Adrenal Gland Pathology Flashcards
What is the adrenal cortex derived from?
Mesoderm
What is the adrenal medulla derived from?
Neuroectoderm
Cushing Syndrome (Hypercortisolism)
Can be due to secretion of ACTH by tumors in pituitary or elsewhere leading to hypercortisolism with bilateral hyperplasia of adrenals
What is the most common cause of Cushing Syndrome?
Exogenous administration of glucocorticoids
Cushing’s Disease
Primary hypothalamic pituitary disease associated with the hypersecretion of ACTH
What gender is more affected by Cushing’s syndrome?
Males
What are the 4 main causes of Cushing syndrome?
- Exogenous glucocorticoids
- Pituitary ACTH oversecretion
- Lung tumor paraneoplastic ACTH secretion
- Adrenal cortisol producing tumor
What will happen to the adrenal glands with exogenous glucocorticoid administration?
Atrophy
What are the clinical features of Cushing syndrome?
- HTN
- Weight gian
- Buffalo hump
- Truncal obesity
- Increased risk of infection
- Bone resorption
What are the clinical manifestations of hyperaldosteronism?
HTN with hypokalemia and increased Na+ retention
What are some primary causes of hyperaldosteronism?
- Familal genetic defect
- Tumors
- Idiopathic
Congenital Adrenal Hyperplasia
Autosomal recessive with a defect in enzyme involved in adrenal steroid synthesis and decreased cortisol synthesis leads to increased androgen synthesis
What is the most common defect in congenital adrenal hyperplasia?
21-alpha hydroxylase
Waterhouse-Friderichsen Syndrome
Sudden withdrawal of long-term corticosteroid therapy leads to stress with underlying acute adrenal insufficiency
What is the most common cause of chronic adrenal insufficiency?
Autoimmune Adrenalitis
What are some of the causes of Waterhouse-Friderichson syndrome?
- Septicemia
- Hypotension
- Massive bilateral adrenal hemorrhage
Addison’s Disease
Autoimmune adrenalitis that leads to atrophy of the glands with thinning of the cortices with the medulla often unaffected
What is hyperpigmentation a sign of?
Increased ACTH - decreased adrenal sufficency
Adrenal Carcinoma
Rare - some inherited cases like Li-Fraumeni syndrome and are quite invasive with poor survival
What is the best criteria for malignancy in adrenocortical carcinoma?
Invasion
Pheochromocytoma
A neoplasma arising from the chromaffin cells in the paraganglion system and can cause HTN
What is seen on histology of pheochromocytoma?
Zellballen (cellular balls or nests)
What are the clinical features of pheochromocytoma?
- HTN
- Headache
- Sweating
- Tremor
- Elevated VMA in urine
Neuroblastoma
Neoplasm arising in the adrenal medulla or paraganglion tissue that are large and bulky
MEN 1
Autosomal dominant
- Pituitary adenoma
- Parathyroid
- Pancreatic tumors
What gene is MEN I associated with and where is it found?
MEN1 - Ch 11 (tumor suppressor)
MEN 2A
Autosomal Dominant
- Medullary thyroid carcinoma
- Pheochromocytoma
- Parathyroid
What gene is MEN 2A associated with and where is it found?
Ret - Ch 10 (protooncogene)
MEN 2B
Autosomal Dominant
- Medullary thyroid carcinoma
- Pheochromocytoma
- Mucosal neuromas
What gene is MEN 2b associated with and where is it found?
Ret - Ch 10 (protooncogene)
Which of the following is true regarding multiple
endocrine neoplasia (MEN) I syndrome?
A. Virtually all patients develop medullary thyroid
carcinoma
B. The genetic abnormality involves the RET gene
C. The genetic abnormality involves a protooncogene
D. Many patients develop parathyroid hyperplasia
E. Some patients may have a Marfanoid habitus
D. Many patients develop parathyroid hyperplasia
