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Endocrinology > NEUROENDOCRINE TUMORS > Flashcards

NEUROENDOCRINE TUMORS Flashcards

1
Q

What are NET’s?

What kinds are there? 3

A

are neoplasms that arise from cells of the endocrine and nervous tissue.

Rare
Benign
Malignant

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2
Q

Neuroendocrine Tumors
can be found where?
6

A 
1. Multiple Endocrine 
Neoplasms
--MEN type I & II
2. Insulinomas
3. Gastrinomas
4. VIPomas
5. Glucagonomas
  1. Carcinoid
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3
Q

Neuroendocrine Tumors. Whats the difference between well-differentiated and undifferentiated tumors?

A

Well-Differentiated- slow growing and easliy treatable

Undifferentiated- harder to treat and faster growing

The biologic behavior of these two entities differs remarkably, and the distinction is important for planning therapy

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4
Q

MEN Syndromes are conditions that cause what?

How are they usually acquired?

A

Conditions which cause overactivity and enlargement and tumors of certain endocrine glands.

Usually inherited conditions.

Autosomal dominant – each child has 50% chance of inheriting the gene.

Families will have only one type of MEN, they are not at risk for developing another type of MEN.

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5
Q

Tumors associated with MEN 1? 3
MEN 2a? 3

MEN 2b? 3

A

Types are distinguished by patterns of organs affected
MEN 1 = parathyroid tumors, pancreatic tumors, pituitary tumors

MEN 2a = medullary thyroid cancers, pheochromocytoma, parathyroid

MEN 2b = Medullary thyroid cancers, pheochromocytoma, neuromas (no parathyroid!)

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6
Q

Almost all who inherit MEN1 develop over-activity of the what?

What is the first clinical manifestation?

What symtpoms do we see in hyperparathyroidism? 4

A

over-activity of the parathyroid.

(which is usually the first clinical manifestation.- hyperparathroidism,

bones, stones, moans and groans

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7
Q

What will develop probblems next in MEN1?

What will be over produced because of this? 2

What occurs in about 15-20% of pts with MEN1?

A

Pancreas over-activity is next. (Occurs in about 75% of patients)

  1. Gastrin over-production common after 30 years of age. (about 15% of patients)
  2. Insulin over-production common under 30 years of age. (about 15% of patients)

Pituitary adenoma occurs in about 15-20% of patients

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8
Q

Should everyone with an endocrine gland problem be tested for MEN1?

A

NO

People who have over-activity of 2 or more of the glands involved in MEN should be examined for MEN1

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9
Q

Does MEN1 cause cancer??

A

Usually not, typically benign tumors

Pancreas is most likely culprit if it does happen

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10
Q

Diagnostic for MEN?

Regular screening for MEN 1 monitoring (family history of it)?
3

A

Predictive genetic testing – (chrom 11 q 13)

Regular screening for endocrine gland overactivity:

  1. Calcium & PTH - yearly from the age of 5
  2. Prolactin
  3. Gastrin
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11
Q

Treatment for MEN 1

  1. Hyperparathyroidism?
  2. Pituitary adenomas? 4
  3. Pancreatic/Gastrointestinal tumors?
    2
A
  1. Surgery
2. 
Dopamine agonist
--Cabergoline (1st choice)
--Bromocriptine (2nd choice)
Transsphenoidal surgery
  1. Proton pump inhibitor
    Surgery
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12
Q

MEN 2a affected organs?

3

A
  1. Thyroid
  2. Adrenal (adrenal medulla)
    - -Pheochromcytomas
  3. Hyperparathyroidism
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13
Q

What is the men 2a gene abnormality?

A

RET proto-oncogene chromosome 10

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14
Q

MEN 2a prophylactic treatment?

A

Will almost certainly develop medullary thyroid cancer!!
Very aggressive
Begins early in life and grows quickly

  1. Patients with MEN2 gene should have their thyroid surgically removed while they are young.
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15
Q

Other diseases for MEN 2a?

2

A
  1. Cutaneous lichen amyloidosis

2. Hirschsprung disease (no nerve cells to keep the colon moving, no bowel movements)

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16
Q

Testing for MEN2a:

Diagnosis?

Monitoring? 3

A

Predictive genetic testing – ret protooncogene mutation

Regular screening for endocrine activity:

Screening is aimed at detecting the earliest signs of Medullary Ca.
1. pentagastrin causes raised levels of calcitonin within 2-3 minutes in affected individuals.

  1. Urinary catecholamine (adrenaline and noradrenaline)
  2. Calcium or parathyroid levels should also be obtained every 2 years.
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17
Q

MEN 2b causes what kind of tumors/ diseases?

4

A

Medullary thyroid cancers
Pheochromocytoma
Mucosal neuromas
Marfanoid Habitus

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18
Q

What is Mucosal neuromas?

How are they treated?

A

small benign tumors of nervous tissue found in the mucosa of the body (e.g. the linings of the nasal sinuses, the lips, tongue, the respiratory tract, the gastrointestinal tract, the biliary tract and the pancreatic systems).

They are treated conservatively, removed only if they cause problems (e.g. by obstruction airways or the passage of food).

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19
Q

What is Marfanoid Habitus

4

A
  1. Arachnodactyly- long fingers and extremities
  2. Tall with disproportionately long legs and arms - the span of the arms is greater than the height
  3. pectus excavatum
  4. spinal abnormalities
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20
Q

Testing forMEN 2b

2

A

Same as for MEN 2a, except that screening for parathyroid abnormalities is not done.

Therefore the pentagastrin test or medullary thyroid cancer and the urinary catecholamine tests are used.

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21
Q

Treatment of MEN 2A and MEN 2B focuses on what?

With either syndrome, it may be necessary to perform what?

If pheochromocytomas are present, they should be what?

A

surgical removal of tumors that might spread to other parts of the body or cause life-threatening biochemical disturbances.

operations in stages to minimize the overall risk of complications.

removed in a separate operation before proceeding with any other operations.

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22
Q

Confirmed medullary thyroid cancer should be treated with what? 2

In most cases, it is recommended that the lymph node dissection should be performed where?
2

A
  1. surgical removal of the entire thyroid gland,
  2. followed by careful exploration and dissection of the lymph nodes in the neck.

in the central compartment from the

  1. hyoid bone to the innominate veins and
  2. medial to the jugular veins
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23
Q

Insulinoma is a tumor of what?

Describe the pathology of the tumor and what it causes?

A

Rare beta-cell tumor (in the islets of Langerhans) that secretes insulin.

Hyperinsulinemia – not responsive to falling glucose concentrations in the fasting state – result is persistent hypoglycemia.

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24
Q

What are the signs of insulinoma?

3

A
  1. Patients eat frequently to prevent hypoglycemia
  2. Gain weight
  3. Symptoms typically begin with evidence of CNS glucose lack
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25
Q

Symptoms typically begin with evidence of CNS glucose lack. What are these symptoms?
7

A
  1. Blurred vision or diplopia
  2. Headache
  3. Slurred speech
  4. Weakness
  5. Anxiety or psychotic behavior
  6. Can have sweating and palpations
  7. Convulsions, coma
    - -When hypoglycemia is severe
26
Q

Why are the usual symptoms of hypoglycemia not too evident?

A

Hypoglycemic unawareness

27
Q

What is Whipples Triad?

3

A

Whipple’s Triad

  1. History of hypoglycemia symptoms
  2. Associated fasting blood glucose of 45 mg/dL or less
  3. Immediate recovery upon administration of glucose
28
Q

Diagnostic Tests
for Insulinoma?
3

Why isnt CT or MRI very diagnostic for Insulinoma?

A
  1. 72-hour monitored fast
    if
    –glucose levels fall to less than 40 mg/dL while insulin levels are more than 20 U/mL
    –Insulin to glucose ratio is greater than 0.4 (normal: less than .3)
    –Increased levels of C peptide and pro-insulin are also diagnostic
29
Q

With an insulinoma we have to rule out other causes of hypoglycemia such as?
5

A
  1. reactive hypoglycemia
  2. adrenal insufficiency
  3. end-stage liver disease
  4. Non-pancreatic tumors
  5. Secretly administration of oral hypoglycemics or insulin
30
Q

What are the preferred initial test for an insulinoma?
2

What is next?

Last resort?

A

Transabdominal ultrasonography and CT are our preferred initial test,

endoscopic ultrasonography

arterial stimulation with hepatic venous sampling when an insulinoma has not been localized by noninvasive techniques

Accurate preoperative localization of an insulinoma is desirable.

31
Q

Insulinoma treatment?
1

Medication treatments? 3

What do we do if its malignant?

A

Surgical resection of pancreas

Diazoxide
Octreotide
Continuous SQ glucagon infusion to prevent hypoglycemia

Malignant insulinoma - streptozotocin

32
Q

What is the MOA for diazoxide?

What is the MOA for octreotide?
4

A

(inhibits insulin secretion)

  1. inhibits secretion of many hormones, such as gastrin, CCK, glucagon, insulin, secretin, pancreatic polypeptide
  2. reduces secretion of fluids by the intestine and pancreas.
  3. reduces gastrointestinal motility and inhibits contraction of the gallbladder.
  4. It inhibits the secretion of certain hormones from the anterior pituitary.
33
Q

What does a resection treatment for insulinoma consist of?

What should also be considered for resection?

A

Resection of

  1. peripancreatic and
  2. duodenal nodes is advised in patients with probable malignant tumors.

Hepatic resection should be considered for cure or palliation in patients with metastatic tumors.

34
Q

Gastrinoma
Zollinger-Ellison Syndrome is a tumor of what? 2

What does it produce?

What are the two classifications for these kind of tumors?

A

Tumor of pancreas or duodenum

Produces excess levels of gastrin
-Gastrin stimulates gastric acid and pepsin production

Well-differentiated
Poorly differentiated

35
Q

Gastrinoma Zollinger-Ellison Syndrome symtpoms?
5

In severe cases?
3

A 
1. Frequent peptic ulcers with absence of 
  h-pylori or NSAID consumption
2. Duodenojejunitis
3. Esophagitis
4. Refractory diarrhea- due to ph levels
5. Multiple duodenal ulcers
  1. Rupture,
  2. bleeding,
  3. intestinal obstruction
36
Q

Gastrinoma
Zollinger-Ellison Syndrome
diagnosis?

What is involved in the test protocol?
6

A

Fasting gastrin
Look for results to be >1000 with ZES – This is diagnostic.

  1. Do not eat for 12 hours before test.
  2. Do not drink alcohol for 24 hours before test.
  3. Water can be consumed without limitation up to 1 hour before test.
  4. Do not chew gum or smoke cigarettes for 4 hours before the test.
  5. Stop acid reducing medications
  6. stress can affect it. rest quietly for 30 min beforehand
37
Q

What If the gastrin is >200 and less than 1000? Does the pt not have ZES?

How do we back it up?
4

Imaging studies to back it up?

A

Not necessarily

We have to back it up with testing:

  1. STomach pH
  2. Positive Secretin Provacation test
  3. gastric acid analysis
  4. Somatostatin receptor scintigraphy
  5. Endoscopic ultrasound
  6. MRI
38
Q

Positive Secretin Provacation test is performed how?
3

What do the results tell us?
2

A
  1. A baseline gastrin level is drawn
  2. An intravenous bolus of 2 to 3 U secretin per kg of body weight is administered over 30 seconds.
  3. Serum gastrin levels are then measured at 2, 5, 10, 15, and 20 minutes.
  4. Normally, no change or a slight suppression of gastrin concentration will be seen.
  5. Patients with gastrinoma demonstrate a paradoxical increase of 100 to 200 pg/mL in serum gastrin at 5 minutes with maximum levels reaching several thousand pg/mL. Approximately 90% of patients with gastrinoma will have a positive secretin test.
39
Q

A type of radionuclide scan used to find carcinoid and other types of tumors. A radioactive octreotide attaches to tumor cells that have receptors for somatostatin.

What types of imaging is this?

A

Somatostatin receptor scintigraphy

40
Q

Gastrinoma
Zollinger-Ellison Syndrome treatment?
2

A

Control gastric hypersecretion

  1. High Doses of Proton pump inhibitors
  2. Surgical removal of gastinoma
41
Q

Patients who meet criteria for operability should undergo exploration for possible removal of the tumor. Why?

A

50% of gastrinomas metastasize to lymph nodes or the liver, and are therefore considered malignant.

42
Q

What is removed in a gastrimona surgery?

2

A
  1. excision of the duodenal wall is performed if a duodenal gastrinoma is found
  2. all lymph nodes in the gastrinoma triangle are removed
43
Q

What is a VIPoma also called?

What is it and what does it secrete?

Where is it usually located?

A

Verner-Morrison Syndrome

Pancreatic neoplasm secreting vasoactive intestinal polypeptide (VIP).

Tail of the pancreas

44
Q

Functions of Vasoactive intestinal polypeptide?

7

A
  1. It helps control and send nerve signals
  2. It helps relax certain muscles along the gastrointestinal tract
  3. It increases the amount of water and electrolytes released from the pancreas and gut
  4. It triggers the release of hormones from the pancreas, gut, and hypothalamus
  5. It helps break down fat and glycogen
  6. It stimulates bile flow
  7. It blocks gastrin and gastric acid release
45
Q

Classic clinical syndrome
of VIPoma? 2

This will cause what? 3

A
  1. Severe, intermittent, watery diarrhea
  2. Flushing (vasodilation properties)
  3. Dehydration & Weakness from fluid and electrolyte losses.
  4. Decrease gastrin production
  5. Large amounts of potassium are lost in the stool.
46
Q

VIPoma: Diagnosis
Labs?

Imaging?
2

A

Serum VIP levels
>75 pg/mL

CT scans
Ultrasound

47
Q

VIPoma: Treatment

2

A
  1. Fluid loss & correction

2. Surgical Resection (tail end of pancreas)

48
Q

What is helpful in controlling the diarrhea and allowing for replacement of fluid?

A

Somatostatin analogs

-Octreotide or Lanreotide

49
Q

Glucagonoma is a tumor of what cells that secretes what?

What does this increase in the blood then?

What is a distinctive feature of this tumor?

A

Pancreatic Islet Cell tumor that secretes glucagon

Increases levels of glucose in blood

Produces a distinctive rash

50
Q

Symptoms for glucogonoma?

3

A

Those of diabetes mellitus – Type 1

  1. Weight loss
  2. hyperglycemia
  3. Necrolytic migratory erythema
51
Q

Describe the rash, Necrolytic migratory erythema, associated with glucogonoma
3

A
  1. begins as erythematous papules or plaques involving the face, perineum, and extremities.
  2. Over 7 to 14 days, the lesions enlarge and coalesce. Central clearing then occurs, leaving bronze-colored, indurated areas centrally, with blistering, crusting, and scaling at the borders.
  3. The affected areas are often pruritic and painful
52
Q

Glucagonoma diagnosis? 2

Treatment? 2

A

Diagnosis:
1. Serum glucagon level >500 pg/ml (norm 50-200)
2. Imaging to identify tumor location
MRI, CT

  1. Pancreatic resection (body and tail)
  2. Zinc ointment for skin rash
53
Q

Glucogonoma tumors tend to be of what size and malignant or benign?

A

Tend to be large tumors with metastases.

54
Q

Carcinoid Tumors
are usually without symtpoms. But if they do occur they come from wherE?

What syndrome is this associated with?

A

Sx’s from tumors in ileum & jejunum

MEN type I

55
Q

What are the most common sites for carcinoid tumors?

4

A

Ileum
Jejunum
Right Colon
Appendix

56
Q

Signs of symtpoms of carcinoid tumors?

4

A
  1. Periodic abdominal pain
  2. Flushing
  3. Diarrhea/malabsorption
  4. Wheezing and dyspnea
57
Q

How will the flushing present in carcinoid tumors?

3

A
  1. Episodic
  2. Exercise, stress, certain foods
  3. Increasing duration
58
Q

Imaging tests for Carcinoid tumors? 4

Procedures to diagnose carcinoid tumors? 2

A
  1. Radiography (Upper/Lower GI series w/ contrast)
  2. CT
  3. Octreoscan
  4. MRI
  5. Colonoscopy (yellow from lipids and fats)
  6. Histology
59
Q

Surgical management of carcinoid tumors?

2

A
  1. Appendectomy

2. Block resection w/ adjacent lymph nodes

60
Q

Medical management of carcinoid tumors?

2

A
  1. Reduction
    Chemotherapy
    Numerous chemo meds used and in combination
  2. Palliative
    Octreotide

Endocrinology (23 decks)

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