Lecture 95 -path of Adrenal gland and Endocrine Pancreas

Question 1

what are multiple endocrine neoplasms?

pattern of inheritence ?

Answer 1

• What are they: Group of inherited d/o that cause neoplasia or hyperplasia in several endocrine organs

AD

Question 2

MEN 1

mutation to…
affected organs

Answer 2

Mutation in MEN1 gene(tumor suppressor)

3Ps
Pancrease – ZES, Insulinoma
Pituitary - Prolactinor GH
PTH tumors

Question 3

what is common to MEN 2A and MEN 2B

mutaiton?

Answer 3

Medullary Thyroid Carcinoma, and Pheochromo

Marfinoid habitus
RET protooncogene mutations

Question 4

MEN 2A

Answer 4

Parathyroid Hyperplasia – 10-20%

Pheochromocytoma (40-50%) – bilateral, increased extra adrenal paraganglioma

Medullary Thyroid carcinoma (100%) – Mulitfocal; C cell; Calcitonin

A/w marfinoid habitus

Question 5

MEN 2B

Answer 5

Pheochromocytoma

Medullary thyroid carcinoma

Ganglioneuromatosis – Mucosal Neuromas (oral, intestinal)

a/w Marfinoid Habitus

Question 6

2 pancreatic endocrine tumors

shared histology and gross pathology

Answer 6

Insulinoma –

Gastrinoma –ZES

Coarse “salt and pepper” chromatin
Circumscribed, solid

Question 7

Clinical presentation of Insulinoma

what % are benign?

Answer 7

90% benign

episodic hypoglycemia (confusion, stupor, LOC, diplopia)

Question 8

what is ZES?

clinical presentation

assoicated with what MEN?

Answer 8

Gastrinoma – gastrin secreting tumor of to the pancrease; acid hypersecrteion causes recurrent ulcers,

severe peptic ulcers + diarrhea (malabsorption)

MEN1

Question 9

true or false - metastatic disease to adrenals is less common than primary disaese

Answer 9

false – mets are more common; frequently from lung and breast cnacers

Question 10

Cortical hyperplasia is always (unilateral/bilateral)

histology

Answer 10

Bilateral

histo – hyperplasia of fasciculata and reticularis

Question 11

§ Adrenal Cortical Adenoma

• what hormones are secreted?

how is it usually discovered?

gross path:

what are spiroolactone bodies?

-

Answer 11

Glucocorticoids or Aldo

discovered incidentally

Gross; well circumscribed

	® Laminated cytoplasic inclusions in aldo secreting adenomas due to spironolactone treatment for HTN

Question 12

Cortical Carcinoma –

gross path:

course:

Answer 12

Encapsulated, infiltrative and obliterates the rest of the gland
Necrosis, hemorrhage, cystic changes

				®Prognosis: Often invade adrenal vein, IVC, and LN 

Mets are Common: LNs, Lungs,

Median Survival: 2years

Question 13

what is a Pheochromocytoma

how does it present?

how is it diagnosed?

what % are malignant?

Answer 13

Most common tumor of adrenal medulla in adults — Chromaffin Cells

Clinical Presentation: Paroxysmal (“spells”) HTN; Tachycardia, Palpitations, HA, Sweating, tremor, and sense of apprehension

Increased catecholamines: Urine VMA and Metanephrine

10% malignant

Question 14

what is a paraganglioma?

Answer 14

® Same as Pheo, but arise in the ganglia of sympathetic chain

Question 15

Neuroblastoma

what is it?
what cells?

classic presentation?

labs?

Answer 15

§ Most common tumor of the adrenal medulla in kids (80% in pt

Question 16

Neuroblastoma

histo

how does it extend? – to where

prognosis

Answer 16

® Small blue cell tumor
® Homer Wright Rosettes (in 25% of cases)

® Extension is local: Kidney, spinal cord

Distant mets: liver, bone, LN

3 year survival – 30%