adrenal gland Flashcards
What are the 3 main categories of adrenal disease?
Hyperfunctioning, Adrenal insufficiency, normal functionE
What is cushings syndrome? What are the two types? What is the diagnostics test?
excess glucocorticoid
Exogenous - large doses of steroid
Endogenous - overproduction of cortisol by adrenal cortex
urniary 17-hydroxycorticosteroid level
What is the majority cause of cushings syndrome? What are the types?
Excess ACTH
ACTH dependent - bilateral adrenal hyperplasia
ACTH independent - adenoma, carcinoma, PPNAD, AIMAH
What are the causes of excess ACTH in ACTH dependent cushings?
Pituitary, oat cell tumor, bronchial adenoma, ovary, pancreas, thymus, thyroid
Truncal obesity, hirsutism, abdominal striae, and muscle atrophy suggest what syndrome?
Cushings
How do you distinguish macronodular hyperplasia from pituitary adenoma versus a cortisol producing adenoma
Size of contralateral adrenal gland - will be atrophic in cortisol secreting adenoma and enlarged in pituitary adenoma
What is conns syndrome? What are the types?
Primary aldosteronism
Adrenal hyperplasia - idiopathic hyperaldosteronism (more common), primary adrenal hyperplasia
Adrenal tumor
What are the two types of primary aldosteronism hyperplasia subtype? What are the differences in treatment?
Idiopathic hyperaldosteronism - medically
Primary adrenal hyperplasia - surgical if unilateral, otherwise medical
Hypokalemia, hypertension, increased aldosterone, low renin
Conn syndrome
What is the pathophysiology of conns syndrome?
ALdosterone causes sodium retention which leads to increased plasma volume and hypertension. Potassium is exchanged for sodium in the distal tubule, thus causing hypokalemia
How is primary vs secondary hyperaldosteronism determined?
Serum renin levels - will be elevated in secondary and low in primary
What is the most common form of CAH? What are the symptoms?
deficiency of 21-hydroxylase
excess androgen effect
What are the two types of adrenal insufficiency?
Primary - tissue destruction of adrenal glands
Secondary - inadequate stimulation
What are 5 causes of primary adrenal insufficiency
Idiopathic Granulomatous disease Metastasis - rarely, because so much cortex (>90%) must be destroyed Lymphoma Hemorrhage
What is a distinguishing characteristic of primary vs secondary adrenal insufficiency?
Primary will have destruction of all producing cells and have decreased cortisol, androgen, and aldosterone.
Secondary will have normal aldosterone production
How much of the adrenal gland has to be destroyed before addisons occurs?
90%
why do addisons patients get hyperpigmentation?
Because of decreased cortisol production, there will be increased ACTH production which is on the same gene as melanocyte stimulating hormones
What is the radiographic appearance of addisons?
Depends on the cause
Granulomatous disease will be calcified with bilateral enlargement with maintanance of shape
Which tumors are larger - conns or cushings
Cushings
Which has larger adrenal glands - exogenous or endogenous?
Exogenous
When is venous sampling helpful?
in determining masculinizing/feminizing tumors
What are pheochromocytomas composed of? What is the name for an extra-adrenal pheochromocytoma?
Chromaffin cells
Paragangliomas
What is MEN2A and MEN2B
2A - PMP, medullary thyroid, pheo, parathyroid
2B - Medullary, pheo, marfan, neuromas
What, aside from MEN, are the associations with pheochromocytoma? What is carney’s triad?
NF1, VHL
gastric leiomyosarcoma, pulmonary chondroma, extra adrenal pheochromocytoma
Where are most pheos located? What is the difference in location between sporadic and MEN associated pheos?
90% in medulla
Sporadic - up to 25% outside adrenal, normal medulla
MEN - multicentric and intraadrenal, hyperplastic medulla
T or F: extraadrenal pheos are more likely to be malignant
True
What is administered prior to removal of pheo?
alpha (phenoxybenzamine) and beta blockers (propranolol)
How do pheos enhance? how do they appear on T1/T2?
heterogeneously
hypo T1
hyper T2
What are the NM scans used for pheochromocytoma?
I131-MIBG or Indium 111 pentetreotide (octreotide) scan
PET scans are also useful and can detect tumors that fail to concentrate MIBG
Where is the most common site for a neuroblastoma?
Adrenal gland
What age range for neuroblastomas?
What are neuroblastomas? What is the malignant and benign maturation tumor?
Primitive tumors from sympathetic nervous system tissue
Benign - ganglioneuroma, greater degree of cellular maturation
Malignant - ganglioneuroblastoma, greater degree of cellular maturation with more mature ganglion cells mixed with neuroblasts
What is the radiographic appearance of neuroblastoma?
US - heterogenous echogenicity with poorly defined margins
CT - can have calcification on unenhanced study. Large and heterogenous w/wo low density areas of necrosis or hemorrhage. CAN INVADE paraspinal musculature and foramen
MRI - hetergenous signal. T1 will be hypo to liver/kidney, T2 will be hyper to liver and iso to kidney
Which tumors can invade paraspinal musculature and neural foramina
Neuroblastomas
Which tumor has a “dumbbell” appearance? Why?
Neuroblastoma - tumor growth through the foramen with epidural expansion
Distinguish posttherapy fibrosis from recurrent neuroblastoma?
Fibrosis is hypo on T2
Recurrence is hyper
What are the NM scans useful for neuroblastomas?
Bone scans - due to high rate of bone mets
MIBG or Octreoscan
What is the makeup of an adrenal adenoma
cords of clear cells separated by fibrovascular trabeculae
What percentage of adenoma are lipid poor?
20%
How does an adrenal adenoma image on MRI? How is it different/similar to mets?
Low signal on T1 and T2
Low on T2 differentiates between mets (high T2, low T1)
With regards to adrenal carcinoma, M or F get functional tumors more?
Female
What is the most common functional syndrome with adrenal carcinoma?
Cushings
What is the CT appearance of adrenal carcinoma?
Large mass with central hypoattenuation representing necrosis, calcification in 30%
What are the important areas to check with adrenal carcinoma regarding extension/mets?
Hepatic or regional lymph node metastasis
Extension into left renal vein or IVC
What is needed to confidently rule out hepatic mets in adrenal carcinoma
Fat plane separating tumor and adrenal gland
What invasive method can be used to diagnose adrenal origin?
Selective arteriography of the renal, inferior phrenic, celiac, hepatic, or middle adrenal
What is the vasculature to adrenal carcinoma?
HYPOvascular,
most vascularity is on the periphery which is supplied by the inferior phrenic artery
What is the MR imaging of adrenal carcinoma?
heterogenous and hyperintense on T1 and T2
heterogenous enhancement
What is a myelolipoma? How does it look on US?
benign tumor of mature adipose cells and hematopoietic tissue
Highly echogenic mass with propagation speed artifact (similar to comet tail)
What is the main difference between adenoma and myelolipoma?
Adenoma - homogenous lowe density
Myelolipoma - heterogenous low density
Which side is adrenal hemorrhage more common in? Why?
Right
The right adrenal vein enters the IVC directly, which in trauma with the increase in venous pressure, is directly transmitted back to the right adrenal vein
What are the associated conditions with adrenal hemorrhage
septicemia, HTN, renal vein thrombosis, tumor, anticoagulation therapy, steroids
What age is adrenal hemorrhage most common?
Newborn
What is the result of most adrenal hemorrhages?
Resorption
a small amount can persist as a pseudocyst
What are the types of adrenal cyst? which is most common? M or F prediliction?
Female prediliction
Endothelial (most common), lymphangiomatous, epithelial, parasitic, pseudocysts (second most common)
Which sex is hemangioma more prominent?
Female prediliction
Large mass with thick irregular wall and hypodense ceter
What are CT findings suggesting mets over adenoma
Size >3cm poorly defined margins invasion of adjacent structures inhomogenous attenuation thick, irregular, enhancing rim
What is the nuclear medicine scan that is useful in adenoma vs mets
scintigraphy with NP59
What is the difference in contrast enhancement between adenoma and mets?
Adenoma washout quicker!
What is the equation to determine enhancement washout?
% enhancement washout = enhancement washout / enhancement
Enhancement washout = enhanced attenuation value - delayed contrast attenuation value
Enhancement = enhanced - unenhanced
What is the equation to determine washout if no unenhanced is available
% relative washout = enhancement washout / enhanced attenuation value
What are the cutoff percentages to diagnose adenoma?
Regular - if washout >60%, adenoma is confidently diagnosed
Relative - if washout >40%, adenoma confidently diagnosed
What is the basis of comparison on in and out of phase imaging?
Paraspinal muscles
What is the general cutoff on MRI in/out of phase imaging in terms of signal intensity loss?
> 20%
What is the most common complication in adrenal biopsy? What is the complication in an anterior approach to the left adrenal gland?
Pneumothorax
Pancreatitis
Which lymphoma is more common in the adrenal glands?
NHL
What is the appearance of lymphoma in the adrenal glands?
Homogenous soft tissue mass with enhancement