Adrenocortical carcinoma: Difference between revisions

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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|
  DiseasesDB    = |
  ICD10          = {{ICD10|C|74|0|c|73}}|
  ICD9          = {{ICD9|194}} |
  ICDO          = |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj = ped |
  eMedicineTopic = 41 |
}}
{{SI}}
{{CMG}}


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{{CMG}}; {{AE}} {{RT}} {{AAM}} {{MAD}}


==Overview==
'''''Synonyms and keywords:''''' Adrenal cortical cancer; Adrenal cortex cancer


'''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]]==


== Signs and Symptoms ==
==[[Adrenocortical carcinoma historical perspective|Historical Perspective]]==


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]]==


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]]==


== Diagnosis ==
==[[Adrenocortical carcinoma causes|Causes]]==


===Laboratory findings===
==[[Adrenocortical carcinoma differential diagnosis|Differentiating Adrenocortical carcinoma from other Diseases]]==


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]]==


===Radiology===
==[[Adrenocortical carcinoma risk factors|Risk Factors]]==


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]]==


====MRI====
==[[Adrenocortical carcinoma natural history|Natural History, Complications and Prognosis]]==


([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===
 
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>
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.
 
==Differential Diagnosis==
 
* [[Adrenocortical adenoma]]
* [[Renal cell carcinoma]]
* Adrenal medullary tumors
* [[Hepatocellular carcinoma]]


==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]]


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==
[[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;
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"/>


[[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"/>
==Prognosis==
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>
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/>
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+.
==References==
<references/>
== External links ==
*[http://survivor-support.rare-cancer.org/forum/ Adrenocortical Support Group]
*[http://www.endotext.org/adrenal/adrenal22/adrenalframe22.htm www.endotext.org article]
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Latest revision as of 18:35, 24 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Ahmad Al Maradni, M.D. [3] Mohammed Abdelwahed M.D[4]

Synonyms and keywords: Adrenal cortical cancer; Adrenal cortex cancer

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Adrenocortical carcinoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | X Ray | MRI | CT | Ultrasound | Other Imaging Studies | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Radiation Therapy | Cost-effectiveness of Therapy | Future or Investigational Therapies

Case Study

Case #1

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