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| {{CMG}}; {{AE}} {{S.M.}} | | {{CMG}}; {{AE}} {{S.M.}} |
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| == Overview (Patient information) ==
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| Adrenocortical carcinoma is a cancer of the [[adrenal glands]].
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| == What are the symptoms of Adrenal carcinoma? ==
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| Symptoms that suggest increased cortisol or other adrenal gland hormone production: Fatty, rounded hump high on the back just below the neck (buffalo hump) Flushed rounded face with pudgy cheeks (moon face) Obesity Stunted growth in height (short stature) Virilization - the appearance of male characteristics, including increased body hair (especially on the face), pubic hair, acne, deepening of voice, and enlarged clitoris (girls)
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| Symptoms that suggest increased aldosterone production are the same as symptoms of low potassium and include:
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| Increased thirst Muscle cramps Urination Weakness
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| == What causes Adrenal carcinoma? ==
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| Adrenocortical carcinoma is most common in children younger than 5 and adults in their 30s and 40s.
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| Adrenocortical carcinoma may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor.
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| Adrenocortical carcinoma 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.
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| The cause is unknown. About 2 people per million develop this type of tumor.
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| == Diagnosis ==
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| A physical exam may reveal high blood pressure and changes in body shape, such as breast enlargement in men (gynecomastia) or male characteristics in women (virilization). Blood tests will be done to check hormone levels: ACTH level will be low. Aldosterone level will be high. Cortisol level will be high. Potassium level will be low.
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| Imaging tests may include:
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| Abdominal x-ray CT scan of the abdomen MRI of the abdomen
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| == When to seek urgent medical care? ==
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| Call your health care provider if you or your child has symptoms of adrenocortical carcinoma, Cushing syndrome, or failure to grow.
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| == Treatment options ==
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| Primary treatment is surgery to remove the tumor. Adrenocortical carcinoma may not improve with chemotherapy. Medications may be given to reduce production of cortisol, which causes many of the symptoms.
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| == What to expect (Outlook/Prognosis)? ==
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| 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.
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| == Possible complications ==
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| The tumor can spread to the liver, bone, lung, or other areas.
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| == Sources ==
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| * <nowiki>http://www.nlm.nih.gov/medlineplus/ency/article/001663.htm</nowiki>
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| == Overview ==
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| '''Adrenocortical carcinoma''', also '''adrenal cortical carcinoma''' (ACC) and '''adrenal cortex cancer''', is an aggressive [[cancer]] originating in the [[Adrenal cortex|cortex]] ([[steroid hormone]]-producing tissue) of the [[adrenal gland]]. Adenocortical carcinoma is remarkable for the many hormonal syndromes which can occur in patients with steroid hormone-producing ("functional") tumors, including [[Cushing's syndrome]], [[Conn syndrome]], [[virilization]], and [[Feminization (biology)|feminization]].
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| == Overview ==
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| Adrenal carcinoma may be classified according to its activity into two subtypes: functioning and non-functioning.
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| == Classification ==
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| Adrenal cancer is classified to:
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| * Functional
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| * Non-functional
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| == Pathophysiology ==
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| === Pathology ===
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| Adrenal tumors are often not biopsied prior to surgery, so diagnosis is confirmed on examination of the surgical specimen by a [[Anatomical pathology|pathologist]].
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| == Prognosis ==
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| ACC, generally, carries a poor prognosis and is unlike most tumours of the adrenal cortex, which are [[benign]] ([[Adenoma|adenomas]]) and only occasionally cause [[Cushing's syndrome]]. Five-year disease-free survival for a complete resection of a [[Cancer staging|stage]] I-III ACC is approximately 30%.
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| == History and Symptoms ==
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| Adrenocortical carcinoma may present differently in children and adults. Most tumors in children are functional, and [[virilization]] is by far the most common presenting symptom, followed by [[Cushing's syndrome]] and [[precocious puberty]]. Among adults presenting with hormonal syndromes, Cushing's syndrome alone is most common, followed by mixed Cushing's and virilization ([[glucocorticoid]] and [[androgen]] overproduction). [[Feminization]] and [[Conn syndrome]] ([[mineralcorticoid]]<nowiki/>excess) occur in less than 10% of cases. Rarely, [[pheochromocytoma]]-like hypersecretion of [[Catecholamine|catecholamines]] has been reported in adrenocortical cancers. Non-functional tumors (about 40%, authorities vary) usually present with abdominal or flank pain, or they may be asymptomatic and detected incidentally.
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| All patients with suspected adrenocortical carcinoma should be carefully evaluated for signs and symptoms of hormonal syndromes. For Cushing's syndrome ([[glucocorticoid]] excess) these include [[weight gain]], [[muscle wasting]], purple lines on the abdomen, a fatty [[Buffalo hump|"buffalo hump"]] on the neck, a [[Moon face|"moonlike" face]], and thinning, fragile skin. Virilism ([[androgen]] excess) is most obvious in women, and may produce [[Hirsutism|excess facial and body hair]], [[acne]], enlargement of the [[clitoris]], deepening of the voice, coarsening of facial features, and [[Amenorrhea|cessation of menstruation]]. [[Conn syndrome]] ([[mineralcorticoid]] excess) is marked by [[high blood pressure]], which can result in [[headache]], and [[hypokalemia]] (low serum potassium), which can produce muscle weakness, confusion, and [[palpitations]]. low plasma [[renin]] activity, and high serum [[aldosterone]]. [[Feminization]] ([[estrogen]] excess) is most readily noted in men, and includes [[Gynecomastia|breast enlargement]], decreased [[libido]] and [[impotence]].
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| # [[Adrenal carcinoma history and symptoms#cite%20ref-4|Jump up↑]] Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL. ''Harrison's Principles of Internal Medicine''. New York: McGraw-Hill, 2005. <nowiki>ISBN 0-07-139140-1</nowiki>
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| == Physical Examination ==
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| All patients with suspected adrenocortical carcinoma should be carefully examined for Cushing's syndrome ([[glucocorticoid]] excess) these include [[weight gain]], [[muscle wasting]], purple lines on the abdomen, a fatty [[Buffalo hump|"buffalo hump"]] on the neck, a [[Moon face|"moonlike" face]], and thinning, fragile skin. Virilism ([[androgen]] excess) is most obvious in women, and may produce [[Hirsutism|excess facial and body hair]], [[acne]], enlargement of the [[clitoris]], deepening of the voice, coarsening of facial features, and [[Amenorrhea|cessation of menstruation]]. [[Conn syndrome]] ([[mineralcorticoid]] excess) is marked by [[high blood pressure]], which can result in [[headache]], and [[hypokalemia]] (low serum potassium), which can produce muscle weakness, confusion, and [[palpitations]]. low plasma [[renin]] activity, and high serum [[aldosterone]]. [[Feminization]] ([[estrogen]]<nowiki/>excess) is most readily noted in men, and includes [[Gynecomastia|breast enlargement]], decreased [[libido]] and [[impotence]].
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| # [[Adrenal carcinoma physical examination#cite%20ref-3|Jump up↑]] Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL. ''Harrison's Principles of Internal Medicine''. New York: McGraw-Hill, 2005. <nowiki>ISBN 0-07-139140-1</nowiki>
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| == Laboratory findings ==
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| Hormonal syndromes should be confirmed with laboratory testing. Laboratory findings in Cushing syndrome include increased [[serum glucose]] (blood sugar) and increased urine [[cortisol]]. Adrenal virilism is confirmed by the finding of an excess of serum [[androstenedione]] and [[dehydroepiandrosterone]]. Findings in [[Conn syndrome]] include [[Hypokalemia|low serum potassium]], low plasma [[renin]] activity, and high serum [[aldosterone]]. Feminization is confirmed with the finding of excess serum [[estrogen]]
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| == CT ==
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| Radiological studies of the [[abdomen]], such as [[CT scan|CT scans]] and [[magnetic resonance imaging]] are useful for identifying the site of the tumor, differentiating it from other diseases, such as [[adrenocortical adenoma]], and determining the extent of invasion of the tumor into surrounding organs and tissues. CT scans of the [[chest]] and [[Bone scan|bone scans]] are routinely performed to look for [[Metastasis|metastases]] to the [[Lung|lungs]] and [[Bone|bones]] respectively. These studies are critical in determining whether or not the tumor can be [[Surgery|surgically]] removed, the only potential [[cure]] at this time.
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| == Medical Therapy ==
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| [[Radiation therapy]] and [[radiofrequency ablation]] may be used for [[Palliative care|palliation]] in patients who are not surgical candidates.
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| [[Chemotherapy]] regimens typically include the drug [[mitotane]], an inhibitor of [[steroid]] synthesis which is toxic to cells of the [[adrenal cortex]], as well as standard cytotoxic drugs. One widely used regimen consists of [[cisplatin]], [[doxorubicin]], [[etoposide]]) and mitotane. The endocrine cell toxin [[streptozotocin]] has also been included in some treatment protocols. Chemotherapy may be given to patients with unresectable disease, to shrink the tumor prior to surgery ([[neoadjuvant chemotherapy]]), or in an attempt to eliminate microscopic residual disease after surgery ([[adjuvant chemotherapy]]).
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| [[Hormonal therapy (oncology)|Hormonal therapy]] with steroid synthesis inhibitors such as [[aminoglutethimide]] may be used in a palliative manner to reduce the symptoms of hormonal syndromes.
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| == Surgery ==
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| The only curative treatment is complete [[Surgery|surgical]] excision of the tumor, which can be performed even in the case of invasion into large blood vessells, such as the [[renal vein]] or [[inferior vena cava]]. The 5-year survival rate after successful surgery is 50-60%, but unfortunately, a large percentage of patients are not surgical candidates.
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| <br />
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| ==References==
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| {{Reflist|2}}
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