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56 Cards in this Set

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Compare adrenal cortex and medullar in the following aspects:

- embryonic origin
- when does it migrate
- extra-adrenal tissue or not
Adrenal cortex
- embryonic origin: mesoderm
- migrate at 4-6 wks
- no extra-adrenal tissue

adrenal medulla
- embryonic origin: ectoderm
- migrate at 5th wks
- extra-adrenal tissue: paravertebral and para-aortic, organ of Zuckerkandl
Where are ectopic adrenal cortical tissue usually found?
- near kidney
- in pelvis along the path of migration from urogenital ridge
- vaginsa and coccyx
What is the clinical significance of ectopic adrenal cortical tissue?
if ACTH is elevated, ectopic tissue may get hyperplastic and hypertrophic
What is this adrenal disease?
cortical nodule
- not associated with cortical hyperfunction
- risk factors: age, HTN, diabetes
- associated with cushings(excess ACTH) or paraneoplastic ACTH by small cell carcinoma of lung
What is the difference between cortical nodule and diffuse nodular hyperplasia?
diffuse nodular hyperplasia: entire cortex is thickened
Pathogenesis of congenital adrenal hyperplasia.
- autosomal recessive biochemical defect affecting any of the 5 steps in synthesizing cortisol cholesterol. 95% exhibit absent 21-hydroxylase.
- diffuse cortical hyperplasia (especially zona reticularis)
- adrenogenital syndrome (pure virilization, salt losing disorder)
What enzyme defect is often present in most cases of congenital adrenal hyperplasia.
21-hydroxylase

- convert progesterone to 11- deoxycorticostone
- convert 17-hydroxyprogesterone to 11-deoxycortisol
How to treat congenital adrenal hyperplasia?
- replace cortisol
- early surgical correction of external sex organs for genetic females
What are some etiology of acquired cortical hyperplasia?
secondary cause
- pituitary hyperfunction: cushing syndrome (hypercortisolism)
- ectopic ACTH from small cell lung carcinoma

primary cause
- primary hyperaldolsteronism
What is this adrenal disease?

gross:
- cortical hyperplasia: yellow cortex, diffusely thickened, multinodular
micro: rich cells in zonba fasculata and reticularis
- bilateral
acquired cortical hyperplasia
What is this congenital adrenal disease?
acquired cortical hyperplasia
- diffusely thickened and multinodular cortex.
- rich cells in zona fasciculata and reticularis
What is this adrenal disease?

- weakness
- GI disturbances
- gradual skin hyperpigmentation
- hyperkalemia
- hyponatremia
- volume depletion, hypotension
Addison disease
- low cortisol
- low aldosterone
- high ACTH
List the 3 autoimmune adrenalitis.
- Addison disease
- autoimmune polyendocrine syndrome 1 (APS1): mutation in AIRE gene 21q22. candidiasis, ectodermal dystrophy, parathyroid hypofunction
- autoimmune polyendocrine syndrome 2: adrenalitis with thyroiditis or type 1 diabetes
What is this disease?

- candidiasis
- ectodermal dystrophy
- parathyroid hypofunction
APS 1 (antoimmune polyendocrine syndrome 1)
- mutation in AIRE gene 21q22
What is this disease?

- adrenalitis
- thyroiditis
APS2 (antoimmune polyendocrine syndrome 2)
What is this disease?

- adrenalitis
- type 1 diabetes
APS2 (antoimmune polyendocrine syndrome 2)
- autoimmunity against islet cells of pancreas -> type 1 diabetes
What is the function of AIRE protein? What happens when there is a genetic mutation?
- promotes self antigen in the thymus which lead to apoptosis of self-reactive T cells
- mutation leads to autoimmunity against adrenal gland (APS1)
What does the skin look like in someone with Addison disease?
- generalized hyperpigmentation: skin, palmar creases, nail beds, gingivae
What disease is this?

- septic shock in newborns due to Nisseria meningitidis
- DIC with widespread purpura
Waterhouse-Friderichsen syndrome: see adrenal homorrhage
List the types of neoplasms of the adrenal cortex.
- adenoma
- carcinoma
- myelolipoma
- metastatic carcinoma from lung and breast
What is this tumor of the adrenal cortex?

gross:
- solitary nodule, encapsulated
- small: <50gm, <5cm
cortical adenoma
- yellow (lipid laden)
- balck (lipofuscin laiden)
What cortical tumor is this?
cortical adenoma
- yellow (lipid laiden)
What cortical tumor is this?
cortical adenoma
- black (lipofuscin laiden)
Name 2 causes of primary hyperaldosteronism.
- acquired cortical hyperplasia
- cortical adenoma
What is this cortical tumor?

- large mass: 900 gm
- invade central vein
- wide range of differentiation
- atypical mitoses
cortical carcinoma
- gross: large, hemorrhage, necrosis, invasion of central vein
- mean age 50yrs.
What is this cortical tumor?
cortical carcinoma
What is this cortical tumor?
cortical carcinoma
- high mitotic rate
- atypical mitoses
What is this cortical tumor?
cortical carcinoma
- invasion of veins
- 50% show endocrine dysfunction
What is this cortical tumor?
myelolipoma
- mixed fat and hematopoietic cells
- CT: hypodense area of fat, focal bright calcifications
What are some common place to find myelolipoma?
- adrenal gland
- spleen

- most are solitary and unilateral
List the tumors associated with adrenal medulla.
- medullary hyperplasia
- neurooblastoma
- pheochromocytoma
What is this adrenal tumor?

- bilateral, grossly nodular or diffuse
- associated with MEN 2
- symptoms similar to pheochromocytoma
adrenal medullary hyperplasia
What is the most common solid organ malignancy in children? 2nd most common?
- most common: brain tumor
- 2nd most common: neuroblastoma
What is the most common malignancy in children overall?
acute leukemia
Tumor genetics of neuroblastoma.
- 17q gain
- 1p deletion
- MYC amplification
What are some sites of origin of neuroblastoma?
- adrenal medulla: 40%
- paravertebral region in abdomen: 25%
- midline alomg sympathetic chain: 20%
- posterior mediastinum: 15%
What is this tumor?

- homer-wright rosettes
- palisading rosettes
neuroblastoma
What are some common locations you find pheochromacytoma?
think midline

- intra-adrenal
- extra-adrenal: abdominal paragnaglia, organ of Zuckerkandl
What is this tumor?

- soft, tan-red
- encapsulated
- highly vascular
pheochromocytoma
- picture: dense core granules
What is this adrenal tumor?
pheochromocytoma
- zellballen arrrangement: ball of cells
What is this adrenal tumor?

- staining of chromogranin
pheochromocytoma
What is this adrenal tumor?

- gross: soft, tan-red, capsulated, highly vascular
pheochromocytoma
- nuclear pleomorphism
Compare tumor genetics of MEN2 and MEN3.
MEN2: germline mutation in RET protooncogene resulting in gain of function.

MEN3: mutation in RET affecting single AA of RET protein, altering tyrosine kinase domain.
MEN2 or MEN3?

germline mutation in RET protooncogene resulting in gain of function
MEN2
MEN2 or MEN3?

mutation in RET affecting single AA of RET protein, altering tyrosine kinase domain.
MEN3
MEN2 or MEN3?

- pheochromocytoma
- paratyroid hyperplasia
- medullary carcinoma of thyroid
- indolent
MEN2
MEN2 or MEN3?

- pheochromocytoma
- medullary carcinoma of thyroid
- mucosal neuromas
- marfanoid habitus
- aggressive
MEN3
What is this tumor?

- gross: 225 gm tumor in retroperitoneum near right kidney
- histo: similar to pheochromocytoma
extra-adrenal paraganglioma
What is this?
carotid body paraganglioma
Name some adrenal-based causes of glucocorticoid excess. (3)
- cortical adenoma
- cortical carcinoma
- micronodular hyperplasia
Name some extra-adrenal-based causes of glucocorticoid excess. (3)
- pituitary ACTH-adenoma
- ectopic ACTH syndrome
- iatrogenic steroid therapy
Name some adrenal-based causes of glucocorticoid deficiency (Addison's). (3)
- TB
- antoimmune adrenalitis
- adrenal hemorrhage
Name some extra-adrenal-based causes of glucocorticoid deficiency (Addison's). (2)
- exogenous suppression of ACTH after steroid therapy
- lesions destroying pituitary gland
Name some adrenal-based causes of mineralocorticoid excess.
- corical adenoma
- cortical carcinoma
- congenital adrenal hyperplasia
Name some extra-adrenal-based causes of mineralocorticoid excess.
- licorice intoxication: competively inhibits 11-beta-HSD
Name some adrenal-based causes of mineralocorticoid deficiency.
- TB
- autoimmune afrenalitis
- adrenal hemorrhage
- 18-hydroxylase deficiency