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Adrenocortical Carcinoma

 Syn. ACC , Adrenal cortical carcinoma , Primary adrenal carcinoma

Signs & Symptoms

 Supraclavicular fat pads
Note: due to hypercortisolism, due to Cushing syndrome
Ref: Uptodate [ Epidemiology and clinical manifestations of Cushing's syndrome] Harrison [ 2725] Cecil [ 647]
Weight gain
Note: due to glucocorticoid excess, due to Cushing syndrome, due to atypical depression, Usually develop very rapidly (over three to six months)
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors] Harrison [ 2725] Cecil [ 647]
Progressive central obesity
Note: due to Cushing syndrome
Ref: Uptodate [ Epidemiology and clinical manifestations of Cushing's syndrome] Harrison [ 2725] Cecil [ 647]
Progressive obesity
Note: due to Cushing syndrome
Ref: Uptodate [ Epidemiology and clinical manifestations of Cushing's syndrome] Harrison [ 2725] Cecil [ 647]

Physical Examination

 Supraclavicular fat pads
Note: due to hypercortisolism, due to Cushing syndrome
Ref: Uptodate [ Epidemiology and clinical manifestations of Cushing's syndrome] Harrison [ 2725] Cecil [ 647]
Facial plethora
Note: due to hypercortisolism
Ref: Uptodate [ Epidemiology and clinical manifestations of Cushing's syndrome] Harrison [ 2725] Cecil [ 647]
Hypertension
Note: due to Cushing syndrome, due to hyperaldosteronism
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors] Harrison [ 647] Cecil [ 647]

Lab / Serology

 Low serum aldosterone
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]
High serum aldosterone precursors
Note: adrenal carcinomas
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]
High serum deoxycorticosterone
Note: adrenal carcinomas
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]

Imagin Study

 FDG uptake
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]
Size greater than 4 cm
Note: Size alone is of poor predictive value
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors] Harrison [ 2731] Cecil [ 651]
Adrenal incidentaloma
Note: Patients with nonfunctioning tumors.
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors] Harrison [ 2731] Cecil [ 651]

Complication

 Virilization Gr. children
Note: In children
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]
Clinical syndrome of hormone excess
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors] Cecil [ 651]
Local invasion
Note: At initial presentation.
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]

Risk Factors

 Age between 30 and 50
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]
Age less than five
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]
Female
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]

Cause

 Abnormalities in 11p15
Note: Beckwith-Wiedemann syndrome.
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors] Harrison [ 2731]
Inactivating mutations of the MEN1 gene on chromosome 11q
Note: Associated with MEN1
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors]
Activating somatic mutation of the CTNNB1 gene
Ref: Uptodate [ Clinical presentation and evaluation of adrenocortical tumors] Harrison [ 2731]

Other