M&n Week 4: Pathology Of The Adrenal Medulla

Front 1

Which cells secrete calcitonin?

Back 1

Parafollicular "C" cells of thyroid gland

Front 2

Merkel discs in the skin are derived from what cells?

Back 2

Neural crest cells

Front 3

Medullary carcinoma of the thyoroid is derived from which cells?

Back 3

Parafollicular cells
(C cells)

*Secrete calcitonin

Front 4

Medullary carcinoma of the thyroid can occur in association with other endocrine neoplasms as part of what syndrome?

Back 4

Multiple Endocrine Neoplasia Syndrome
(MEN)

Front 5

Group of genetically inherited diseases resulting in proliferative lesions of multiple endocrine organs.

Back 5

Multiple Endocrine Neoplasia Syndrome
(MEN)

Front 6

Which specialized neuroendocrine cells compose the adrenal medulla?

Back 6

Chromaffin cells

Front 7

The adrenal medulla is composed of two cell types derived from neural crest cells. What are they?

What type of neoplasms arise from these cell types?

Back 7

1. Ganglion cells --> neuroblastic tumor
2. Pheochromocytes --> pheochromocytoma

Front 8

Neuroblastomas are a family of "neuroblastic tumors" found in which 2 locations?

Where do most of these tumors arise?

Back 8

1. Adrenal medulla
2. Autonomic ganglia

*Most arise in adrenal medulla or along sympathetic ganglia in abdomen or posterior mediastinum

Front 9

Neuroblastomas are most common in which individuals?

Back 9

Infants and children
(< 5 yo. Median age at diagnosis = 18 mo)

Front 10

How do neuroblastomas generally present in the clinic?

Back 10

Children < 2yo
1. Abdominal mass
2. Weight loss
3. Malaise
4. Fever

Older children:
symptoms from metastases to bone, lung, or GI tract

Front 11

Most neuroblastomas secrete what hormones?

Back 11

Catecholamines

Front 12

What does the laboratory diagnosis of neuroblastoma involve?

Back 12

Elevated metabolic catecholamine by-products detected in urine:
1. Metanephrine, normetanephrine
2. Vanillylmandelic acid (VMA)
3. Homovanilic acid (HVA)

Front 13

"Small, blue, round cell tumors" are indicative of what type of endocrine tumor?

List 3 other tumors that should be part of a differential diagnosis based on this histologic finding.

Back 13

Neuroblastoma

Other "small, blue, round tumors:"
1. Ewings sarcoma
2. Wilms tumor
3. Lymphoma (small-cell)

Front 14

What is the typical histologic feature of neuroblastomas?

Back 14

Homer-Wright rosettes
(tumor cells arranged about central area filled with fibrillary material)

Front 15

What is the typical clinical course of neuroblastomas-- at what point do they become non-resectable?

Back 15

Usually age 2 is the cut-off:
Infants <2yo = tumors may remain localized and amenable to complete resection

Older children= often unresectable and may present with widespread metastases

Front 16

What are the common sites for neuroblastoma metastases?

Back 16

1. liver
2. lung
3. bone marrow

Front 17

Describe the prognosis of neuroblastomas based on age.

Back 17

Children < 18 mo = excellent prognosis (regardless of stage)

Chilren > 5 yo = poor prognosis (regardless of stage)

Front 18

Compare neuroblastomas and pheochromocytomas ability to cause hypertension.

Back 18

Neuroblastomas --> not hypertensive

Pheochromocytomas --> hypertensive

Front 19

What is the dominant clinical manifestation of pheochromocytomas?

Back 19

Hypertension
(90% cases)

Front 20

What are the clinical presentations of pheochromocytoma?

Back 20

1. Sustained elevated BP
2. Paroxysmal episodes of abrupt increase in BP
3. Tachycardia
4. Headaches
5. Sweating
6. Tremor
7. Anxiety

(All symptoms of increased catecholamines)

Front 21

Which condition is a surgically correctable form of hypertension?

Back 21

Pheochromocytoma

Front 22

What would lab results show in a patient with pheochromocytoma?

Back 22

Elevated urinary catecholamine metabolites:
1. Vanillylmandelic acid (VMA)
2. Metanephrine

Front 23

List the "rule of 10s" associated with Pheochromocytoma.

Back 23

1. 10% extra-adrenal
2. 10% of sporadic adrenal pheochromocytomas are bilateral in adrenal glands
3. 10% biologically malignant
4. 10% familial (autosomal dominant disease or in association with MEN syndrome)

Front 24

What type of tumor arises in extra-adrenal paraganglia and is FUNCTIONAL?

Back 24

Extra-adrenal pheochromocytoma

Front 25

What type of tumor arises in extra-adrenal paraganglia and is NON-FUNCTIONAL?

Back 25

Paraganglioma

Front 26

Which 2 locations do extra-adrenal neuroblastomas typically develop?

Back 26

1. Paravertebral paraganglia
2. Paraganglia related to great vessels of head and neck (aorticopulmonary chain)

Front 27

Are neuroblastomas located in paravertebral paraganglia usually functional or non-functional?

Back 27

(paravertebral paraganglia-- i.e. organs of Zunkerkandl and rarely urinary bladder)

*Typically functional --> release catecholamines

Front 28

Are paragangliomas related to paraganglia of the great vessels of the head and neck typically functional or non-functional?

Back 28

Nonfunctional

Front 29

Which type of extra-adrenal paraganglioma is more likely to secrete catecholamines-- paravertebral or those associated with great vessels of head and neck?

Back 29

Paravertebral paragangliomas are usually functional, so they are more likely to secrete catecholamines

Front 30

Are tumors more common in the endocrine or exocrine pancreas?

Back 30

Exocrine pancreas
(islet cell tumors represent only 2% of all pancreatic neoplasms)

Front 31

List the four major and two minor cell types located in the islets of Langerhans.

Back 31

Major:
Alpha, beta, delta (somatostatin), and PP (pancreatic polypeptide)

Minor:
1. D1 Cells --> VIP
2. Enterochromaffin cells--> serotonin

Front 32

What type of tumors cause carcinoid syndrome?

Back 32

Tumors derived from enterochromaffin cells of pancreas, which secrete serotonin

Front 33

What type of pancreatic cells make up VIPomas?

Back 33

D1 cells of pancreas

Front 34

Which pancreatic cells produce serotonin?

Back 34

Enterochromaffin cells

Front 35

Are islet cell tumors more common in children or adults?

Back 35

Adults

Front 36

List 3 of the most common clinical syndromes associated with functional pancreatic endocrine neoplasms:

Back 36

1. Hyperinsulinism (B-cell tumors)
2. Hypergastrinemia and Zollinger-Ellison syndrome
3. Multiple endocrine neoplasia (MEN) syndromes

Front 37

What is the most common islet cell tumor?

Back 37

B-cell tumors (insulinomas)

Front 38

Do insulinomas often cause hypoglycemia?

Back 38

Eh, not really.
Only 20% produce enough to cause hypoglycemia

Front 39

Where do gastrin-producing tumors most commonly arise?

Back 39

"Gastrinoma triangle:"
1. Pancreas
2. Peripancreatic tissue
3. Duodenum

Front 40

What are the clinical presentations of gastrinomas and Zollinger-Ellison syndrome?

Back 40

1. Diarrhea
2. Malabsorption
3. Fluid/electrolyte imbalance
4. Peptic ulcers

*Increase in gastric parietal cells--> gastric acid hypersecretion

Front 41

Gastrinomas within the wall of the duodenum associated with intractable peptic ulcers are due to what syndrome?

Back 41

Zollinger-Ellison syndrome

Front 42

What is the treatment for gastrinomas and Zollinger-Ellison syndrome?

Back 42

1. Drugs that blockade acid hypersecretion
2. Surgical resection of islet tumor

Front 43

Are the majority of gastrinomas malignant or nonmalignant?

Back 43

Malignant (>50%)

Front 44

Are most gastrinomas sporadic or found in conjuction with MEN?

Back 44

Sporadic (75% cases)

Front 45

Which gastrinomas are generally easier to resect-- sporadic or those in conjuction with MEN?

Back 45

Sporadic are easier (typically solitary)

(With MEN-1, patients often have multiple tumors or metastatic disease)

Front 46

Are Multiple Endocrine Neoplasia Syndromes autosomal dominant or recessive?

Back 46

Autosomal dominant

Front 47

MEN-1 Syndrome is also known as...?

Back 47

Wermer Syndrome

Front 48

Which endocrine organs are affected in MEN-1 Syndrome?

What is the result?

Back 48

(3 P's)

1. Parathyroid --> primary hyperparahyroidism
2. Pancreas --> islet cell tumor
3. Pituitary --> Anterior pituitary tumor (prolactinoma)

Front 49

What is the most common manifestation and initial manifestation in most patients with MEN-1 Syndrome?

Back 49

Primary hyperparathyroidism
(either hyperplasia or adenoma)

Front 50

What are the 4 dominant clinical manifestations of MEN-1 (Wermer's syndrome)?

Back 50

1. Recurrent hypoglycemia (insulinoma)
2. Intractable peptic ulcers (ZE syndrome)
3. Nephrolithiasis (PTH-induced hypercalcemia)
4. Symptoms of prolactin excess (pituitary tumor)

Front 51

List 3 manifestations of MEN IIa (Sipple syndrome).

Back 51

1. Pheochromocytoma
2. Medullary thryroid carcinoma
3. Parathyroid hyperplasia

Front 52

List 3 manifestations of MEN IIb.

Back 52

1. Pheochromocytoma
2. Medullary thyroid carcinoma
3. Mucocutaneous neuromas (often around lips)

Front 53

What type of tumor occurs in almost all patients with MEN IIa and MEN IIb?

Back 53

Medullary thyroid carcinoma

Front 54

Another name for MEN IIa is...?

Back 54

Sipple syndrome

Front 55

MEN IIa and MEN IIb are both linked to defects of which chromosome?

Back 55

Chromosome 10

Front 56

What is the leading cause of morbidity and mortality in MEN II?

How can this be prevented?

Back 56

Medullary thyroid carcinoma

*Prophylactic thyroidectomy