Pathology

Front 1

Neuroblastoma

Back 1

- MALIGNANT catecholamine-producing tumor of early childhood
- urinary catecholamaines and metabolites are same as those seen in pheochromocytoma
- causes hypertension
- origicantes in adrenal medulla and presents as abdominal mass

Front 2

describe the genetics behind neuroblastoma

Back 2

- occasionally converts into more differentiated "ganglioneuroma"
- tumor is characterized by amplification of N-myc oncogene with thousands of copies per cell
- malignant cells sometimes differentiate into benign cells (seen as a reduction in gene amplification)

Front 3

describe the N-myc gene seen in neuroblastoma

Back 3

- thousands of copies of N-myc in a cell
- amplification results in karyotypic changes (homogeneously staining regions or double minute chr)
- number of N-myc genes correlates to the aggressiveness of the tumor

Front 4

list the endocrine tumors (islet cell tumors)

Back 4

1. Insulinoma (beta cell tumor)
2. Gastrinoma
3. Glucagonoma (alpha cell tumor)

Front 5

insulinoma (beta cell tumor)

Back 5

- most common islet tumor
- can be either benign or malignant
- greatly increased insulin secretion
- increased C-peptide levels
- see Whipple triad

Front 6

how do you differentiate endogenous insulin from exogenous insulin administration?

Back 6

- look for levels of C-peptide
- C peptide is broken off from the pre-insulin molecule during the synthesis of insulin
- high C-peptide is increased in patients with insulinoma
- not seen in the bolus given with commercial insulin

Front 7

Whipple triad

Back 7

1. episodic hyperinsulinemia and hypoglycemia
2. CNS dysfunction related to hypoglycemia (confusion, anxiety, stupor, convulsions, coma)
3. reversal of CNS symptoms with glucose administration

Front 8

what CNS changes do you see with hypoglycemia?

Back 8

- confusion
- anxiety
- stupor
- convulsions
- coma

Front 9

Gastrinoma

Back 9

- a type of islet cell tumor
- often malignant
- can occur in extrapancreatic sites
- results in gastrin hypersecretion and hypergastrinemia
- associated with Zollinger-Ellison syndrome

Front 10

Zollinger-Ellison syndrome

Back 10

- gastric hypersecretion of HCl
- recurrent peptic ulcer disease
- hypergastrinemia

Front 11

Glucagonoma (alpha cell tumor)

Back 11

- rare islet cell tumor
- results in secondary DM
- causes skin lesion called necrolytic migratory erythema

Front 12

when do you see necrolytic migratory erythema?

Back 12

a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower abdomen, buttocks, perineum, and groin.

Front 13

VIPoma

Back 13

- rare islet cell tumor
- tumor that secretes vasoactive intestinal peptide (VIP)
- assocatied with the WDHA syndrome: Watery Diarrhea, Hypokalemia, and Achlorhydria

Front 14

WDHA syndrome (Verner-Morrison sydrome)

Back 14

- aka pancreatic cholera
- Watery Diarrhea, Hypokalemia, Achlorhydria
- seen in VIPoma (an islet cell tumor)

Front 15

Multiple Endocrine Neoplasia (MEN) Syndromes

Back 15

- autosomal dominant
- more than one endocrine organ is HYPERfunctional
- may be associated with hyperplasias or tumors
1. MEN I (Wermer syndrome)
2. MEN IIa (Sipple syndrome)
3. MEN IIb (MEN III)

Front 16

MEN I (Wermer syndrome)

Back 16

- hyperplasias or tumors of the PITUITARY, PARATHYROID, or PANCREATIC islets
- can also include hyperplasias of the thyroid or adrenal cortex
- pancreatic component can manifest as: Zollinger- Ellison syndrome, hyperinsulinism, pancreatic cholera
- linked to mutations in the MEN I gene

Front 17

MEN IIa (Sipple sydrome)

Back 17

- pheyochromocytoma, medullary carcinoma of the thyroid, and hyperparathyroidism from hyperplasia or tumor
- linked to mutations in the ret oncogene

Front 18

MEN IIb (MEN III)

Back 18

- pheochromocytoma, medullayr carcinoma, and multiple mucocutaenous neuromas or ganglioneuromas
- does NOT include hyperparathyroidism (like MEN IIa)
- linked to mutations in the ret oncogene (not the same ones as MEN IIa)