Path - Adrenals Flashcards
in Cushing syndrome, pituitary changes results from high levels of endogenous or exogenous glucocorticoids, which is termed what?
Crooke hyaline change
In Cushing syndrome, the normal granular basophilic cytoplasm of the ACTH-producing cells in the anterior pituitary becomes what?
homogenous and paler
- this alteration is the result of the accumulation of intermediate keratin filaments in the cytoplasm
what glands show one of the following abnormalities:
- cortical atrophy
- diffuse hyperplasia
- macronodular or micronodular hyperplasia
the adrenals
suppression of endogenous ACTH results in bilateral cortical atrophy, due to lack of stimulation of the zona fasciculata and reticularis by ACTH is caused by what?
exogenous glucocorticoids
- the zona glomerulosa is of normal thickness in such cases, because this portion of the cortex functions independently
when are the adrenals either are hyperplasic or contain a cortical neoplasm?
in cases of endogenous hypercortisolism
What is found in individuals with ACTH-dependent Cushing syndrome?
diffuse hyperplasia
- both glands are enlarged, either subtly or markedly
- the adrenal cortex is diffusely thickened and variably nodular
microscopically, the hyperplastic cortex demonstrates an expanded “lipid-poor” zona reticularis, comprising compact, eosinophilic cells, surrounded by an outer zone of vacuolated “lipid-rich” cells, resembling those seen in the zona fasiculata
- any nodules present are usually composed of vacuolated lipid rich cells, resembling those seen in the zona fasciculata
diffuse hyperplasia
in this type of hyperplasia, the adrenals are almost entirely replaced by prominent nodules of varying size, which contain an admixture of lipid-poor and lipid-rich cells
- unlike diffuse hyperplasia, the areas between the macroscopic nodules also demonstrate evidence of microscopic nodularity
macronodular hyperplasia
this type of hyperplasia is composed of 1-3cm darly pigmente micronodules, with atrophic intervening areas
- the pigment is believed to be lipofuscin, a wear and tear pigment
micronodular hyperplasia
these neoplasms that cause Cushing syndrome may be malignant or benign
- functional adenomas or carcinomas of the adrenal cortex as the source of cortisol are not morphologically distinct from nonfunctioning adrenal neoplasms
- the the benign and malignant lesions are more common in women in their 30’s-50’s
primary adrenocortical neoplasms
- in benign and malignant tumors, the adjacent adrenal cortex and that of the contralateral adrenal gland are atrophic as a result of suppression of endogenous ACTH by high cortisol levels
these tumors are yellow surrounded by thin or well-developed capsules, and most weight less than 30gm
- microscopically they are composed of cells that are similar to those encountered in the normal zona fasciculata
adrenocortical adenomas
these tumors tend to be larger than adenomas, are unencapsulated masses frequently exceeding 200-300gm, that have anaplastic characteristics
adrenocortical carcinomas
what is the most common cause of hypercortisolism?
exogenous administration of steroids
what is most often is secondary to an ACTH-producing pituitary microadenoma (Cushing disease), followed by primary adrenal neoplasms (ACTH-independent hypercortisolism) and paraneoplastic ACTH production by tumors (small cell lung cancer)
endogenous hypercortisolism
how do the morphologic features in the adrenal glands vary in Cushing syndrome?
they vary from bilateral cortical atrophy (in exogenous steroid-induced disease), to bilateral diffuse or nodular hyperplasia (most common finding in endogenous Cushing syndrome), to an adrenocortical neoplasm
these tumors are almost always solitary, small, well-circumscribed lesions, more often found on the left than on the right
- they tend to occur in the 30-40’s, and in women more than men
- they are often burried within the gland and do not produce visible enlargement
- they are bright yellow on cut section and are composed of lipid-laden cortical cells that more closely resemble fasciculata cells than glomerulosa cells
- the cells tend to be uniform in size and shape, occasionally there is modest nuclear and cellular pleomorphism
aldosterone-producing adenomas
what is a characteristic feature of aldosterone-producing adenomas?
the presence of eosinophillic, laminated cytoplasmic inclusions, known as SPIRONOLACTONE BODIES
- found after treatment with antihypertensive drug spironolactone
in contrast to cortical adenomas associated with Cushing syndrome, those associated with hyperaldosteronism do not usually suppress what?
ACTH secretion
- therefore the adjacent adrenal cortex and that of the contralateral gland are not atrophic
this type of hyperplasia is marked by diffuse and focal hyperplasia of cells resembling those of the normal zona glomerulosa
- the hyperplasia is often wedge-shaped, extending from the periphery toward the center of the gland
- the enlargement may be subtle and as a rule an they must be carefully excluded as the cause for hyperaldosteronism
bilateral idiopathic hyperplasia
in all cases of what, the adrenals are bilaterally hyperplastic, sometimes increasing 10-15 times their normal weights because of the sustained elevation in ACTH
congenital adrenal hyperplasia
the adrenal cortex is thickened and nodular, and on cut section the widened cortex appears brown, because of total depletion of all lipid
- the proliferating cells are mostly compact, eosinophilic, lipid-depleted cells, intermixed with lipid-laden clear cells
- hyperplasia of corticotroph (ACTH-producing) cells is present in the anterior pituitary in most persons with CAH
CAH
what are the two settings that the adrenal cortex can secrete excess androgens
- adrenocortical neoplasms (usually virilizing carcinomas)
2. CAH
what consists of a group of autosomal recessive disorders, characterized by defects in steroid biosynthesis, usually cortisol
- the most common type is caused by deficiency of the enzyme 21-hydroxylast
CAH
what does reduction in cortisol production cause?
a compensatory increase in ACTH secretion, which in turn stimulated androgen production
- androgens have virilizing effects, including masculinization in females (ambiguous genitalia, oligomenorrhea, hirsutism), precocious puberty in males, and in some instances, salt wasting and hypotension
bilateral hyperplasia of the adrenal cortex is characteristic, and a subset of 21-hydroxylase-deficient patients also demonstrate what?
adrenomedullary dysplasia
