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==Risk Factors==
==Risk Factors==
Risk factor associated with adrenocortical carcinoma are:
Risk factor associated with adrenocortical carcinoma are:
 
* Lynch syndrome
*[[Li-Fraumeni syndrome]]
*[[Beckwith-Wiedemann syndrome]]
*[[Beckwith-Wiedemann syndrome]]
*[[Carney complex]]
*[[Carney complex]]
*[[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]].
*Neurofibromatosis type 1
*MEN1
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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
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| 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
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*  
* MSH2, MSH6, MLH1, PMS2
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* 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
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* Colorectal cancer
* Endometrial cancer
* Sebaceous neoplasms
* Ovarian cancer
* Pancreatic cancer
* Brain cancer
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| 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
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type 1
* Mass effect symptoms; symptoms related to  excess glucocorticoid, mineralocorticoid, androgen, or estrogen  secretion
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* 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
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* Serum DHEAS
* Malignant peripheral nerve sheet tumor
* Measures of clinicallyindicated
* Pheochromocytoma
* steroid
* Café au lait spots
* Neurofibroma
* Optic glioma
* Lisch nodule
* Skeletal abnormalities
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|Cushing's syndrome
|'''MEN1 45'''
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* Rapid [[Obesity|weight gain]], particularly of the [[trunk]] and [[face]] with [[limbs]] sparing ([[central obesity]])
* Proximal [[muscle weakness]]
* A [[round face]] often referred to as a "[[moon face]]"
* Excess [[sweating]]
* [[Headache]]
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* Imaging may show adenoma if presents
* MENIN
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* 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 hyperplasia
* Collagenoma
* Angiofibroma
* Adrenal adenoma/hyperplasia
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| 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
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* [[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.
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* 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
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* [[Plasma]] fractionated [[Metanephrine|metanephrines]] 
* Primary pigmented nodular
* 24-hour [[urinary]] fractionated [[Metanephrine|metanephrines]]
* Adrenal disease
* Large-cell calcifying Sertoli cell tumors
* Thyroid adenoma
* Myxoma
* Somatotroph pituitary adenoma
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| 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 41
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** Symptoms and signs of primary malignancy especially lung cancer.
** General constitutional symptoms:
** Fever
** Fatigue
** Weight loss
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* Irregular shape and inhomogeneous nature
* IGF2, CDKN1C, 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
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* Wilms’ tumor
* Hepatoblastoma
* Macrosomia
* Adrenocortical cytomegaly
* Adrenal adenoma
* Adrenal cyst
* Hemihypertrophy
* Macroglossia
* Omphalocele
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Revision as of 18:36, 18 September 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]

Overview

The most potent risk factors in the development of adrenocortical cancer are TP53 mutation, Beckwith-Wiedemann syndrome and Carney complex.[1]

Risk Factors

Risk factor associated with adrenocortical carcinoma are:

Differential Diagnosis Gene mutations Clinical picture
Lynch syndrome
  • MSH2, MSH6, MLH1, PMS2
  • Colorectal cancer
  • Endometrial cancer
  • Sebaceous neoplasms
  • Ovarian cancer
  • Pancreatic cancer
  • Brain cancer
Neurofibromatosis

type 1

  • NF1
  • Malignant peripheral nerve sheet tumor
  • Pheochromocytoma
  • Café au lait spots
  • Neurofibroma
  • Optic glioma
  • Lisch nodule
  • Skeletal abnormalities
MEN1 45
  • MENIN
  • Foregut neuroendocrine tumors
  • Pituitary tumors
  • Parathyroid hyperplasia
  • Collagenoma
  • Angiofibroma
  • Adrenal adenoma/hyperplasia
Carney complex
  • PRKAR1A
  • Primary pigmented nodular
  • Adrenal disease
  • Large-cell calcifying Sertoli cell tumors
  • Thyroid adenoma
  • Myxoma
  • Somatotroph pituitary adenoma
BWS 41
  • IGF2, CDKN1C, H19
  • Wilms’ tumor
  • Hepatoblastoma
  • Macrosomia
  • Adrenocortical cytomegaly
  • Adrenal adenoma
  • Adrenal cyst
  • Hemihypertrophy
  • Macroglossia
  • Omphalocele

References

  1. ↑ National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq#section/_1

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