Adrenal carcinoma: Difference between revisions

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'''For patient information, click [[Adrenal carcinoma (patient information)|here]]'''
'''For patient information, click [[Adrenal carcinoma (patient information)|here]]'''
{{Adrenal carcinoma}}
{{Adrenal carcinoma}}
{{CMG}};{{AE}} {{S.M.}}
{{CMG}} {{AE}} {{S.M.}}
 
{{SK}} [[Adrenocortical carcinoma]], [[Adrenal cortical cancer|Adrenal cortical carcinoma]], [[Adrenal cortical cancer]], [[Adrenal cortex cancer]], Adrenal cancer, [[Adrenal tumor (patient information)|Adrenal tumor]], [[Neuroblastoma]], [[Pheochromocytoma]], [[Ganglioneuroma]], [[Adrenal adenoma|Adrenocortical adenoma]], [[Adenomatoid tumor]], [[Myelolipoma]], [[Schwannoma]].
 
== Overview ==
== Overview ==
'''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]].
'''[[Adrenal gland|Adrenal]] [[carcinoma]] or [[Adrenal tumor (patient information)|adrenal tumor]]''' is an aggressive [[disease]] which can [[Origin|originate]] either in the [[Adrenal cortex|cortex]] ([[steroid hormone]]-[[Product (biology)|producing]] [[Tissue (biology)|tissue]]) or [[medulla]] of the [[adrenal gland]]. According to the 2017 [[World Health Organization|WHO]] [[classification]] of [[Adrenal tumor (patient information)|adrenal tumors]], [[Adrenal cortical cancer|adrenal cortical tumors]] are subclassified into [[Adenoma|cortical adenoma]], [[Adrenocortical carcinoma|cortical carcinoma]], [[Sex cord-Stromal Tumor|sex cord stromal tumors]], [[adenomatoid tumor]], [[mesenchymal]] and [[stromal]] [[tumors]] ([[myelolipoma]], [[schwannoma]]), [[Haematological malignancy|hematological tumors]] and [[secondary]] [[tumors]], whereas [[tumors]] of [[adrenal medulla]] are subclassified into [[pheochromocytoma]], [[paraganglioma]], [[Neuroblastoma|neuroblastic tumors]], [[Pheochromocytoma|composite pheochromocytoma]], and [[Paraganglioma|composite paraganglioma]]. [[Pathogenesis]] includes many [[Genetic pathway|genetic pathways]], most prominent being [[Wnt signaling pathway|Wnt-Beta catenin pathway]] and also [[Association (statistics)|association]] with other [[diseases]] such as [[multiple endocrine neoplasia]] ([[MEN1]] and [[Multiple endocrine neoplasia type 2|MEN2]]), [[familial adenomatous polyposis]], [[Beckwith-Wiedemann syndrome]], [[Li-Fraumeni syndrome]], [[Lynch syndrome]],[[von Hippel-Lindau disease]], [[Carney complex|carney Complex]]/[[Carney syndrome|Syndrome]], [[Neurofibromatosis type I|neurofibromatosis type 1]] and [[congenital adrenal hyperplasia]]. [[Adrenocortical carcinoma]] is remarkable for the many [[hormonal]] [[syndromes]] which can occur in [[patients]] with [[steroid hormone]]-[[Product (biology)|producing]] ("[[Function (biology)|functional]]") [[tumors]], including [[Cushing's syndrome]], [[Conn syndrome]], [[virilization]], and [[Feminization (biology)|feminization]]. [[Adrenal]] [[carcinoma]] can be [[Treatments|treated]] with both [[Medical therapy template|medical therapy]] and [[surgery]] [[Dependent variable|depending]] upon the [[Stages of human development|stage]] of the [[tumor]].


==Historical perspective==
==Historical perspective==
*In 2017, [[World Health Organization|WHO]] presented an update on [[Recent changes|recent]] [[classification]] of [[Adrenal tumor|adrenal tumors]] (in fourth edition of the [[World Health Organization]] [[classification]] of [[endocrine tumors]])<ref name="pmid28477311">{{cite journal| author=Lam AK| title=Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. | journal=Endocr Pathol | year= 2017 | volume= 28 | issue= 3 | pages= 213-227 | pmid=28477311 | doi=10.1007/s12022-017-9484-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28477311  }} </ref>
*In 1978, Sullivan devised a [[Cancer staging|staging]] [[system]] for [[adrenocortical carcinoma]] [[Base|based]] on [[Modifications (genetics)|modifying]] the original McFarlane [[Cancer staging|staging]] [[system]].
*In 2004, [[International Union Against Cancer]] ([[UICC]]) and [[World Health Organization]] ([[World Health Organization|WHO]]) [[Proposition|proposed]] a [[new]] [[Cancer staging|staging]] [[system]] which was [[Base|based]] on the [[Modifications (genetics)|modified]] original [[Version (eye)|version]] of Sullivan-McFarlane [[Cancer staging|staging]] [[criteria]].
*In 2017, [[World Health Organization|WHO]] presented an update on [[Recent changes|recent]] [[classification]] of [[Adrenal tumor|adrenal tumors]] (in fourth edition of the [[World Health Organization]] [[classification]] of [[endocrine tumors]]).<ref name="pmid28477311">{{cite journal| author=Lam AK| title=Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. | journal=Endocr Pathol | year= 2017 | volume= 28 | issue= 3 | pages= 213-227 | pmid=28477311 | doi=10.1007/s12022-017-9484-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28477311  }} </ref>


== Classification ==
== Classification ==
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****[[Confusion|Confu]]<nowiki/>[[Confusion|sion]]
****[[Confusion|Confu]]<nowiki/>[[Confusion|sion]]
****[[Palpitations]]
****[[Palpitations]]
*'''[[pheochromocytoma|Pheochromocytom]]'''<nowiki/>'''[[pheochromocytoma|a]]-like hypers'''<nowiki/>'''ecretion of [[Catecholamine|catechol]]'''<nowiki/>'''[[Catecholamine|amines]]''' ([[Rare|rarely]])
*'''[[pheochromocytoma|Pheochromocytom]]'''<nowiki/>'''[[pheochromocytoma|a]]-like hypers'''<nowiki/>'''ecretion of [[Catecholamine|catechol]]'''<nowiki/>'''[[Catecholamine|amines]]''' ([[Rare|rarely]]) [[Causes|causing]] the following [[symptoms]]:
====Presentation of non-functional adrenal carcinoma====
**[[High blood pressure]]
**[[Cardiac arrythmia|Cardiac arrythmias]]
**[[Headache]]
**[[Palpitations]]
**[[Anxiety attack|Anxiety attacks]]
**[[Sweating]]
**[[Weight loss]]
**[[Tremor]]
 
====Pre<nowiki/>sentati<nowiki/>on of<nowiki/> non-functional<nowiki/> adrenal carcinoma====
*Non-[[Function (biology)|functional]] [[tumors]] (40%) usually [[Presenting symptom|present]] with:
*Non-[[Function (biology)|functional]] [[tumors]] (40%) usually [[Presenting symptom|present]] with:
**[[Abdominal]] or [[Flank pain|flank]] or [[back pain]]
**[[Abdominal]] or [[Flank pain|flank]] or [[back pain]]
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===Imaging studies===
===Imaging studies===


*Following table shows the list of different imaging tests that are helpful in diagnosing adrenal carcinoma:
*Following table shows the list of different [[Imaging studies|imaging tests]] that are helpful in [[Diagnosis|diagnosing]] [[Adrenal tumor (patient information)|adrenal carcinoma]]:


{| class="wikitable"
{| class="wikitable"
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! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Details}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Details}}
|-
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''CT'''<ref name="pmid15671003">{{cite journal| author=Szolar DH, Korobkin M, Reittner P, Berghold A, Bauernhofer T, Trummer H et al.| title=Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT. | journal=Radiology | year= 2005 | volume= 234 | issue= 2 | pages= 479-85 | pmid=15671003 | doi=10.1148/radiol.2342031876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15671003  }} </ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[CT]]'''<ref name="pmid15671003">{{cite journal| author=Szolar DH, Korobkin M, Reittner P, Berghold A, Bauernhofer T, Trummer H et al.| title=Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT. | journal=Radiology | year= 2005 | volume= 234 | issue= 2 | pages= 479-85 | pmid=15671003 | doi=10.1148/radiol.2342031876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15671003  }} </ref>
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*Abdominal [[CT scan]] is useful for:
*[[Abdominal]] [[CT scan]] is useful for:
**Identifying the tumor site
**Identifying the [[tumor]] site
**Differentiating adrenocortical carcinoma from [[adrenocortical adenoma]]
**[[Differentiate|Differentiating]] [[adrenocortical carcinoma]] from [[adrenocortical adenoma]]
**Local tumor recurrence
**[[Local]] [[tumor]] [[Recurrence plot|recurrence]]
**Determining the extent of tumor invasion into the surrounding organs and tissues and distant metastasis
**Determining the [[Extent of reaction|extent]] of [[tumor]] [[invasion]] into the surrounding [[organs]] and [[tissues]] and [[Distance matrix|distant]] [[metastasis]]
* Chest CT scan is routinely performed to look for [[Metastasis|metastases]] to the [[Lung|lungs]] and is critical in determining whether or not the tumor can be [[Surgery|surgically]] removed (the only potential [[cure]] after metastasis)
*[[Chest]] [[Computed tomography|CT scan]] is routinely [[Performance status|performed]] to [[Lookahead|look]] for [[Metastasis|metastases]] to the [[Lung|lungs]] and is critical in determining whether or not the [[tumor]] can be [[Surgery|surgically]] removed (the only [[potential]] [[cure]] after [[metastasis]])
*CT scan of adrenocortical carcinoma shows:
*[[CT scan]] of [[adrenocortical carcinoma]] shows:
**Central tumor necrosis
**[[Central]] [[tumor]] [[necrosis]]
**Calcifications
**[[Calcification|Calcifications]]
**Larger and more heterogeneous tumor
**Larger and more [[heterogeneous]] [[tumor]]
*CT scan of pheochromocytoma shows hypervascularity and marked enhancement after IV contrast administration
*[[CT scan]] of [[pheochromocytoma]] shows hypervascularity and marked [[Enhancer|enhancement]] after [[IV]] [[contrast]] administration
|-
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''MRI'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Magnetic resonance imaging|MRI]]'''
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*MRI like CT scan helps to determine tumor site, extent of tumor invasion, distant metastasis, its differentiation from other tumors, and local tumor recurrence
*[[Magnetic resonance imaging|MRI]] like [[Ct scan|CT scan]] helps to determine [[tumor]] site, [[Extent of reaction|extent]] of [[tumor]] [[invasion]], [[Distance matrix|distant]] [[metastasis]], its [[differentiation]] from other [[tumors]], and [[local]] [[tumor]] [[Recurrence plot|recurrence]]
*Adrenocortical carcinoma appears as a large heterogeneous mass with low fat content on MRI
*[[Adrenocortical carcinoma]] [[Appearance|appears]] as a [[Large-print|large]] [[heterogeneous]] [[mass]] with low [[fat]] [[Content validity|content]] on [[Magnetic resonance imaging|MRI]]
*CT scan of pheochromocytoma shows hypervascularity and marked enhancement after IV contrast administration
*[[Computed tomography|CT scan]] of [[pheochromocytoma]] shows hypervascularity and marked enhancement after [[IV]] [[contrast]] administration
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''Bone scan'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Bone scan]]'''
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* Bone scan is performed routinely to look for bone metastasis
*[[Bone scan]] is [[Performance status|performed]] routinely to [[Lookahead|look]] for [[bone metastasis]]
|-
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''PET scan'''<ref name="pmid19190108">{{cite journal| author=Groussin L, Bonardel G, Silvéra S, Tissier F, Coste J, Abiven G et al.| title=18F-Fluorodeoxyglucose positron emission tomography for the diagnosis of adrenocortical tumors: a prospective study in 77 operated patients. | journal=J Clin Endocrinol Metab | year= 2009 | volume= 94 | issue= 5 | pages= 1713-22 | pmid=19190108 | doi=10.1210/jc.2008-2302 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19190108  }} </ref><ref name="pmid12634969">{{cite journal| author=Khan TS, Sundin A, Juhlin C, Långström B, Bergström M, Eriksson B| title=11C-metomidate PET imaging of adrenocortical cancer. | journal=Eur J Nucl Med Mol Imaging | year= 2003 | volume= 30 | issue= 3 | pages= 403-10 | pmid=12634969 | doi=10.1007/s00259-002-1025-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12634969  }} </ref><ref name="DongCui2014">{{cite journal|last1=Dong|first1=Aisheng|last2=Cui|first2=Yong|last3=Wang|first3=Yang|last4=Zuo|first4=Changjing|last5=Bai|first5=Yushu|title=18F-FDG PET/CT of Adrenal Lesions|journal=American Journal of Roentgenology|volume=203|issue=2|year=2014|pages=245–252|issn=0361-803X|doi=10.2214/AJR.13.11793}}</ref><ref name="pmid10405717">{{cite journal| author=Shulkin BL, Thompson NW, Shapiro B, Francis IR, Sisson JC| title=Pheochromocytomas: imaging with 2-[fluorine-18]fluoro-2-deoxy-D-glucose PET. | journal=Radiology | year= 1999 | volume= 212 | issue= 1 | pages= 35-41 | pmid=10405717 | doi=10.1148/radiology.212.1.r99jl3035 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10405717  }} </ref><ref name="pmid20490488">{{cite journal| author=Ansquer C, Scigliano S, Mirallié E, Taïeb D, Brunaud L, Sebag F et al.| title=18F-FDG PET/CT in the characterization and surgical decision concerning adrenal masses: a prospective multicentre evaluation. | journal=Eur J Nucl Med Mol Imaging | year= 2010 | volume= 37 | issue= 9 | pages= 1669-78 | pmid=20490488 | doi=10.1007/s00259-010-1471-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20490488  }} </ref><ref name="pmid16505394">{{cite journal| author=Blake MA, Slattery JM, Kalra MK, Halpern EF, Fischman AJ, Mueller PR et al.| title=Adrenal lesions: characterization with fused PET/CT image in patients with proved or suspected malignancy--initial experience. | journal=Radiology | year= 2006 | volume= 238 | issue= 3 | pages= 970-7 | pmid=16505394 | doi=10.1148/radiol.2383042164 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16505394  }} </ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Positron emission tomography|PET scan]]'''<ref name="pmid19190108">{{cite journal| author=Groussin L, Bonardel G, Silvéra S, Tissier F, Coste J, Abiven G et al.| title=18F-Fluorodeoxyglucose positron emission tomography for the diagnosis of adrenocortical tumors: a prospective study in 77 operated patients. | journal=J Clin Endocrinol Metab | year= 2009 | volume= 94 | issue= 5 | pages= 1713-22 | pmid=19190108 | doi=10.1210/jc.2008-2302 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19190108  }} </ref><ref name="pmid12634969">{{cite journal| author=Khan TS, Sundin A, Juhlin C, Långström B, Bergström M, Eriksson B| title=11C-metomidate PET imaging of adrenocortical cancer. | journal=Eur J Nucl Med Mol Imaging | year= 2003 | volume= 30 | issue= 3 | pages= 403-10 | pmid=12634969 | doi=10.1007/s00259-002-1025-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12634969  }} </ref><ref name="DongCui2014">{{cite journal|last1=Dong|first1=Aisheng|last2=Cui|first2=Yong|last3=Wang|first3=Yang|last4=Zuo|first4=Changjing|last5=Bai|first5=Yushu|title=18F-FDG PET/CT of Adrenal Lesions|journal=American Journal of Roentgenology|volume=203|issue=2|year=2014|pages=245–252|issn=0361-803X|doi=10.2214/AJR.13.11793}}</ref><ref name="pmid10405717">{{cite journal| author=Shulkin BL, Thompson NW, Shapiro B, Francis IR, Sisson JC| title=Pheochromocytomas: imaging with 2-[fluorine-18]fluoro-2-deoxy-D-glucose PET. | journal=Radiology | year= 1999 | volume= 212 | issue= 1 | pages= 35-41 | pmid=10405717 | doi=10.1148/radiology.212.1.r99jl3035 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10405717  }} </ref><ref name="pmid20490488">{{cite journal| author=Ansquer C, Scigliano S, Mirallié E, Taïeb D, Brunaud L, Sebag F et al.| title=18F-FDG PET/CT in the characterization and surgical decision concerning adrenal masses: a prospective multicentre evaluation. | journal=Eur J Nucl Med Mol Imaging | year= 2010 | volume= 37 | issue= 9 | pages= 1669-78 | pmid=20490488 | doi=10.1007/s00259-010-1471-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20490488  }} </ref><ref name="pmid16505394">{{cite journal| author=Blake MA, Slattery JM, Kalra MK, Halpern EF, Fischman AJ, Mueller PR et al.| title=Adrenal lesions: characterization with fused PET/CT image in patients with proved or suspected malignancy--initial experience. | journal=Radiology | year= 2006 | volume= 238 | issue= 3 | pages= 970-7 | pmid=16505394 | doi=10.1148/radiol.2383042164 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16505394  }} </ref>
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*2-[fluorine-18]fluoro-2-deoxy-d-glucose (FDG) PET scan is helpful in differentiating benign from malignant tumors/lesions as there's increased FDG uptake by malignant lesions comparative to benign lesions.
*[[FDG-PET|2-[fluorine-18]fluoro-2-deoxy-d-glucose (FDG) PET scan]] is helpful in [[Differentiate|differentiating]] [[benign]] from [[malignant tumors]]/[[lesions]] as there's increased [[Fluorodeoxyglucose|FDG]] [[Uptake signal sequence|uptake]] by [[malignant]] [[lesions]] [[Comparative anatomy|comparative]] to [[benign]] [[lesions]].
*FDG PET scan is especially very useful in defining the distribution of those pheochromocytomas that fail to concentrate MIBG.
*[[FDG-PET|FDG PET scan]] is especially very useful in defining the [[Distribution (pharmacology)|distribution]] of those [[Pheochromocytoma|pheochromocytomas]] that [[Failure|fail]] to [[concentrate]] [[Metaiodobenzylguanidine|MIBG]].
*FDG PET scan is important in making the surgical decision as unnecessary removal of benign adrenal lesions can be avoided in case of the absence of FDG uptake without any prior history of poorly FDG-avid cancer.
*[[FDG-PET|FDG PET scan]] is important in [[MakeBot|making]] the [[Surgery|surgical]] [[decision]] as [[Unnecessary Fuss|unnecessary]] removal of [[benign]] [[Adrenal Gland|adrenal]] [[lesions]] can be [[Avoidance response|avoided]] in [[Case-based reasoning|case]] of the absence of [[FDG]] [[Uptake signal sequence|uptake]] without any prior [[History and Physical examination|history]] of poorly [[FDG]]-[[Avidity|avid]] [[cancer]].
|-
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''123I-metaiodobenzylguanidine SPECT'''<ref name="pmid10405717" />
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''123I-[[metaiodobenzylguanidine]] [[Single photon emission computed tomography|SPECT]]'''<ref name="pmid10405717" />
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* 123I-metaiodobenzylguanidine SPECT (MIBG scintigraphy) shows greater accumulation in well-differentiated tumors than in less-well-differentiated tumors in case of pheochromocytoma.
* 123I-[[metaiodobenzylguanidine]] [[SPECT]] ([[Metaiodobenzylguanidine|MIBG]] [[scintigraphy]]) shows greater accumulation in well-[[Differentiate|differentiated]] [[tumors]] than in less-well-[[Differentiate|differentiated]] [[tumors]] in [[Case-based reasoning|case]] of [[pheochromocytoma]].
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''Molecular imaging'''<ref name="pmid18397978">{{cite journal| author=Hahner S, Stuermer A, Kreissl M, Reiners C, Fassnacht M, Haenscheid H et al.| title=[123 I]Iodometomidate for molecular imaging of adrenocortical cytochrome P450 family 11B enzymes. | journal=J Clin Endocrinol Metab | year= 2008 | volume= 93 | issue= 6 | pages= 2358-65 | pmid=18397978 | doi=10.1210/jc.2008-0050 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18397978  }} </ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Molecular imaging]]'''<ref name="pmid18397978">{{cite journal| author=Hahner S, Stuermer A, Kreissl M, Reiners C, Fassnacht M, Haenscheid H et al.| title=[123 I]Iodometomidate for molecular imaging of adrenocortical cytochrome P450 family 11B enzymes. | journal=J Clin Endocrinol Metab | year= 2008 | volume= 93 | issue= 6 | pages= 2358-65 | pmid=18397978 | doi=10.1210/jc.2008-0050 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18397978  }} </ref>
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* 123Iodometomidate (IMTO) is a highly specific radiotracer for imaging of adrenocortical tissue as it provides the molecular imaging of cytochrome P450 family of adrenal 11B (Cyp11B) enzymes.
* 123Iodometomidate (IMTO) is a highly [[Specific activity|specific]] [[radiotracer]] for [[imaging]] of [[adrenocortical]] [[Tissue (biology)|tissue]] as it provides the [[molecular imaging]] of [[cytochrome P450]] [[family]] of [[adrenal]] 11B (Cyp11B) [[enzymes]].
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''Adrenal angiography'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Adrenal]] [[angiography]]'''
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* In adrenal angiography, a contrast dye is injected and a series of X-ray images are taken afterwards to look for any block in adrenal arteries.
* In [[Adrenal gland|adrenal]] [[angiography]], a [[contrast]] [[dye]] is [[injected]] and a series of [[X-ray]] [[images]] are taken afterwards to [[Lookahead|look]] for any [[Blockhead|block]] in [[Adrenal artery|adrenal arteries]].
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''Adrenal venography'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Adrenal]] [[venography]]'''
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* In adrenal venography, a contrast dye is injected and a series of X-ray images are taken afterwards to look for any block in adrenal veins and to check for any abnormal hormonal levels (by inserting a catheter in adrenal veins).
* In [[adrenal]] [[venography]], a [[contrast]] [[dye]] is [[injected]] and a series of [[X-ray]] [[images]] are taken afterwards to [[Lookahead|look]] for any [[Blocking (statistics)|block]] in [[adrenal]] [[veins]] and to [[check]] for any [[abnormal]] [[hormonal]] levels (by [[Insert|inserting]] a [[catheter]] in [[Adrenal gland|adrenal]] [[veins]]).
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''Ultrasound exam'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Ultrasound]] [[Examination|exam]]'''
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* After surgery, ultrasound is done to look for any recurrence or involvement of the surrounding organs.
* After [[surgery]], [[ultrasound]] is [[done]] to [[Lookahead|look]] for any [[Recurrence plot|recurrence]] or involvement of the surrounding [[Organ (anatomy)|organs.]]
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===Biopsy===
===Biopsy===
*Two types of biopsies can be done for confirming the diagnosis of adrenal carcinoma:
*Two types of [[biopsies]] can be [[done]] for [[Confirmatory factor analysis|confirming]] the [[diagnosis]] of [[adrenal]] [[carcinoma]]:
**FNAC
**[[FNA|FNAC]]
**Core biopsy
**[[Core (anatomy)|Core]] [[biopsy]]


===Adrenalectomy===
===Adrenalectomy===
*After surgery, a part of adrenal gland is removed and viewed under microscope to look for any local recurrence or any metastasis to surrounding organs or distally.
*After [[surgery]], a part of the [[adrenal gland]] is removed and viewed under [[microscope]] to [[Lookahead|look]] for any [[local]] [[Recurrence plot|recurrence]] or any [[metastasis]] to the surrounding [[organs]] or [[Distal|distally]].


==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
*Different treatment options for the medical therapy of adrenal carcinoma are given in the table below:<ref name="urlAdrenocortical Carcinoma Treatment (PDQ®)–Patient Version - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq |title=Adrenocortical Carcinoma Treatment (PDQ®)–Patient Version - National Cancer Institute |format= |work= |accessdate=}}</ref>
*Different [[Treatments|treatment]] options for the [[Medical therapy template|medical therapy]] of [[adrenal]] [[carcinoma]] are given in the table below:<ref name="urlAdrenocortical Carcinoma Treatment (PDQ®)–Patient Version - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq |title=Adrenocortical Carcinoma Treatment (PDQ®)–Patient Version - National Cancer Institute |format= |work= |accessdate=}}</ref>


{| class="wikitable"
{| class="wikitable"
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|-
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Radiation therapy|'''Radiation therapy''']]
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Radiation therapy|'''Radiation therapy''']]
|There are following two types of radiation therapies that can be used for the treatment of adrenal carcinoma:
|There are following two types of [[Radiation therapy|radiation therapies]] that can be used for the [[Treatments|treatment]] of [[adrenal]] [[carcinoma]]:


* '''[[External beam radiation therapy|External beam radiation therapy:]]'''
*'''[[External beam radiation therapy|External beam radiation therapy:]]'''
** It uses a machine outside the body which sends radiations towards the cancer
** It uses a machine outside the [[Human body|body]] which sends [[Radiation|radiations]] towards the [[cancer cells]].
* '''Internal beam radiation therapy:'''
*'''Internal [[Beam divergence|beam]] [[radiation therapy]]:'''
** It uses a radioactive substance which is sealed in needles, wires, seeds, or catheters and are placed directly into or near the cancer.
** It uses a [[radioactive]] [[substance]] which is [[Sealguard|sealed]] in [[Needle|needles]], [[Wire|wires]], [[Seed|seeds]], or [[catheters]] and are placed directly into or near the [[cancer cells]].
|-
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Radiofrequency ablation|'''Radiofrequency ablation''']]
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Radiofrequency ablation|'''Radiofrequency ablation''']]
|
|
* A localized treatment which uses high-energy radio waves to heat & destroy the cancer cells.
* A [[Local|localized]] [[Treatments|treatment]] which uses high-[[energy]] [[Radio-frequency|radio]] [[waves]] to [[heat]] & [[Destroying angel|destroy]] the [[cancer cells]].
* May be used for [[Palliative care|palliation]] in patients who are not surgical candidates.
* May be used for [[Palliative care|palliation]] in [[patients]] who are not [[Surgery|surgical]] [[Candidate gene|candidates]].
|-
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Chemotherapy]]''' ('''[[chemoembolization]]''')<ref name="pmid30918109">{{cite journal| author=Kwok GTY, Zhao JT, Glover AR, Gill AJ, Clifton-Bligh R, Robinson BG et al.| title=microRNA-431 as a Chemosensitizer and Potentiator of Drug Activity in Adrenocortical Carcinoma. | journal=Oncologist | year= 2019 | volume= 24 | issue= 6 | pages= e241-e250 | pmid=30918109 | doi=10.1634/theoncologist.2018-0849 | pmc=6656493 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30918109  }} </ref><ref name="pmid12015757">{{cite journal| author=Abraham J, Bakke S, Rutt A, Meadows B, Merino M, Alexander R et al.| title=A phase II trial of combination chemotherapy and surgical resection for the treatment of metastatic adrenocortical carcinoma: continuous infusion doxorubicin, vincristine, and etoposide with daily mitotane as a P-glycoprotein antagonist. | journal=Cancer | year= 2002 | volume= 94 | issue= 9 | pages= 2333-43 | pmid=12015757 | doi=10.1002/cncr.10487 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12015757  }} </ref><ref name="pmid9827725">{{cite journal| author=Berruti A, Terzolo M, Pia A, Angeli A, Dogliotti L| title=Mitotane associated with etoposide, doxorubicin, and cisplatin in the treatment of advanced adrenocortical carcinoma. Italian Group for the Study of Adrenal Cancer. | journal=Cancer | year= 1998 | volume= 83 | issue= 10 | pages= 2194-200 | pmid=9827725 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9827725  }} </ref><ref name="pmid10699907">{{cite journal| author=Williamson SK, Lew D, Miller GJ, Balcerzak SP, Baker LH, Crawford ED| title=Phase II evaluation of cisplatin and etoposide followed by mitotane at disease progression in patients with locally advanced or metastatic adrenocortical carcinoma: a Southwest Oncology Group Study. | journal=Cancer | year= 2000 | volume= 88 | issue= 5 | pages= 1159-65 | pmid=10699907 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10699907  }} </ref>
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Chemotherapy]]''' ('''[[chemoembolization]]''')<ref name="pmid30918109">{{cite journal| author=Kwok GTY, Zhao JT, Glover AR, Gill AJ, Clifton-Bligh R, Robinson BG et al.| title=microRNA-431 as a Chemosensitizer and Potentiator of Drug Activity in Adrenocortical Carcinoma. | journal=Oncologist | year= 2019 | volume= 24 | issue= 6 | pages= e241-e250 | pmid=30918109 | doi=10.1634/theoncologist.2018-0849 | pmc=6656493 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30918109  }} </ref><ref name="pmid12015757">{{cite journal| author=Abraham J, Bakke S, Rutt A, Meadows B, Merino M, Alexander R et al.| title=A phase II trial of combination chemotherapy and surgical resection for the treatment of metastatic adrenocortical carcinoma: continuous infusion doxorubicin, vincristine, and etoposide with daily mitotane as a P-glycoprotein antagonist. | journal=Cancer | year= 2002 | volume= 94 | issue= 9 | pages= 2333-43 | pmid=12015757 | doi=10.1002/cncr.10487 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12015757  }} </ref><ref name="pmid9827725">{{cite journal| author=Berruti A, Terzolo M, Pia A, Angeli A, Dogliotti L| title=Mitotane associated with etoposide, doxorubicin, and cisplatin in the treatment of advanced adrenocortical carcinoma. Italian Group for the Study of Adrenal Cancer. | journal=Cancer | year= 1998 | volume= 83 | issue= 10 | pages= 2194-200 | pmid=9827725 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9827725  }} </ref><ref name="pmid10699907">{{cite journal| author=Williamson SK, Lew D, Miller GJ, Balcerzak SP, Baker LH, Crawford ED| title=Phase II evaluation of cisplatin and etoposide followed by mitotane at disease progression in patients with locally advanced or metastatic adrenocortical carcinoma: a Southwest Oncology Group Study. | journal=Cancer | year= 2000 | volume= 88 | issue= 5 | pages= 1159-65 | pmid=10699907 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10699907  }} </ref>
|
|
* 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.
*[[Chemotherapy regimens]] [[Typical set|typically]] include the [[drug]] [[mitotane]], an [[inhibitor]] of [[steroid]] [[synthesis]] which is [[toxic]] to the [[Cells (biology)|cells]] of [[adrenal cortex]], as well as [[standard]] [[cytotoxic drugs]].
* One widely used regimen consists of [[cisplatin]], [[doxorubicin]], [[etoposide]] and mitotane.
* One [[Wide and fast|widely]] used regimen consists of [[cisplatin]], [[doxorubicin]], [[etoposide]] and [[mitotane]].
* The endocrine cell toxin [[streptozotocin]] has also been included in some treatment protocols.
* The [[endocrine]] [[Cell (biology)|cell]] [[toxin]] [[streptozotocin]] has also been included in some [[Treatments|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]]).
*[[Chemotherapy]] may be given to the [[patients]] with unresectable [[disease]], to shrink the [[tumor]] prior to [[surgery]] ([[neoadjuvant chemotherapy]]), or in an attempt to [[Elimination reaction|eliminate]] [[microscopic]] [[residual]] [[disease]] [[after surgery]] ([[adjuvant chemotherapy]]).
|-
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Hormonal therapy (oncology)|'''Hormonal therapy''']]  
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Hormonal therapy (oncology)|'''Hormonal therapy''']]  
|
|
* Steroid synthesis inhibitors such as [[aminoglutethimide]] may be used in a palliative manner to reduce the symptoms of hormonal syndromes.
*[[Steroid]] [[synthesis]] [[Inhibitor|inhibitors]] such as [[aminoglutethimide]] may be used in a [[palliative]] manner to [[Reduced|reduce]] the [[symptoms]] of [[hormonal]] [[syndromes]].
|-
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Biological therapy]]''' ('''[[immunotherapy]]''')
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Biological therapy]]''' ('''[[immunotherapy]]''')
|
|
* Biotherapy uses patient's own immune system to fight against the cancer.
*[[Biotherapy]] uses [[Patient|patient's]] own [[immune system]] to fight against the [[cancer]].
* Substances made by the human body or made in the laboratory are used to boost, direct, or restore the body's natural defenses against cancer.
*[[Substance|Substances]] made by the [[human body]] or made in the [[laboratory]] are used to [[Boosting|boost]], direct, or restore the [[Human body|body's]] [[Natural health|natural]] [[Defense Physiology|defenses]] against [[cancer]].
|-
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Targeted therapy]]'''
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[Targeted therapy]]'''
|
|
* Uses drugs or other substances in order to identify and attack specific cancer cells without providing any harm to the normal cells.
* Uses [[drugs]] or other [[Substance|substances]] such as [[Steroidogenic factor 1|Steroidogenic factor (SF)-1]] [[antagonist]] [[therapy]] in order to identify and [[Attack therapy|attack]] [[Specific activity|specific]] [[cancer cells]] without providing any harm to the [[normal]] [[Cells (biology)|cells]].
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |'''[[mTOR]] [[antagonists]]'''
|
*[[Temsirolimus]] ([[second]]-[[generation]] [[mTOR]] [[Inhibitor|inhibitor)]] in [[Combination therapy|combination]] with cixutumumab (anti-[[Insulin-like growth factor 1 receptor|IGF-1R]] [[antibody]]) can be used in [[Treatments|treating]] [[adrenocortical carcinoma]].
|}
|}


===Surgery===
===Surgery===
* 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 only [[Cure|curative]] [[Treatments|treatment]] is complete [[Surgery|surgical]] [[excision]] of the [[tumor]], which can be [[Performance status|performed]] even in the [[Case-based reasoning|case]] of [[invasion]] into large [[blood vessels]], such as the [[renal vein]] or [[inferior vena cava]].
*Goal of the surgery is R0 tumor resection and removal of any involved tissues or viscera in an en bloc fashion.
*[[Goal-directed therapy|Goal]] of the [[surgery]] is R0 [[tumor]] [[resection]] and removal of any involved [[tissues]] or [[viscera]] in an en bloc fashion.
*Feasibility of the surgical resection in a newly diagnosed case of adrenocortical carcinoma is the most important contributor to the overall survival.  
*Feasibility of the [[surgical resection]] in a [[New|newly]] [[Diagnose|diagnosed]] [[Case-based reasoning|case]] of [[adrenocortical carcinoma]] is the most important contributor to the overall [[Survival analysis|survival]].
*Complete surgical resection of adrenocortical carcinoma is necessary, if possible for the patients presenting with stage I to stage III disease.
*Complete [[surgical resection]] of [[adrenocortical carcinoma]] is [[Necessary and sufficient|necessary]], if [[Possibility theory|possible]] for the [[patients]] [[Presenting symptom|presenting]] with stage I to stage III [[disease]].
*Patients undergoing a successful resection have a five-year survival of 50%-60%, however, a large percentage of patients are not surgical candidates unfortunately
*[[Patients]] undergoing a successful [[resection]] have a [[Five-year survival rate|five-year survival]] of 50%-60%, however, a large [[percentage]] of [[patients]] are not [[Surgery|surgical]] [[Candidate gene|candidates]] unfortunately
*Median survival of unresectable patients is less than one year
*[[Median]] [[Survival analysis|survival]] of unresectable [[patients]] is less than one [[year]]
*5-year disease-specific survival stratified according to the stage of the disease (ACC) at the time of diagnosis is given below:
*5-[[year]] [[disease]]-[[Specific activity|specific]] [[Survival analysis|survival]] [[stratified]] according to the stage of the [[disease]] (ACC) at the [[Time constant|time]] of [[diagnosis]] is given below:


{| class="wikitable"
{| class="wikitable"
|+5-year disease-specific survival rate stratification in relation to the disease stage at the time of ACC tumor resection
|+5-year disease-specific survival rate stratification in relation to the disease stage at the time of ACC tumor resection
!Stage of the disease at the time of tumor resection
!style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Stage of the disease at the time of tumor resection}}
!5-year disease-specific survival
!style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|5-year disease-specific survival}}
|-
|-
|Stage I disease
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Stage I  
|82%
|82%
|-
|-
|Stage II
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Stage II
|58%
|58%
|-
|-
|Stage III
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Stage III
|55%
|55%
|-
|-
|Stage IV
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Stage IV
|18%
|18%
|}
|}


* The 2004, International Union Against Cancer (UICC) and the World Health Organization (WHO) proposed new staging system based on Sullivan-McFarlane criteria for adrenocortical carcinoma (ACC) is given in the table below and is used to make the surgical decision about the tumor resection:
* The 2004, [[International Union Against Cancer]] ([[International Union Against Cancer|UICC]]) and [[World Health Organization]] ([[World Health Organization|WHO]]) [[Proposition|proposed]] [[new]] [[Cancer staging|staging]] [[system]] [[Base|based]] on Sullivan-McFarlane [[criteria]] for [[adrenocortical carcinoma]] ([[Adrenocortical carcinoma|ACC]]) is given in the table below and is used to make the [[Surgery|surgical]] [[decision]] about the [[tumor]] [[resection]]:


{| class="wikitable"
{| class="wikitable"
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|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |I
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |I
|
|
* T1, N0, M0
*[[T1]], N0, M0
|
|
* T1, N0, M0
*[[T1]], N0, M0
|-
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |II
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |II
Line 579: Line 597:
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |III
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |III
|
|
* T1-2, N1, M0
*[[T1]]-2, N1, M0
* T3, N0, M0
*[[T3]], N0, M0
|
|
* T1-2, N1, M0
*[[T1]]-2, N1, M0
* T3-4, N0-1, M0
*[[T3]]-4, N0-1, M0
|-
|-
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |IV
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |IV
|
|
* T1–4, N0-1, M1
*[[T1]]–4, N0-1, M1
* T3, N1, M0
*[[T3]], N1, M0
* T4, N0-1, M0
*[[T4]], N0-1, M0
|
|
* T1–4, N0-1, M1
*[[T1]]–4, N0-1, M1
|}
|}
T1: tumor  5 cm; T2: tumor  5 cm; T3: tumor infiltration in surrounding tissue; T4: tumor infiltration in adjacent organs [ENSAT additionally the presence of a tumor thrombus in the Vena Cava or Vena Renalis]; N0: absence of positive lymph nodes; N1: presence of positive lymph nodes; M0: absence of distant metastases; M1: presence of distant metastasis.<br />
[[T1]]: [[tumor]]  5 [[Centimetre|cm]]; T2: [[tumor]]  5 [[Centimetre|cm]]; [[T3]]: [[tumor]] [[Infiltration (medical)|infiltration]] in surrounding [[Tissue (biology)|tissue]]; [[T4]]: [[tumor]] [[Infiltration (medical)|infiltration]] in adjacent [[organs]] [ENSAT additionally the presence of a [[tumor]] [[thrombus]] in the [[Inferior vena cava|Vena Cava]] or Vena Renalis]; N0: absence of positive [[lymph nodes]]; N1: presence of positive [[lymph nodes]]; M0: absence of [[Distance matrix|distant]] [[metastases]]; M1: presence of [[Distance matrix|distant]] [[metastasis]].<br />


== Differentiating Adrenal carcinoma from other Diseases==
== Differentiating Adrenal carcinoma from other Diseases==

Latest revision as of 20:53, 19 August 2020

For patient information, click here

Adrenal Carcinoma Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Synonyms and keywords: Adrenocortical carcinoma, Adrenal cortical carcinoma, Adrenal cortical cancer, Adrenal cortex cancer, Adrenal cancer, Adrenal tumor, Neuroblastoma, Pheochromocytoma, Ganglioneuroma, Adrenocortical adenoma, Adenomatoid tumor, Myelolipoma, Schwannoma.

Overview

Adrenal carcinoma or adrenal tumor is an aggressive disease which can originate either in the cortex (steroid hormone-producing tissue) or medulla of the adrenal gland. According to the 2017 WHO classification of adrenal tumors, adrenal cortical tumors are subclassified into cortical adenoma, cortical carcinoma, sex cord stromal tumors, adenomatoid tumor, mesenchymal and stromal tumors (myelolipoma, schwannoma), hematological tumors and secondary tumors, whereas tumors of adrenal medulla are subclassified into pheochromocytoma, paraganglioma, neuroblastic tumors, composite pheochromocytoma, and composite paraganglioma. Pathogenesis includes many genetic pathways, most prominent being Wnt-Beta catenin pathway and also association with other diseases such as multiple endocrine neoplasia (MEN1 and MEN2), familial adenomatous polyposis, Beckwith-Wiedemann syndrome, Li-Fraumeni syndrome, Lynch syndrome,von Hippel-Lindau disease, carney Complex/Syndrome, neurofibromatosis type 1 and congenital adrenal hyperplasia. Adrenocortical 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. Adrenal carcinoma can be treated with both medical therapy and surgery depending upon the stage of the tumor.

Historical perspective

Classification

Updated 2017 WHO classification of adrenal tumors
Tumors of adrenal gland
Tumors of adrenal cortex
Tumors of the adrenal medulla

and extra-adrenal paraganglia

Pathophysiology

Normal anatomy and physiology of adrenal glands

Normal anatomy and function of Adrenal glands
Adrenal gland layers Functions
Adrenal cortex (outer layer)
Adrenal medulla (Inner layer)
source: By David Richfield (User:Slashme) and Mikael Häggström. Derived from previous version by Hoffmeier and Settersr.In external use, this diagram may be cited as:Häggström M, Richfield D (2014). "Diagram of the pathways of human steroidogenesis". Wikiversity Journal of Medicine 1 (1). DOI:10.15347/wjm/2014.005. ISSN 20018762. - Self-made using bkchem and inkscape, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=6355511

{{#ev:youtube|https://www.youtube.com/watch?v=JlI5N2N4d-k}}

Epigenetics

VEGF signaling, source: By Mikael Häggström.When using this image in external works, it may be cited as:Häggström, Mikael (2014). "Medical gallery of Mikael Häggström 2014". WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.008. ISSN 2002-4436. Public Domain.orBy Mikael Häggström, used with permission. - [1]Interactions of VEGF ligands and VEGF receptors ResearchVEGF.com, retrieved on November, 13, 2009, Public Domain, https://commons.wikimedia.org/w/index.php?curid=3475250
WNT pathwayssource: By Fred the OysteriThe source code of this SVG is valid.This vector graphics image was created with Adobe Illustrator., GFDL, https://commons.wikimedia.org/w/index.php?curid=36340188
microRNA function, source: By Kelvinsong - Own work, CC BY 3.0, https://commons.wikimedia.org/w/index.php?curid=23311105

Gross pathology

Adrenocortical carcinoma

A large adrenal cortical carcinoma resected from a 27-year-old woman. The tumor measured 17 cm in diameter and invaded kidney and spleen which necessitated en bloc removal of these organs with the tumor. - By AFIP Atlas of Tumor Pathology - [1], Domena publiczna, https://commons.wikimedia.org/w/index.php?curid=6719487

Pheochromocytoma

Bilateral pheochromocytoma in MEN2. Gross image. Source: https://upload.wikimedia.org/wikipedia/commons/5/5f/Bilateral_pheo_MEN2.jpg

Microscopic pathology

Pathologic criterias for adrenocortical carcinoma

Pathologic criteria Details Age applicability
Weiss criteria

Adrenocortical carcinoma can be diagnosed by the presence of at least 3 of the 9 Weiss criteria:

  1. High nuclear grade (III or IV)
  2. High mitotic rate i.e. presence of >5 mitotic figures/50 high-power fields, definition suffers from HPFitis (counting 10 random fields in area of greatest number of mitotic figures on 5 slides with the greatest number of mitosis)
  3. Atypical mitoses (abnormal distribution of chromosomes or excessive number of mitotic spindles)
  4. Cleared or vacuolated cytoplasm in >/= 25% of the tumor cells
  5. Sheeting (diffuse architecture of patternless sheets of cells)) in >= 1/3 of tumor cells
  6. Necrosis in nests (microscopic necrosis)
  7. Venous invasion (veins must have smooth muscles in wall; tumor cell clusters or sheets forming polypoid projections into the vessel lumen or polypoid tumor thrombi covered by endothelial layer)
  8. Adrenal sinusoid invasion (sinusoid is endothelial lined vessel in adrenal gland with little supportive tissue; consider only sinusoids within tumor)
  9. Capsular invasion (nests or cords of tumor extending into or through capsule with a stromal reaction); either incomplete or complete)

Modified Weiss criteria (score of 3 or more suggests malignancy):

 Adults
Volante criteria

Simplified criteria by Volante et al (not widely used) is as follows:

Wieneke et al and Dehner & Hill

Wieneke et al and Dehner & Hill proposed the following very simple system:

Pediatrics
Micrograph of an adrenocortical carcinoma (left of image - dark blue) and the adrenal cortex it arose from (right-top of image - pink/light blue). Benign adrenal medulla is present (right-middle of image - gray/blue). H&E stain. - Source: https://librepathology.org
Micrograph of pheochromocytoma. Source: By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=5938524
Histopathology of adrenal pheochromocytoma. Adrenectomy specimen. Source: CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=535945
Micrograph of pheochromocytoma. Source: CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=535944

{{#ev:youtube|7jMFENhPaOM}}

{{#ev:youtube|7yjxG3KmX98}}

Epidemiology and demographics

Risk factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Symptoms in children

{{#ev:youtube|https://www.youtube.com/watch?v=ea1sXgd5ui8&t=697s}}

Symptoms in adults

Presentation of non-functional adrenal carcinoma

Physical Examination

Laboratory findings

Laboratory findings of different hormonal syndromes in adrenal carcinoma
Hormonal syndrome Laboratory findings
Cushing syndrome
Virilization
Conn syndrome
Feminization

Imaging studies

Imaging studies for the diagnosis of adrenal carcinoma
Imaging study Details
CT[46]
MRI
Bone scan
PET scan[47][48][49][50][51][52]
123I-metaiodobenzylguanidine SPECT[50]
Molecular imaging[53]
Adrenal angiography
Adrenal venography
Ultrasound exam

Biopsy

Adrenalectomy

Treatment

Medical Therapy

Different types of medical therapies for treatment of adrenal carcinoma
Type of medical therapy Details
Radiation therapy There are following two types of radiation therapies that can be used for the treatment of adrenal carcinoma:
Radiofrequency ablation
Chemotherapy (chemoembolization)[55][56][57][58]
Hormonal therapy
Biological therapy (immunotherapy)
Targeted therapy
mTOR antagonists

Surgery

5-year disease-specific survival rate stratification in relation to the disease stage at the time of ACC tumor resection
Stage of the disease at the time of tumor resection 5-year disease-specific survival
Stage I 82%
Stage II 58%
Stage III 55%
Stage IV 18%
Comparison of UICC and ENSAT staging systems for adrenocortical carcinoma
Stage UICC/WHO 2004 ENSAT 2008
I
  • T1, N0, M0
  • T1, N0, M0
II
  • T2, N0, M0
  • T2, N0, M0
III
  • T1-2, N1, M0
  • T3, N0, M0
  • T1-2, N1, M0
  • T3-4, N0-1, M0
IV
  • T1–4, N0-1, M1
  • T3, N1, M0
  • T4, N0-1, M0
  • T1–4, N0-1, M1

T1: tumor  5 cm; T2: tumor  5 cm; T3: tumor infiltration in surrounding tissue; T4: tumor infiltration in adjacent organs [ENSAT additionally the presence of a tumor thrombus in the Vena Cava or Vena Renalis]; N0: absence of positive lymph nodes; N1: presence of positive lymph nodes; M0: absence of distant metastases; M1: presence of distant metastasis.

Differentiating Adrenal carcinoma from other Diseases

Bilateral

Unilateral

References

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