Adrenocortical carcinoma risk factors: Difference between revisions

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{{Adrenocortical carcinoma}}
{{Adrenocortical carcinoma}}
{{CMG}} {{AE}} {{AAM}}
{{CMG}}; {{AE}} {{AAM}} {{MAD}}
==Overview==
==Overview==
The most potent risk factors in the development of adrenocortical cancer are [[TP53| TP53 mutation]], [[Beckwith-Wiedemann syndrome]] and [[Carney complex]].<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq#section/_1</ref>
The most important risk factors for developing adrenocortical cancer are [[Lynch syndrome]][[Beckwith-Wiedemann syndrome|, Beckwith-Wiedemann syndrome,]] [[Carney complex|Carney complex,]] [[Neurofibromatosis type I|Neurofibromatosis type 1,]] [[Multiple endocrine neoplasia type 1]] ([[MEN1]]), and [[Carney complex]].
==Risk Factors==
==Risk Factors==
Risk factor associated with adrenocortical carcinoma are:
Risk factors associated with adrenocortical carcinoma are:
 
* [[Lynch syndrome]]<ref name="pmid26309352">{{cite journal| author=Carethers JM, Stoffel EM| title=Lynch syndrome and Lynch syndrome mimics: The growing complex landscape of hereditary colon cancer. | journal=World J Gastroenterol | year= 2015 | volume= 21 | issue= 31 | pages= 9253-61 | pmid=26309352 | doi=10.3748/wjg.v21.i31.9253 | pmc=4541378 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26309352  }}</ref>
*[[Li-Fraumeni syndrome]]
*[[Beckwith-Wiedemann syndrome]] ([[Beckwith-Wiedemann syndrome|BWS]])<ref name=":0" />
*[[Beckwith-Wiedemann syndrome]]
*[[Carney complex]]<ref name="pmid26130139">{{cite journal| author=Correa R, Salpea P, Stratakis CA| title=Carney complex: an update. | journal=Eur J Endocrinol | year= 2015 | volume= 173 | issue= 4 | pages= M85-97 | pmid=26130139 | doi=10.1530/EJE-15-0209 | pmc=4553126 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26130139  }}</ref>
*[[Carney complex]]
*[[Neurofibromatosis type I|Neurofibromatosis type 1]] <ref name="pmid250305152" />
*[[Adrenal gland|Adrenal]] incidentaloma must be differentiated from other diseases that cause adrenal masses such as adrenal [[adenoma]], [[adrenocortical carcinoma]], [[Cushing's syndrome]], [[pheochromocytoma]], and [[metastasis]].
*[[Multiple endocrine neoplasia type 1]]([[MEN1]])<ref name=":1" />
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! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Gene mutations}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Clinical picture}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Clinical picture}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Imagings}}
!Laboratory tests
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Adrenal adenoma
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Lynch syndrome]]<ref name="pmid26309352" />
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*  
* [[MSH2]], [[MSH6]], [[MLH1]], [[PMS2]]
|
* Round and homogeneous density, smooth contour and sharp margination [15]
* Diameter less than 4 cm, unilateral location
* Low unenhanced CT attenuation values (<10 HU) (image 1)
* Rapid contrast medium washout (10 minutes after administration of contrast, an absolute contrast medium washout of more than 50 percent)
* Isointensity with liver on both T1 and T2 weighted MRI sequences
* Chemical shift evidence of lipid on MRI
|
|
* [[Colorectal cancer]]
* [[Endometrial cancer]]
* [[Sebaceous gland carcinoma|Sebaceous neoplasms]]
* [[Ovarian cancer]]
* [[Pancreatic cancer]]
* [[Brain cancer]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Adrenocortica'''l carcinoma
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Neurofibromatosis type I|Neurofibromatosis type 1]] <ref name="pmid250305152">{{cite journal| author=Hirbe AC, Gutmann DH| title=Neurofibromatosis type 1: a multidisciplinary approach to care. | journal=Lancet Neurol | year= 2014 | volume= 13 | issue= 8 | pages= 834-43 | pmid=25030515 | doi=10.1016/S1474-4422(14)70063-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25030515 }}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
* Mass effect symptoms; symptoms related to  excess glucocorticoid, mineralocorticoid, androgen, or estrogen  secretion
|
|
* Irregular shape
* [[NF1]]
* Inhomogeneous density because of central areas of low attenuation due to tumor necrosis (image 4)
* Tumor calcification
* Diameter usually >4 cm
* Unilateral location
* High unenhanced CT attenuation values (>20 HU)
* Inhomogeneous enhancement on CT with intravenous contrast
* Delay in contrast medium washout (10 minutes after administration of contrast, an absolute contrast medium washout of less than 50 percent)
* Hypointensity compared with liver on T1 weighted MRI and high to intermediate signal intensity on T2 weighted MRI
* High standardized uptake value (SUV) on FDG-PET-CT study
* Evidence of local invasion or metastases
|
|
* Serum DHEAS
* [[Malignant]] [[Peripheral nervous system|peripheral nerve]] [[Nerve sheath|sheet]] [[tumor]]
* Measures of clinicallyindicated
* [[Pheochromocytoma]]
* steroid
* [[Café au lait spot|Café au lait spots]]
* [[Neurofibroma]]
* [[Optic nerve glioma|Optic glioma]]
* [[Lisch nodule]]
* Skeletal abnormalities
|-
|-
|Cushing's syndrome
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Multiple endocrine neoplasia type 1|MEN1]]<ref name=":1">{{Cite journal
|
 
* Rapid [[Obesity|weight gain]], particularly of the [[trunk]] and [[face]] with [[limbs]] sparing ([[central obesity]])
| author = [[B. Gatta-Cherifi]], [[O. Chabre]], [[A. Murat]], [[P. Niccoli]], [[C. Cardot-Bauters]], [[V. Rohmer]], [[J. Young]], [[B. Delemer]], [[H. Du Boullay]], [[M. F. Verger]], [[J. M. Kuhn]], [[J. L. Sadoul]], [[Ph Ruszniewski]], [[A. Beckers]], [[M. Monsaingeon]], [[E. Baudin]], [[P. Goudet]] & [[A. Tabarin]]
* Proximal [[muscle weakness]]
| title = Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des Tumeurs Endocrines database
* A [[round face]] often referred to as a "[[moon face]]"
| journal = [[European journal of endocrinology]]
* Excess [[sweating]]
| volume = 166
* [[Headache]]
| issue = 2
| pages = 269–279
| year = 2012
| month = February
| doi = 10.1530/EJE-11-0679
| pmid = 22084155
}}</ref>               
|
|
* Imaging may show adenoma if presents
* MENIN
|
|
* 24-hour urine [[cortisol]]
* [[Foregut]] [[neuroendocrine tumors]]
* Midnight salivary [[cortisol]]
* [[Pituitary tumors]]
* Low dose [[dexamethasone]] suppression test; high [[cortisol]] level after the [[dexamethasone]] test is suggestive of [[hypercortisolism]].
* [[Parathyroid gland|Parathyroid]] [[hyperplasia]]
* Collagenoma
* [[Angiofibroma]]
* [[Adrenal adenoma]]/[[hyperplasia]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Pheochromocytoma
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Carney complex]]<ref name="pmid26130139" />
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Palpitations]] especially in epinephrine producing tumors.<sup>[[Pheochromocytoma history and symptoms#cite note-pmid8325290-3|[3]]]</sup>
* [[Anxiety]] often resembling that of a [[panic attack]]
* [[Sweating]]
* [[Headaches]] occurs in 90 % of patients.
* Paroxysmal attacks of [[hypertension]] but some patients have normal [[blood pressure]].
* It may be [[asymptomatic]] and discovered incidentally after [[Screening (medicine)|screening]] for [[MEN, type 2|MEN]] patients.
|
|
* Increased attenuation on nonenhanced CT (>20 HU)
* [[PRKAR1A]]
* Increased mass vascularity (image 2)
* Delay in contrast medium washout (10 minutes after administration of contrast, an absolute contrast medium washout of less than 50 percent)
* High signal intensity on T2 weighted MRI (image 3)
* Cystic and hemorrhagic changes
* Variable size and may be bilateral
|
|
* [[Plasma]] fractionated [[Metanephrine|metanephrines]] 
* [[Adrenal disease]]
* 24-hour [[urinary]] fractionated [[Metanephrine|metanephrines]]
* [[Sertoli cell]] [[tumors]]
* [[Thyroid adenoma]]  
* [[Myxoma]]
* [[Somatotrope|Somatotroph]] [[pituitary adenoma]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Adrenal metastasis
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[BWS]]<ref name=":0">{{Cite journal
| style="padding: 5px 5px; background: #F5F5F5;" |
 
** Symptoms and signs of primary malignancy especially lung cancer.
| author = [[H. Segers]], [[R. Kersseboom]], [[M. Alders]], [[R. Pieters]], [[A. Wagner]] & [[M. M. van den Heuvel-Eibrink]]
** General constitutional symptoms:
| title = Frequency of WT1 and 11p15 constitutional aberrations and phenotypic correlation in childhood Wilms tumour patients
** Fever
| journal = [[European journal of cancer (Oxford, England : 1990)]]
** Fatigue
| volume = 48
** Weight loss
| issue = 17
| pages = 3249–3256
| year = 2012
| month = November
| doi = 10.1016/j.ejca.2012.06.008
| pmid = 22796116
}}</ref>
|
|
* Irregular shape and inhomogeneous nature
* [[IGF2]], [[CDKN1C]], [[H19 (gene)|H19]]
* Tendency to be bilateral
* High unenhanced CT attenuation values (>20 HU) and enhancement with intravenous contrast on CT
* Delay in contrast medium washout (10 minutes after administration of contrast, an absolute contrast medium washout of less than 50 percent)
* Isointensity or slightly less intense than the liver on T1 weighted MRI and high to intermediate signal intensity on T2 weighted MRI (representing an increased water content)
* Elevated standardized uptake value on FDG-PET scan
|
|
* [[Wilm's tumor|Wilm’s tumor]]
* [[Hepatoblastoma]]
* [[Macrosomia]]
* [[Adrenocortical]] cytomegaly
* [[Adrenal adenoma]]
* [[Adrenal Gland|Adrenal]] [[cyst]]
* [[Hemihypertrophy]]
* [[Macroglossia]]
* [[Omphalocele]]
|}
|}


Latest revision as of 15:39, 17 October 2017

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

Overview

The most important risk factors for developing adrenocortical cancer are Lynch syndrome, Beckwith-Wiedemann syndrome, Carney complex, Neurofibromatosis type 1, Multiple endocrine neoplasia type 1 (MEN1), and Carney complex.

Risk Factors

Risk factors associated with adrenocortical carcinoma are:

Differential Diagnosis Gene mutations Clinical picture
Lynch syndrome[1]
Neurofibromatosis type 1 [4]
MEN1[5]
  • MENIN
Carney complex[3]
BWS[2]

References

  1. ↑ 1.0 1.1 Carethers JM, Stoffel EM (2015). "Lynch syndrome and Lynch syndrome mimics: The growing complex landscape of hereditary colon cancer". World J Gastroenterol. 21 (31): 9253–61. doi:10.3748/wjg.v21.i31.9253. PMC 4541378. PMID 26309352.
  2. ↑ 2.0 2.1 H. Segers, R. Kersseboom, M. Alders, R. Pieters, A. Wagner & M. M. van den Heuvel-Eibrink (2012). "Frequency of WT1 and 11p15 constitutional aberrations and phenotypic correlation in childhood Wilms tumour patients". European journal of cancer (Oxford, England : 1990). 48 (17): 3249–3256. doi:10.1016/j.ejca.2012.06.008. PMID 22796116. Unknown parameter |month= ignored (help)
  3. ↑ 3.0 3.1 Correa R, Salpea P, Stratakis CA (2015). "Carney complex: an update". Eur J Endocrinol. 173 (4): M85–97. doi:10.1530/EJE-15-0209. PMC 4553126. PMID 26130139.
  4. ↑ 4.0 4.1 Hirbe AC, Gutmann DH (2014). "Neurofibromatosis type 1: a multidisciplinary approach to care". Lancet Neurol. 13 (8): 834–43. doi:10.1016/S1474-4422(14)70063-8. PMID 25030515.
  5. ↑ 5.0 5.1 B. Gatta-Cherifi, O. Chabre, A. Murat, P. Niccoli, C. Cardot-Bauters, V. Rohmer, J. Young, B. Delemer, H. Du Boullay, M. F. Verger, J. M. Kuhn, J. L. Sadoul, Ph Ruszniewski, A. Beckers, M. Monsaingeon, E. Baudin, P. Goudet & A. Tabarin (2012). "Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des Tumeurs Endocrines database". European journal of endocrinology. 166 (2): 269–279. doi:10.1530/EJE-11-0679. PMID 22084155. Unknown parameter |month= ignored (help)

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