Flashcards in Pathology of the Adrenal gland Deck (79):
What is the role of aldosterone?
Causes reabsorption of Na and water, and secretion of K
What is the function of the adrenal glands, in order?
G= Aldosterone secretion
R= Sex hormones
"it gets sweeter as it gets deeper"
What are the two primary adrenal cortical neoplasms? Which is more common?
-Cortical adenoma-more common
What are the typical characteristics of an adrenal cortical adenoma? Where in the gland does it lie?
Well circumscribed, yellow-orange lesion that usually lies in the cortex or protrudes into the medulla or the subcapsular region
Why are adrenal cortical adenomas yellow-orange in color?
High lipid content
What is the genetic mutation that is affected with neuroblastomas?
Do adrenal adenomas lie within or outside the cortex?
May lie within, or protrude into medulla
What are the general characteristics of large (greater than 1 cm) adrenal cortical adenomas?
Areas of hemorrhage, cystic changes, and calcification
How can you differentiate between functional and nonfunctional adrenal adenomas?
Only through lab findings
Adenomas are distinguished from nodular hyperplasia how?
Solitary, circumscribed mass
What are the histological characteristics of adrenal cortical adenomas? (3)
Vacuolated d/t the presence of intracytoplasmic lipid
-Mild nuclear pleomorphism
-No mitotic activity
Why are adrenal cortical adenomas vacuolated?
d/t the presence of intracytoplasmic lipid
True or false: adrenal cortical carcinomas are not very malignant, relatively
What are the characteristics of the cut surface of adrenocortical neoplasms?
Yellow on cut surface, but usually contain areas of hemorrhage, cystic changes, and necrosis
What are the histological characteristics of adrenocortical neoplasms?
Range from well differentiated to markedly anaplastic cells
Where do adrenal adrenocortical carcinomas usually metastasize to?
Lymph nodes viscera and lungs
What is the only way to differentiate between malignant adrenocortical neoplasms from benign ones?
If it spreads
What happens to the kidneys with adrenal carcinomas?
What are the four major characteristics of anaplastic cells in adrenocortical carcinomas?
2. Abnormal nuclear morph
4. Loss of polarity
What does it mean for cells to be pleomorphic?
Different sizes and shapes
What are the characteristics of abnormal nuclear morphology of adrenal cortical carcinomas?
variable nuclear condensation
What characteristics, besides metastases, are found with adrenocortical carcinomas and not with adenomas?
What are two characteristics of adrenocortical neoplasms that 100% define them to be malignant?
What are the three major characteristics of adrenal cortical hyperplasia (Color, thickness, nodularity)?
What is the difference between hypertrophy and hyperplasia?
Hypertrophy = increase in cell size
Hyperplasia = increase in number of cells
What are the three distinctive hyperadrenal syndromes?
What, generally, is Cushing's syndrome?
Elevation in glucocorticoid levels
What is Cushing's disease?
Pituitary adenoma causing an increase in ACTH secretion
What is Cushing's syndrome?
Excess cortisol or ACTH for some reason besides a pituitary pathology
What are the four major physical manifestations of Cushing's?
Hypo or hypertension with Cushing's?
True or false: menstrual abnormalities are common in Cushing's
True or false: neuropsych problem are common in Cushing's
What are the characteristics of the moon facies seen with Cushing's?
-Cannot see ears
-Cannot see nose from side
What is primary hyperaldosteronism?
-Aldosterone producing adrenocortical neoplasm, usually an adenoma
-Primary adrenocortical hyperplasia
What is Conn syndrome?
A solitary aldosterone secreting adenoma or the adrenal gland
What are the characteristics of the cut surface of an aldosterone producing adenoma?
Bright yellow d/t high lipid content
Are the adenomas seen in Conn's syndrome usually solitary or diffuse? Small or large? Encapsulated or not?
What are the key lab values that are abnormal in hyperaldosteronism? (4)
What is the classical presentation of a pt with primary hyperaldosteronism?
Young person with resistant HTN
Is renin usually low or high with hyperaldosteronism? Why?
Low, since HTN will increase delivery of solutes to the distal nephron
What is the eponym for chronic primary adrenal insufficiency?
What is the name for acute adrenocortical insufficiency?
What is Waterhouse-Friderichsen syndrome? What is the usual causative agent?
Adrenal gland failure due to hemorrhage into the adrenal glands, usually occurring after sepsis with neisseria meningitidis
In whom does Waterhouse-Friderichsen syndrome usually occur in?
In whom does Addison's disease usually occur in? How much of the adrenal gland must be destroyed for this to occur?
Adults who suffer at least 90% destruction of their adrenal cortex
True or false: Addison's disease is an autoimmune condition
What are the two infectious agents that classically lead to the development of Addison's disease?
Which carcinoma classically lead to the development of Addison's disease?
Carcinomas of the lung and breast
What are the three major histological characteristics of Addison's disease?
-Lipid depletion of the adrenal cortex
-Lymphocytic infiltration of the cortex
Which part of the adrenal gland is usually spared with autoimmune adrenalitis?
A pt with a h/o autoimmunity presenting with hyperpigmentation should be suspicious for what?
Addison's disease (primary adrenal insufficiency)
What are the major electrolyte disturbances with Addison's disease?
What are the general ssx of Addison's?
HTN or hypotension with Addison's disease? Why?
Hypotension d/t loss of aldosterone and cortisol
What zones of the adrenal glands are spared with secondary adrenocortical insufficiency? What is the clinical significance of this?
Zons glomerulosa and medulla
This means that aldosterone production is unaffected, and thus there are no electrolyte disturbances or BP changes
What are the general causes of secondary adrenocortical insufficiency?
Hypothalamic or pituitary disorder, leading to Decreased production of ACTH
In what type of adrenocortical insufficiency is there hyperpigmentation, primary or secondary?
Primary d/t increased ACTH and POMC production
What are the "five 10%" rules of pheochromocytoma?
-10% extra adrenal
What, generally, is the range of sizes of pheochromocytomas?
1 g - 4 kg
What are the characteristics of the cut surface of a pheochromocytoma?
Gray or brown, and is often associated with hemorrhage, necrosis, or cystic changes
True or false: pheochromocytomas are rarely vascular
What is the agent that is used to fix pheochromocytomas? What does this do?
Causes it to turn brown-black d/t oxidation of catecholamines
What are the two neuroendocrine markers for a pheochromocytoma?
What are the histological characteristics of a pheochromocytoma?
Mature, polygonal to spindle-shaped medullary cells with basophilic granules
What are the reliable histologic predictors of malignancy for pheochromocytom
None--only mets can tell if malignant
Where do pheochromocytomas usually metastasize to? (4)
What four other organ dysfunctions appear with pheochromocytomas?
How do you diagnose a pheochromocytoma?
Measure urinary and serum catecholamines and their metabolites
What is the specific imaging modality for pheochromocytomas?
MIBG scan (I-131 will localize)
What are neuroblastomas? Where anatomically do they usually occur? In whom are they seen?
Tumors derived from neural crest cells d/t an n-Myc amplicifcation.
Usually extracranial and in the adrenal medulla
Sporadically in children under 5 y.o.
What are the histological characteristics of Neuroblastomas?
Small blue cell tumors with Homer-Wright rosettes
What will a scanning EM show with neuroblastomas?
What are the clinical PE findings with neuroblastomas?
Palpable abdominal mass
Where do neuroblastomas usually met to?
Skin and bones
What are the markers for neuroblastomas?
Increased urinary VMA, metanephrines and HVA
What are the cutaneous manifestations of neuroblastomas?
Blueberry muffin baby
What are adrenal gland ganglioneuromas?
Tumor composed of mature ganglion cells and neuronal elements