Adrenal Gland Flashcards
From what germ layer is the adrenal cortex derived?
Mesoderm
From what tissue does the adrenal medulla arise?
Neuroectoderm
What is secreted by the zona glomerulosa?
Mineralocorticoids- aldosterone
What is secreted by the zona fasciculata?
Glucocorticoids- cortisol
What is secreted by the zona reticularis?
Sex steroids- progestins, estrogens, androgens
What is the most common cause of Cushing’s syndrome?
Exogenous administration of glucocorticoids (corticosteroids)
What is Cushing’s disease?
Subset of Cushing’s syndrome:
Hypercortisolism caused by primary hypothalamic pituitary disease associated with hypersecretion of ACTH.
What is the most common cause of Cushing’s disease?
Pituitary adenoma leading to excess secretion of ACTH
Most of the time, are adrenal adenomas functional or non-functional?
Non-Functional!
If they are functional, they produce the mineralocorticoids or glucocorticoids
Generally, functional carcinomas or hyperplasia leads to secretion of sex hormones
What is Conn Syndrome?
Aldosterone producing adenoma of the adrenal gland
What is the most common cause of hyperaldosteronism?
Bilateral idiopathic hyperaldosteronism- nodular hyperplasia of the adrenal glands
What are the clinical manifestations of hyperaldosteronism?
Hypertension and hypokalemia
Describe the pathology of congenital adrenal hyperplasia
AR enzyme deficiency in the synthesis of cortisol leads to excess ACTH and consequently adrenal hyperplasia. Excess precursors build up and this leads to increased production of androgens.
Which is the most common enzyme deficiency leading to congenital adrenal hyperplasia?
21 hydroxylase
Why must withdrawal from corticosteroid therapy be tapered?
Acute withdrawal can lead to adrenal insufficiency
What is Addison’s Disease?
Destruction of the adrenal cortex with multiple etiologies leading to hypofunction
What part of the adrenal glands are destroyed in autoimmune adrenalitis?
All cortical zones
Name two inherited disorders that predispose you to an adrenal carcinoma
Li-Fraumeni - germline mutations in p53
Beckwith Wiedemann Syndrome- (overgrowth disorder)
What is the prognosis of adrenal carcinomas?
Very bad- survival less than 2 years
They have a tendency to invade the vasculature
What is the cell of origin of a pheochromocytoma?
Chromaffin cells (neuroendocrine cells) of the adrenal medulla
What is the main symptoms of a pheochromocytoma?
HTN- due to release of catecholamines
Name 6 genes associated with familial pheochromocytomas
RET (Men II and III)
NF1
VHL
SDHB, SDHC, SDHD
What is required for the definition of malignant pheochromocytoma?
Metastasis
Labile or paroxysmal hypertension is associated with which adrenal pathology?
Pheochromocytoma
Where do neuroblastomas arise?
In the adrenal medulla or extra-adrenal paraganglionic tissues
How do neuroblastomas appear on histology?
Small, round blue cells arranged in rosettes
What is the inheritance pattern of MEN I?
Autosomal dominent
What is the gene affected in MEN I?
MENI gene: 11q13 - a tumor suppressor gene
What are the three organs involved in MEN I?
3 Ps
Pancreas, parathyroid, and pituitary
What is the most common manifestation of MEN I?
Primary hyperparathyroidism- hyperplasia or adenoma
What is the leading cause of death in MEN I?
Endocrine tumors of the pancreas
They are functional tumors and they metastasize
What is the most commonly secreted hormone from the pituitary adenomas of MEN I
Prolactin
What is the inheritance pattern of MEN 2A?
Autosomal Dominant
What is the gene affected in MEN 2A?
RET protooncogene (10q11.2)
What are the three organs affected in MEN 2A?
Thyroid- medullary carcinomas
Adrenal Medulla- pheochromocytomas
Parathyroid- hyperthyroidism
What is the most common primary manifestation of MEN 2A?
Medullary carcinoma of the thyroid is seen in virtually all untreated cases in the first 2 decades
What differentiates MEN 2A from MEN 2B?
No parathyroid manifestations, but instead there are mucosal ganglioneuromas with marfanoid habitus observed
What is the recommendation for patients found to have Ret mutations?
Prophylactic thyroidectomy to prevent the onset of medullary carcinomas