functioning adrenal tumors - scbtmr.org · • thin section ct detects many adrenal nodules •...

FUNCTIONING ADRENAL TUMORS

SCBTMR, San Diego, CaMarch 2010

Isaac R Francis, MDUniversity of Michigan

Ann Arbor, Michigan

Consultant- Research Contract General Electric HealthCare

DISCLOSURES

FUNCTIONING ADRENAL TUMORSDx HYPERCORTISOLISM

• 24 hr. screening with urinary free cortisol or 17-hydroxysteroid estimation

• Single dose-dexamethasone suppression test

• If both negative- Cushing’s excluded

FUNCTIONING ADRENAL TUMORSCUSHING’S SYNDROME

ACTH Dependent causesACTH-independent causes

FUNCTIONING ADRENAL TUMORSCUSHING’S

ACTH-dependent causes:• Pituitary causes (Cushing’s

disease)• Ectopic ACTH: • 80% located in thorax

* Bronchial carcinoid (common)* Thymic carcinoid* Medullary thyroid cancer

* Pancreatic islet-cell tumors* Phoechromocytoma

FUNCTIONING ADRENAL TUMORSACTH-DEPENDENT

CUSHING’S DISEASE

• Adrenal glands usually elongated, thickened and or nodular (70%)

• Can be normal (30%)• Hyperplasia usually smooth,

but in 12-15%- can be nodular– “macronodular hyperplasia”


CUSHING’S DISEASE

• Accuracy for pituitary adenoma detection-CT/MRI-75%

• Inferior Petrosal Sinus Sampling [IPSS]- 90%

• IPSS +CRH stimulation-95%


CUSHING’S SYNDROMEECTOPIC ACTH

• Rare cause of ACTH-dependent Cushing’s syndrome Accounts for small number of cases-approx. 5%

• Most common site- THORAX (80%)-anterior mediastinum, bronchial, lung parenchyma

• Bronchial carcinoid small tumors-hard to detect and indistinguishable from other lung nodules


CUSHING’S SYNDROMEECTOPIC ACTH

• Small cell (oat cell) carcinoma• Carcinoid – thymus and

bronchial/lung• Medullary thyroid cancer• Pancreatic neuroendocrine• Pheochromocytoma* If no cause is found, systemic

venous sampling may be employed


CUSHING’ SYNDROMEECTOPIC ACTH

• Adrenal glands are markedly thickened and nodular

• Thicker and larger than seen in pituitary- dependent Cushing’s

• 19% had nodules larger than 10 mm in size

* Sohaib SA et al AJR 99

FUNCTIONING ADRENAL TUMORSCUSHING’S

ACTH-independent causes:• Adrenal cortical adenoma• Adrenal cortical carcinoma• Account for 90% of cases

Additional rare causes:• ACTH-independent macronodular

adrenal hyperplasia (AIMAH)• Primary Pigmented nodular

hyperplasia (PPNAD)

FUNCTIONING ADRENAL TUMORSACTH-INDEPENDENTCUSHING’S SYNDROME

ADRENAL CORTICAL ADENOMA

IMAGING FINDINGS

• Account for 10-15% of Cushing’s patients

• Range 2-7 cm- Mean size 2-2.5 cm• Thin sections not necessary• Low density – due to lipid content• Contralateral gland maybe atrophic

(due to suppression), but usually appears normal

FUNCTIONING ADRENAL TUMORSACTH-INDEPENDENTCUSHING’S SYNDROME

ADRENAL CORTICAL CARCINOMA

• Tumors usually large (mean > 6cms)

• Often have areas of hemorrhage, necrosis and calcification

• May have IVC involvement

ADRENAL CORTICAL NEOPLASMSCUSHING’S SYNDROME

ADRENAL CORTICAL CARCINOMA

MALIGNANT TUMORS• Direct invasion of adjacent

organs- liver, kidneys• Lymph node metastases• Distant metastases

ADRENAL CORTICAL TUMORSADRENAL CORTICAL CARCINOMA

• 53 proven tumors• 36% with overt hyperfunction• 76% presented as Stage III and

IV• Common sites of metastases

were: Liver, lymph nodes, bone and lungs*Zografos GC, et al J of Surg Oncol 1994

ADRENAL CORTICAL TUMORSADRENAL CORTICAL CARCINOMA

Treatment• Surgical• Laparascopical removal : Only if

there are no imaging features suggestive of malignancy and tumor is under 6 cms

• CT and MR can evaluate for these features prior to surgery

FUNCTIONING ADRENAL TUMORSPRIMARY HYPERALDOSTERONISM

• Hypertension-accts. for 0.1-0.5% of hypertensive cases

• Hypokalemia• Metabolic acidosis• Low plasma renin• Elevated serum/urinary

aldosterone levels


• Adenoma accounts for 75% of cases ( contrast to Cushing’s)

• Adrenal hyperplasia – 25%• Adrenal cortical carcinoma-

rare cause for Conn’s syndrome

FUNCTIONING ADRENAL TUMORSCONN’S ADENOMA OR

ALDOSTERONOMA

IMAGING FINDINGS• Mean size for adenoma 1-1.5

cm• Approx. 20% < 1 cm in size• Thin sections are mandatory

(in contrast to Cushing’s)• Variable appearances


• CT adenoma diagnosis is fairly reliable• But CT diagnosis of adrenal hyperplasia

may be unreliable• Thin section CT detects many adrenal

nodules• Adrenal venous sampling essential in

patients:- with normal adrenal glands- bilateral nodules or thickening- Equivocal CT or MR findings or

discordancy between imaging and lab data

FUNCTIONING ADRENAL TUMORSMEDULLARY HYPERFUNCTION

• Hypertension• Elevated catecholamines-

adrenaline and noradrenaline• May also secrete dopamine,

ACTH, gastrin, serotonin, calcitonin

ADRENAL MEDULLARY NEOPLASMS

PHEOCHROMOCYTOMAS

• Majority of tumors located in adrenal gland (90%)

• Remainder are mostly in abdomen and pelvis

• Small % in neck and chest• Often referred to as the

“10% tumor”• 10% extra-adrenal,

malignant and metastasize

FUNCTIONING ADRENAL TUMORSPHEOCHROMOCYTOMA

EXTRA-ADRENAL LOCATIONS• Retroperitoneal (46%)• Organs of Zuckerkandl(29%)• Pelvis/Urinary bladder (10%)• Thorax Mediastinum/intrapericardial

(10%)• Head and neck (2-4%)


PHEOCHROMOCYTOMAS- ASSOC. SYNDROMES

• Von Hippel-Lindau disease [vHL]

• Neurofibromatosis• MEA (N) II a and b syndromes• Tuberous sclerosis• Carney’s triad - [GI stromal

tumors, pulmonary chondromas, and pheochromocytomas]

• Familial


PHEOCHROMOCYTOMAS-CT• Catecholamine response to ionic

iodinated contrast media unpredictable

• Study with small number of cases shown that non-ionic contrast can be given safely

* Raisanen J et al AJR 1984* Mukherjee JJ et al. Radiology 1997


PHEOCHROMOCYTOMAS-CT

• Tumors are large ( 4-5 cm)- except in MEA[N]

• Can have areas of hemorrhage, necrosis, fluid-fluid levels

• Can show areas of brisk enhancement with IV contrast

• Non-ionic contrast media can be safely used


PHEOCHROMOCYTOMAS-MRI• Areas of high SI on T1-W images

- Hemorrhage• Can be very hyperintense- “light

bulb” bright on T2-W images- this appearance is not pathognomonic

• Approx. 25% of tumors show areas of heterogenous contrast enhancement

• Cannot be readily differentiated from adrenal cortical carcinoma

FUNCTIONING ADRENAL TUMORSEXTRA-ADRENAL TUMORS

(PARAGANGLIOMAS)

• May be less hormonally active• More likely to be malignant

(40%)• Can be associated with MEN IIA,

MEN IIB, neurofibromatosis, vHL, Carney’s triad and familial

* Shadev A et al. Eur Radiol 2005


MIBG-SCINTIGRAPHY• Accuracy for pheochromocytoma detection

around 90%- less than that for CT and MR• Whole body imaging capability makes it

suitable to screen for extra-adrenal sites and for metastases

• MIBG may not be needed in patients biochemical evidence of pheo and adrenal mass seen on CT or MRI

* Miskulin J et al Surgery 2003* Greenblatt DY Ann Surg Oncol 2008


PET AND FUNCTIONING ADRENAL TUMORS

• Detection of tumors larger than 1 cm was 90% with MIBG vs. 25% for octreotide scintigraphy

• Sensitivity for pheochromocytoma detection with FDG is 70%

• 11-C epinephrine, hydroxyephedrine, phenylephrine, and 6-18-F-fluorodopamine have been studied

• 100% accuracy for 6-18-F-fluorodopamine• 91.6% sensitivity and 100% specificity with 11-

C hydroxyephedrine* Trampal C Radiology 2004

* Pacak K Hypertension 2001* Van der Harst E J clin Endo Metab 2001

functioning adrenal tumors - scbtmr.org · • thin section ct detects many adrenal nodules •...

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