Adrenal Pathology Flashcards
What’s the most important cause of ectopic ACTH secretion?
Small cell lung cancer.
When you have an adrenal adenoma (esp. corticotroph), what does the rest of the gland often look like?
Rest of gland looks atrophic, because ACTH etc. will be suppressed by the hormones the adenoma is producing.
You guys all know what malignant tumors look like, right?
Yeah. Hemorrhage, necrosis, atypical nuclei, all that stuff.
What makes adrenal cortical carcinomas nasty pieces of shit?
They have a strong propensity for vascular invasion - often invading the IVC and right atrium. Assholes.
What hormones do adrenal cortical carcinomas make?
It varies. They can make all, or none. Cortisol-producing is most common.
Factors contributing to worse prognosis for adrenal cortical carcinoma?
Large size. Lots of mitosis. Loss of p53. High Ki-67. Venous invasion.
What are the criteria for rating malignancy of adrenal cortical carcinoma (ACC)?
The Weiss Criteria.
4 genetic syndromes associated with ACC? (I don’t want to memorize them either, but she said it’s good to know…)
Li-Fraumeni syndrome (loss of p53)
Beckwith-Wiedemann syndrome
Familial Adenomatous Polyposis coli - loss of APC
Multiple Endocrine Neoplasia 1 - loss of Menin
How can ACC be a cause of fever of unknown origin?
Necrosis -> inflammatory reaction.
What are adrenal tumors-UMP?
Tumors of Uncertain Malignant Potential: Weiss criteria puts them between adenoma and ACC. Most are benign, but some progress.
3 types of hyperplasia of the adrenal cortex? Sex bias for each? Degree of weight change of gland relative to normal?
Simple diffuse (more in women) - 2x normal weight. Bilateral Nodular (more in women) - about 3x normal weight. Ectopic (more in men) - about 4x normal weight.
How do the causes of hypercortisolism vary between adults and children?
Adults more likely to have hyperplasia.
Children more likely to have ACC (if they have hypercortisolism).
What do aldosteronomas look like grossly? Histologically?
Benign, egg-yolky things.
Histologically, the cells have lots of space in the cytoplasm as is seen in the zona glomerulosa.
How much of each adrenal cortex must be destroyed before Addison’s happens?
About 90%.
What’s the clinical triad of pheochromocytoma?
Paroxysmal hypertension
Headaches
Diaphoresis
What’s the important thing to remember about the pheo’s that occur in syndromes associated with pheo’s?
They can be in multiple organs, and are more likely to be malignant.
What’s the rule of 10% for pheo’s?
10% are bilateral
10% are extra-adrenal.
10% are malignant.
But…. then she emphasized to us that it’s not a good rule.
How is malignancy of a pheo determined? (2 ways) (Also, what’s weird about grading of pheo’s)
Presence of metastases.
PASS score.
(Pheo’s can be clearly invasive, necrotic, mitotic without being considered malignant)
Genetic syndromes associated with pheo’s? (name 3)
Multiple Endocrine Neoplasia (MEN) 2A and 2B
Von Hipple Lindau Syndrome (mutation in VHL, tumor suppressor)
Neurofibromatosis
How many people with pheo’s have a germline predisposing mutation?
25-30%
3 multiple endocrine neoplasia (MEN) syndromes?
MEN 1
MEN 2A
MEN 2B
Where to MEN 1 neoplasms occur? Which is most serious?
The 3 P’s:
Pituitary
Parathyroid
Pancreatic Islets - most serious.
Mutated gene in MEN1?
Menin
Mutation in MEN 2A and 2B?
Ret proto-oncogene.
Worst part of MEN 2A and 2B?
Medullary thyroid carcinoma.
3 areas of neoplams in MEN 2A?
Thyroid C-cells
Adrenal Medulla (pheo’s)
Parathyroids
Where does MEN 2B affect?
All the stuff of MEN 2A, plus:
Neural tissue of oral and GI
Causes skeletal and eye lens abnormalities…
How much does she miss her cat?
A lot.
