Adrenocortical carcinoma: Difference between revisions

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{{Infobox_Disease |
__NOTOC__
  Name          = {{PAGENAME}} |
  Image          = Adrenocortical Ca Met to Lung.jpg|
  Caption        = Metastasis of an adrenocortical carcinoma to the lung|
  DiseasesDB    = |
  ICD10          = {{ICD10|C|74|0|c|73}}|
  ICD9          = {{ICD9|194}} |
  ICDO          = |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
}}
{{Adrenocortical carcinoma}}
{{Adrenocortical carcinoma}}
'''For patient information page, please click [[ Adrenocortical carcinoma  (patient information)|here]]'''


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


== Signs and Symptoms ==
'''''Synonyms and keywords:''''' Adrenal cortical cancer; Adrenal cortex cancer


== Diagnosis ==
==[[Adrenocortical carcinoma overview|Overview]]==


===Radiology===
==[[Adrenocortical carcinoma historical perspective|Historical Perspective]]==


===Pathology===
==[[Adrenocortical carcinoma classification|Classification]]==


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>
==[[Adrenocortical carcinoma pathophysiology|Pathophysiology]]==
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 carcinoma causes|Causes]]==


* [[Adrenocortical adenoma]]
==[[Adrenocortical carcinoma differential diagnosis|Differentiating Adrenocortical carcinoma from other Diseases]]==
* [[Renal cell carcinoma]]
* Adrenal medullary tumors
* [[Hepatocellular carcinoma]]


==Treatment==
==[[Adrenocortical carcinoma epidemiology and demographics|Epidemiology and Demographics]]==
 
==[[Adrenocortical carcinoma risk factors|Risk Factors]]==
 
==[[Adrenocortical carcinoma screening|Screening]]==


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"/>
==[[Adrenocortical carcinoma natural history|Natural History, Complications and Prognosis]]==
[[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"/>
== 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]]


==Prognosis==
==Treatment==
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>
[[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]]  
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==
==Case Study==
<references/>
[[Adrenocortical carcinoma case study one|Case #1]]


== External links ==
*[http://survivor-support.rare-cancer.org/forum/ Adrenocortical Support Group]
*[http://www.endotext.org/adrenal/adrenal22/adrenalframe22.htm www.endotext.org article]
*[http://www.bidmc.org/YourHealth/ConditionsAZ.aspx?ChunkID=179661 Beth Israel Deaconess Medical Center: Adrenocortical carcinoma]
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Latest revision as of 18:35, 24 October 2019

Adrenocortical carcinoma Microchapters

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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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