Adrenocortical carcinoma
This carcinoma, adrenal cortex also (ACC) and cancer of the adrenal cortex, is an aggressive cancer originating in the cortex (steroid hormone-producing tissue) of the adrenal gland. This carcinoma is a rare tumor, with an incidence of 1-2 per million inhabitants annually.Adrenocortical carcinoma has a bimodal age distribution, with cases clustering in children under 5 years and adults 30-40 years old. adrenocortical carcinoma is notable for the many hormonal syndromes that can occur in patients with steroid hormone-producing tumors ( "functional"), including Cushing's syndrome, Conn syndrome, virilization, and feminization. This often carcinoma has invaded nearby tissues or organs distant metastases at diagnosis, and the overall survival rate at 5 years is only 20-35%. Steroids line producing angiotensin-II-sensitive widely used cell H295R was originally isolated from a tumor diagnosed as adrenocortical carcinoma.
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This carcinoma may present differently in children and adults. Most tumors in children are functional, and virilization is by far the most common symptom, followed by the syndrome and Cushing early puberty.Among adults who have hormonal syndromes, Cushing's syndrome alone is the most common, followed by mixing Cushing and virilization (glucocorticoid and androgen overproduction). Feminization syndrome Conn (excess mineralocorticoid) occur in less than 10% of cases. Rarely, hypersecretion of pheochromocytoma-like catecholamines has been reported in cancer of the adrenal cortex. The nonfunctioning tumors (about 40%, authorities vary) usually present with abdominal or flank pain, varicocele and renal vein thrombosis or may be asymptomatic and detected incidentally.
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All patients with suspected adrenocortical carcinoma should be evaluated carefully for signs and symptoms of hormonal syndromes. For Cushing's syndrome (excess glucocorticoid) these include weight gain, loss of muscle mass, purple lines on the abdomen, fat "buffalo hump" in the neck, a "moon" face, and thinning , fragile skin. Virilismo (androgen excess) is more evident in women, and can cause excess facial and body hair, acne, enlargement of the clitoris, deepened voice, thickening of facial features, cessation of menstruation. Conn (mineralocorticoid excess) syndrome is marked by high blood pressure that can result in headache and hypokalemia (serum potassium low, which in turn can cause muscle weakness, confusion, palpitations) and renin activity plasma low and high serum aldosterone. Feminization (excess estrogen) is most easily seen in men, including breast enlargement, decreased libido and impotence.
