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| {{Infobox_Disease |
| | __NOTOC__ |
| Name = {{PAGENAME}} |
| | {{Adrenocortical carcinoma}} |
| Image = Adrenocortical Ca Met to Lung.jpg|
| | '''For patient information page, please click [[ Adrenocortical carcinoma (patient information)|here]]''' |
| Caption = Metastasis of an adrenocortical carcinoma to the lung|
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| DiseasesDB = |
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| ICD10 = {{ICD10|C|74|0|c|73}}|
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| ICD9 = {{ICD9|194}} |
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| eMedicineSubj = ped |
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| eMedicineTopic = 41 |
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| {{SI}}
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| {{CMG}}
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| {{Editor Help}} | | {{CMG}}; {{AE}} {{RT}} {{AAM}} {{MAD}} |
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| ==Overview==
| | '''''Synonyms and keywords:''''' Adrenal cortical cancer; Adrenal cortex cancer |
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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]]. Adrenocortical carcinoma is a rare tumor, with incidence of 1-2 per million population annually.<ref name="DeVita">{{cite book |author=edited by Vincent T. DeVita, Samuel Hellman, Steven A. Rosenberg |title=Cancer: principles & practice of oncology |publisher=Lippincott-Raven |location=Philadelphia |year=2005 |pages= |isbn=0-7817-4865-8 |oclc= |doi=}}</ref><ref name="UTDACC">{{cite web | last =Savarese | first =Diane MF | coauthors =Lynnette K Nieman| title =Clinical presentation and evaluation of adrenocortical tumors| work =UpToDate Online v. 15.1| publisher =UpToDate| date = August 8, 2006| url =http://www.uptodateonline.com/utd/content/topic.do?topicKey=adrenal/17707&type=A&selectedTitle=1~16 | accessdate =June 5, 2007}}</ref> Adrenocortical carcinoma has a bimodal distribution by age, with cases clustering in children under 6, and in adults 30-40 years old.<ref name="DeVita"/> 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]]. Adrenocortical carcinoma has often invaded nearby tissues or [[metastasis|metastasized]] to distant organs at the time of diagnosis, and the overall 5-year survival rate is only 20-35%.<ref name="DeVita"/>
| | ==[[Adrenocortical carcinoma overview|Overview]]== |
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| == Signs and Symptoms == | | ==[[Adrenocortical carcinoma historical perspective|Historical Perspective]]== |
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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]]. <ref name="DeVita"/> 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]] excess) occur in less than 10% of cases. Rarely, [[pheochromocytoma]]-like hypersecretion of [[catecholamine]]s has been reported in adrenocortical cancers.<ref name="Weidner's">{{cite book |author=Richard Cote, Saul Suster, Lawrence Weiss, Noel Weidner (Editor) |title=Modern Surgical Pathology (2 Volume Set) |publisher=W B Saunders |location=London |year= |pages= |isbn=0-7216-7253-1 |oclc= |doi=}}</ref> Non-functional tumors (about 40%, authorities vary) usually present with abdominal or flank pain, or they may be asymptomatic and detected incidentally.<ref name="UTDACC"/>
| | ==[[Adrenocortical carcinoma classification|Classification]]== |
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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" 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]].<ref name="DeVita"/><ref name="UTDACC"/><ref>Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL. ''Harrison's Principles of Internal Medicine''. New York: McGraw-Hill, 2005. ISBN 0-07-139140-1</ref>
| | ==[[Adrenocortical carcinoma pathophysiology|Pathophysiology]]== |
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| == Diagnosis == | | ==[[Adrenocortical carcinoma causes|Causes]]== |
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| ===Laboratory findings=== | | ==[[Adrenocortical carcinoma differential diagnosis|Differentiating Adrenocortical carcinoma from other Diseases]]== |
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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]]
| | ==[[Adrenocortical carcinoma epidemiology and demographics|Epidemiology and Demographics]]== |
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| ===Radiology=== | | ==[[Adrenocortical carcinoma risk factors|Risk Factors]]== |
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| Radiological studies of the [[abdomen]], such as [[CT scan]]s 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]]s are routinely performed to look for [[metastasis|metastases]] to the [[lung]]s and [[bone]]s respectively. These studies are critical in determining whether or not the tumor can be [[surgery|surgically]] removed, the only potential cure at this time.<ref name="UTDACC"/>
| | ==[[Adrenocortical carcinoma screening|Screening]]== |
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| ====MRI==== | | ==[[Adrenocortical carcinoma natural history|Natural History, Complications and Prognosis]]== |
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| ([http://www.radswiki.net Images courtesy of RadsWiki])
| | == Diagnosis == |
| | | [[Adrenocortical carcinoma diagnostic study of choice|Diagnostic study of choice]] | [[Adrenocortical carcinoma history and symptoms| History and Symptoms]] | [[Adrenocortical carcinoma physical examination | Physical Examination]] | [[Adrenocortical carcinoma laboratory findings|Laboratory Findings]] | [[Adrenocortical carcinoma x ray|X Ray]] | [[Adrenocortical carcinoma MRI|MRI]] | [[Adrenocortical carcinoma CT|CT]] | [[Adrenocortical carcinoma ultrasound|Ultrasound]] | [[Adrenocortical carcinoma other imaging studies|Other Imaging Studies]] | [[Adrenocortical carcinoma other diagnostic studies|Other Diagnostic Studies]] |
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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]]. Grossly, adrenocortical carcinomas are often large, with a tan-yellow cut surface, and areas of [[hemorrhage]] and [[necrosis]]. On [[histopathology|microscopic examination]], the tumor usually displays sheets of atypical cells with some resemblance to the cells of the normal [[adrenal cortex]]. The presence of invasion and [[mitosis|mitotic activity]] help differentiate small cancers from [[adrenocortical adenoma]]s.<ref name="Weidner's">{{cite book |author=Richard Cote, Saul Suster, Lawrence Weiss, Noel Weidner (Editor) |title=Modern Surgical Pathology (2 Volume Set) |publisher=W B Saunders |location=London |year= |pages= |isbn=0-7216-7253-1 |oclc= |doi=}}</ref>
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| There are several relatively rare variants of adrenal cortical carcinoma: Oncocytic adrenal cortical carcinoma, Myxoid adrenal cortical carcinoma, Carcinosarcoma, Adenosquamous adrenocortical carcinoma, Clear cell adrenal cortical carcinoma.
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| ==Differential Diagnosis==
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| * [[Adrenocortical adenoma]]
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| * [[Renal cell carcinoma]]
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| * Adrenal medullary tumors
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| * [[Hepatocellular carcinoma]]
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| ==Treatment== | | ==Treatment== |
| | [[Adrenocortical carcinoma medical therapy|Medical Therapy]] | [[Adrenocortical carcinoma surgery|Surgery]] | [[ Adrenocortical carcinoma radiation therapy |Radiation Therapy]] | [[Adrenocortical carcinoma cost-effectiveness of therapy|Cost-effectiveness of Therapy]] | [[Adrenocortical carcinoma future or investigational therapies|Future or Investigational Therapies]] |
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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. [[Radiation therapy]] and [[radiofrequency ablation]] may be used for [[palliative care|palliation]] in patients who are not surgical candidates.<ref name="DeVita"/>
| | ==Case Study== |
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| | [[Adrenocortical carcinoma case study one|Case #1]] |
| [[Chemotherapy]] regimens typically include the drug [[mitotane]], an inhibitor of [[steroid]] synthesis which is toxic to cells of the [[adrenal cortex]],<ref name="G&G">{{cite book |author=Laurence L. Brunton, editor-in-chief;
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| John S. Lazo and Keith L. Parker, Associate Editors |title=Goodman & Gilman's The Pharmacological Basis of Therapeutics, 11th Edition |publisher=The McGraw-Hill Companies, Inc. |location=United States of America |year=2006 |pages= |isbn=0-07-142280-3 |oclc= |doi= }}</ref> 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]]).<ref name="DeVita"/>
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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.<ref name="DeVita"/>
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| ==Prognosis==
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| ACC, generally, carries a poor prognosis<ref name=allolio>{{cite journal | author = Allolio B, Fassnacht M | title = Clinical review: Adrenocortical carcinoma: clinical update. | journal = J Clin Endocrinol Metab | volume = 91 | issue = 6 | pages = 2027-37 | year = 2006 | id = PMID 16551738}} [http://jcem.endojournals.org/cgi/content/full/91/6/2027 Free Full Text].</ref>
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| and is unlike most tumours of the adrenal cortex, which are [[benign]] ([[adenoma]]s) 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%.<ref name=allolio/>
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| The most important prognostic factors are age of the patient and stage of the tumor. Poor prognostic factors: mitotic activity, venous invasion, weight of 50g+; diameter of 6.5 cm+, Ki-67/MIB1 labeling index of 4%+, p53+.
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| ==References==
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| <references/>
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| == External links ==
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| *[http://survivor-support.rare-cancer.org/forum/ Adrenocortical Support Group]
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| *[http://www.endotext.org/adrenal/adrenal22/adrenalframe22.htm www.endotext.org article]
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| </br>
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| {{SIB}}
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| [[Category:Endocrinology]] | | [[Category:Endocrinology]] |
| [[Category:Types of cancer]] | | [[Category:Types of cancer]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
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