Suprarenal gland tumors - primal and secondary
This question is asking about hyperadrenalism, which is caused by tumours of the pituitary or the suprarenals (primary or secondary).
There are three types of hyperfunction based on the layer of the cortex affected by the tumour:
- Cushing syndrome (excess cortisol)
- Hyperaldosteronism (excess aldosterone)
- Adrenogenital syndrome (excess androgens)
Mnemonic: GFR - "salt, sugar, sex": zona Glomerulosa makes aldosterone (salt), zona Fasciculata makes cortisol (sugar), zona Reticularis makes androgens (sex). The deeper you go, the sweeter it gets.
Hypercortisolism and Cushing's
Hypercortisolism is typically manifested as Cushing Syndrome. Properly stated cushing disease is primary hypothalamic-pituitary disease associated with hypersecretion of ACTH, but it is by far the most common endogenous cause of hypercortisolism so the terms are often used interchangeably
In the majority of cases the pituitary gland contains an ACTH producing microadenoma, that does not produce a mass effect, the remaining patients have anterior pituitary corticotroph cell hyperplasia without adenoma
Primary adrenal hyperplasias (aka ACTH-independent cushing syndrome) also causes a percentage of cases
Remember: causes of Cushing syndrome:
- Exogenous (iatrogenic) glucocorticoids - the most common cause overall
- Pituitary ACTH adenoma (Cushing disease) - the most common endogenous cause
- Ectopic ACTH, e.g. small cell lung carcinoma
- Adrenal adenoma, carcinoma or nodular hyperplasia (ACTH-independent)
The morphological changes of the pituitary in cushing's are marked, we see Crooke’s hyaline change due to accumulation of intermediate keratin filaments, we can also see atrophy (incases where there is lack of stimulation, occurs with exogenous glucocorticoids or a cortisol-secreting adrenal tumour), or hyperplasia (where there is ACTH dependent cushing's syndrome). Functional adenomas or carcinomas of the adrenal cortex may also be observed.
Clinical features of cushing's are obesity, stretch marks, hypertension, moon faces, buffalo hump, atrophy of fast twitch fibers, limb weakness, hyperglycemia, glucosuria and polydipsia (mimicking diabetes).
Hyperaldosteronism
May be primary or secondary due to an extra adrenal cause. The primary form is autonomous overproduction of aldosterone, which causes decreased renin activity. Potential causes are bilateral idiopathic hyperaldosteronism (bilateral nodular hyperplasia), adrenocortical neoplasms (commonly caused by solitary aldosterone secreting adenoma known as Conn syndrome) or rarely familial hyperaldosteronism.
Aldosterone producing adenomas are almost always small, bright yellow (lipid filled) lesion, uniform in size and shape. They have spironolactone bodies within the cytoplasm of the cells (after spironolactone treatment).
Main clinical feature is hypertension.