Adrenal Pathology

Question 1

What are the main pathological signs of adrenal insufficiency (Addison’s Disease)

Answer 1

Destruction of cortical cells

Chronic inflammatory cells (autoimmune process)

Question 2

What is the pathology definition of pheochromocytoma?

Answer 2

Catecholamine secreting tumor arising from the chromaffin cells of adrenal medulla

Question 3

What are the clinical features of pheochromocytoma?

Answer 3

90% sporadic tumors

Excess catecholamine –> hypertension, palpitations, headaches, diaphoresis, flushing, anxiety, nausea, constipation, pain and epitaxis, elevated urine catecholamine

Question 4

What is the 10% rule and why was it wrong?

Answer 4

Refers to clinical features of pheochromocytoma; underestimates familial distribution of disease at 10%

There are many more familial pheochromocytomas than previously thought (20-35%)
*passed in autosomal dominant fashion

Question 5

What are the familial syndromes associated with pheochromocytoma?

Answer 5

Multiple endocrine neoplasia (MEN) type 2A or 2B

Von Hippel Lindau syndrome

Neurofibromatosis

20-30% germline mutation in SDHB, SDHD, VHL, RET or NF1 gene

Question 6

Describe gross findings of pheochromocytoma

Answer 6

Firm cut surface, vascular, well circumscribed

Question 7

Describe microscopic findings of pheochromocytoma

Answer 7

Growth pattern = nested, trabecular or solid

“Zellballen or Organoid pattern” = round groups of tumor cells supported by rich vascular network

Nuclei of cells showing “salt and pepper” aka neuroednocrine network

Question 8

What are the three main MEN syndromes?

Answer 8

Men 1 (Wermer syndrome)
Men 2A (Sipple syndrome)
Men 2B

Question 9

What is MEN 1 Syndrome (Wermer Syndrome)

Answer 9

Multifocal hyperplasia of
Pituitary
Parathyroid
Pancreatic islet cells r

Different family members may manifest different symptoms due to organ involved

Question 10

What is the gene involved in MEN 1 (Wermer Syndrome)

Answer 10

MENIN with multiple sites of malignancy

Question 11

MEN 2A and 2B are both related to which mutation?

Answer 11

Mutations in ret protoconcogen

Question 12

In MEN 2A and 2B what is the most prognostically important lesion (can be fatal)

Answer 12

Medullary thyroid carcinoma

Question 13

What are the main differences between MEN 2A and 2B?

Answer 13

Both consist of neoplasia and hyperlasia of thyroid C cells and adrenal medulla ONLY MEN 2A includes parathyroids

MEN 2B includes neoplasia of neural tissue of oral and GI, skeletal and eye lens abnormalities (Marfanoid Habitus)