in the cortex region of adrenal gland, which produces steroids. There are two regions in the adrenal gland namely adrenal cortex and adrenal medulla. Adrenal cortex has three layers: Glomerulosa, reticulata, fasciculata. Glomerulosa secretes Mineralocrticoids; Reticulata secretes glucocortcoids while fasiculata sceretes the Andrgens. Now, the tumour of adrenal cortex of a gland can produce excessive secretion of these hormones, So, there is manifestation like Cushig syndrome, Conn's disease, virilization in females and feminization in males. It is a rare type of cancer. Variety of adrenocortical carcinoma is clear cell adrenocortical carcinoma, myxoid adrenocortical carcinoma, adenosquamous carcinoma, oncocytic adrenocortical carcinoma.
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The genes like h19, insulin growth factor, p57 are important for the development of the foetus. Expression of the h19 gene is markedly reduced in both nonfunctioning and functioning adrenalcortical carcinomas. This cancer is commonly seen in people who have family history of Li-Fraumeni syndrome, which is characterized by the inactivation of tumour suppressor genes p53 and RB (retinoblastoma) located on 17th and 13th chromosomes may get changed. There is also a loss of activity of the p57kip2 gene product in virilizing adenomas and adrenal cortical carcinomas. Thus the cause of this cancer is basically a defect in genes.
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The symptoms are different in the children and adults. In children, there is Cushing syndrome and precious puberty while in adults there is virilization and Cushing syndrome. Feminization and Conn's diseases are only 10% of adrenocortical carcinoma. Sometimes, pheocytochroma-like condition due to excessive secretion from adrenal medulla may occur, but it is rare.
Cushing syndrome includes weight gain, muscle wasting, purple colour on the abdomen, moon like face due to deposition of the lipids
Virilization is in females due to excess testosterone in them. Virilization is excess facial and body hair, acne, enlargement of the clitoris, deepening of the voice, coarsening of facial features, cessation of menstruation.
Feminization occurs in males, which is characterized by the breast enlargement, decreased libido and impotence. These symptoms are mainly due to estrogen excess by excessive secretion of androgens from the adrenal gland.
Conn's disease includes excessive secretion of mineralocorticoids, which causes the Hypertension (due to excess of Na), Hypokalemia. The aldosterone causes the reabsorption of Na while K+ ions gets secreted in the urine results in hypokalemia, headache, muscle weakness and palpitations, high serum aldosterone.
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Chemotherapy includes the use of cytotoxic drugs like mitotone, which is cytotoxic to the cells of adrenal cortex and stops the synthesis of steroids.
Streptozoctocin is an endocrinal cell poison, which can also be a part of treatment
Chemotherapy is used in two cases, when we have to shrink the size of tumour before surgery or in case where we have to remove the microscopic residues of the tumour after surgery. The former is called as neoaduvant therapy while the latter is called as adjuvant chemotherapy.
Surgery is the only curative treatment for the cancer is the surgical removal of the tumour. Even if the cancer gets spread to the renal vein or inferior vena cava then, the tumour can be removed in that condition also.
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