Pathology of the Adrenal gland Flashcards by tcarlso 3

What is the role of aldosterone?

Causes reabsorption of Na and water, and secretion of K

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What is the function of the adrenal glands, in order?

G= Aldosterone secretion
F= Cortisol
R= Sex hormones

“it gets sweeter as it gets deeper”

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What are the two primary adrenal cortical neoplasms? Which is more common?

Cortical adenoma-more common

- Cortical carcinoma

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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

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Why are adrenal cortical adenomas yellow-orange in color?

High lipid content

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What is the genetic mutation that is affected with neuroblastomas?

n-Myc

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Do adrenal adenomas lie within or outside the cortex?

May lie within, or protrude into medulla

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What are the general characteristics of large (greater than 1 cm) adrenal cortical adenomas?

Areas of hemorrhage, cystic changes, and calcification

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How can you differentiate between functional and nonfunctional adrenal adenomas?

Only through lab findings

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Adenomas are distinguished from nodular hyperplasia how?

Solitary, circumscribed mass

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What are the histological characteristics of adrenal cortical adenomas? (3)

Vacuolated d/t the presence of intracytoplasmic lipid

Mild nuclear pleomorphism
No mitotic activity

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Why are adrenal cortical adenomas vacuolated?

d/t the presence of intracytoplasmic lipid

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True or false: adrenal cortical carcinomas are not very malignant, relatively

False–very malignant

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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

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What are the histological characteristics of adrenocortical neoplasms?

Range from well differentiated to markedly anaplastic cells

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Where do adrenal adrenocortical carcinomas usually metastasize to?

Lymph nodes viscera and lungs

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What is the only way to differentiate between malignant adrenocortical neoplasms from benign ones?

If it spreads

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What happens to the kidneys with adrenal carcinomas?

Compression

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What are the four major characteristics of anaplastic cells in adrenocortical carcinomas?

1, Pleomorphic

Abnormal nuclear morph
Mitoses
Loss of polarity

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What does it mean for cells to be pleomorphic?

Different sizes and shapes

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What are the characteristics of abnormal nuclear morphology of adrenal cortical carcinomas?

variable nuclear condensation

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What characteristics, besides metastases, are found with adrenocortical carcinomas and not with adenomas?

Large size
Necrosis
Mitotic figures
Vascular invasion

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What are two characteristics of adrenocortical neoplasms that 100% define them to be malignant?

Mets

- Vascular invasion

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What are the three major characteristics of adrenal cortical hyperplasia (Color, thickness, nodularity)?

Yellow
Thickened
Multinodular

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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?

- Cushing's syndrome - Hyperaldosteronism - Adrenogenital 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?

- central obesity - Moon facies - Cutaneous striae - Hirsutism

Hypo or hypertension with Cushing's?

HTN

True or false: menstrual abnormalities are common in Cushing's

True

True or false: neuropsych problem are common in Cushing's

True

What are the characteristics of the moon facies seen with Cushing's?

- Cannot see ears - Round face - Cannot see nose from side

What is primary hyperaldosteronism?

-Aldosterone producing adrenocortical neoplasm, usually an adenoma OR -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?

Solitary Small Encapsulated

What are the key lab values that are abnormal in hyperaldosteronism? (4)

- Hypocalcemia - Hypokalemia - Low renin - High aldosterone

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?

Addison's' disease

What is the name for acute adrenocortical insufficiency?

Adrenal crisis

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?

Children

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

True

What are the two infectious agents that classically lead to the development of Addison's disease?

TB | Fungi

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?

- Small glands - Lipid depletion of the adrenal cortex - Lymphocytic infiltration of the cortex

Which part of the adrenal gland is usually spared with autoimmune adrenalitis?

Adrenal medulla

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?

Hyperkalemia | Hyponatremia

What are the general ssx of Addison's?

n/v - Anorexia - Cutaneous hyperpigmentation

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% malignant - 10% nonfunctional - 10% bilateral - 10% extra adrenal - 10% familial

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

False--highly

What is the agent that is used to fix pheochromocytomas? What does this do?

Zenker Causes it to turn brown-black d/t oxidation of catecholamines

What are the two neuroendocrine markers for a pheochromocytoma?

Synaptophysin + | Chromogranin +

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)

Lymph nodes Liver Lungs Bone

What four other organ dysfunctions appear with pheochromocytomas?

- CHF - MI - Arrhythmias - Cerebral hemorrhage

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?

Neurosecretory granules

What are the clinical PE findings with neuroblastomas?

Palpable abdominal mass | Diastolic HTN

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

What is the mutation that causes Wilms tumor?

WT1 gene on chromosome 11

Pathology of the Adrenal gland Flashcards

(79 cards)