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Foundations 3 > MEN 1 > Flashcards

MEN 1 Flashcards

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1
Q

How do MEN 1 occur?

A

Tumors result from Two-Hit Loss of Function at the MEN 1 Gene.

Meaning the other chromosome cannot suppress the tumor genes

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2
Q

How are Multiple Endocrine Neoplasia Type 1 acquired?

A

Familial 90%

Sporadic 10%

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3
Q

Which tumor suppressor protein is needed to prevent MEN 1

A

Menin

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4
Q

What is the most common MEN 1 tumor?

A

Parathyroid Hyperplasia 90%

multiple tumors*

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5
Q

What are the 3 most common types of MEN 1 tumors

A

Three P’s

Parathyroid Hyperplasia 90% (Hyperparathyroidism)

Pancreatic tumor

Pituitary tumors

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6
Q

What are examples of Non-Hormonal Tumors

A 
Facial angiofibroma (85%)
Truncal collagenoma (70%)
Lipoma (30%)
Leiomyoma (10%)
Meningioma (5%?)
Ependymoma (1%)
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7
Q

What are first signs see in patients with Parathyroid Hyperplasia?

A

Hypercalcemia

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8
Q

What are examples of Pacreatic Endocrine tumors?

A 
Gastrinomas
Insulinomas
Glucagonamos
VIPomas
Somatostatinomas
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9
Q

What is the treatment of Parathyroid hyperplasia?

A

Parathyroidectomy

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10
Q

What are some examples of Non-classical tumors in MEN 1

A

Dermal tumors
Angiofibromas
Collagenomas
Lipomas

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11
Q

How MEN 2 occur?

A

Autosomal dominant mutation of RET proto-oncogene (just need one hit)

Once activated you now have this receptor forever that causes proliferation of cells

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12
Q

What kind of tumors occur with MEN Type 2A?

What is a definitive treatment

A

Medullary thyroid CA
Pheochromocytoma
Hyperparathyroidism

Total Thyroidectomy by 5-6 years old

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13
Q

What kind of tumors occur with MEN Type 2B

A

Medullary thyroid CA
Pheochromocytoma
Multiple neuromas
Marfanoid habitus

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14
Q

What are Pheochromocytomas?

Where do they mostly occur

A

tumors that secrete excess catecholamines (Epi or Epi/NE)

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15
Q

How do Hyperparathyroid-Jaw Tumor Syndromes occur?

A

associated with germline inactivating mutation of the
CDC73/HRPT2 tumor suppressor gene

Results in loss of parafibromin tumorsuppressor protein function

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16
Q

Where do Hyperparathyroid-Jaw tumor Syndrome occur?

A

Parathryoid, jaw, kidney, uterus

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17
Q

What is another name for Hyperparathyroidism-Jaw Tumor Syndrome?

A

Cemento-Ossifying Fibroma of the Maxilla

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18
Q

What are findings that are common in HPT-JT syndrome?

A

Adenomas

Frequent cystic tumors

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19
Q

How do Von Hippel-Lindau syndromes occur?

Study These Flashcards
A

Autosomal Dominant disorder of VHL tumor-suppressor gene

20
Q

What are characteristics of Von Hippel Lindau syndrome?

Study These Flashcards
A

Up to 50% of patients will have bilateral pheochomocytomas

NE producing tumors

21
Q

How do Neurofibromatosis type 1 occur

Study These Flashcards
A

Inherited mutation of NF1-tumor suppressor gene

22
Q

What are clinical manifistations of Neurofibromatosis type 1?

Study These Flashcards
A

Pheochromocytoma (2-5%) (EPI & NE producing tumors)

Neurofibromas (2 or more)

Optic glioma
Lisch nodules (iris hamartomas, 2 or more)

*Cafe-au-lait macules (6 or more)

23
Q

What are Succinate Dehydrogenase related Pheochromocytomas

Study These Flashcards
A
  1. PHEOs in younger age
  2. PHEOs often exrtra-adrenal and metastatic
  3. PHEOs may secrete only dopamine
24
Q

What is the different between SDHB and SDHD Pheochromocytomas

Study These Flashcards
A

SDHB:

  • > 95% extra-adrenal, often secrete NE and DA
  • Malignant at initial diagnosis
  • Fx 10%

SDHD

  • Most of them present as head and neck PGLs
  • Most do not secrete catecholamines and are 99% benign
25
How do Carcinoid syndromes come about?
Due to tumor secretion of serotonin and other vasoactive peptides (histamine, prostaglandins
26
What are clinical symptoms from Carcinoid syndrome?
Flushing Diarrhea Wheezing Right sided valvular heart diseas
27
What are test can be used to diagnose Carcinoid syndrome
Urinary excretion of 5-HIAA (serotonin metabolite)
28
What do Gastrinomas cause a rise in?
Gastrin --> hormone that stimulates gastric acid secretions
29
What are signs and symptoms of Gastrinomas
Zollinger-Ellison syndrome Abdominal pain Peptic ulcers Gastroesophageal reflux Diarrhea
30
What test can be used to detect Gastrinomas
Very high gastrin and low stomach pH Secretin Test: Normal response= Gastrin decreases Response in gastrinoma=Gastrin rises
31
Where do Insulinomas occur
Neuroendocrine tumor of the pancreas derived from beta cells
32
What is the most common symptom from Insulinomas
Hypoglycemia High insulin in the face of low blood glucose
33
What is Whipples triad and what does this indicate
Used to diagnose Insulinomas 1. Fasting hypoglycemia 2. Neuroglycopenia symptoms (confusion, h/a, blurred vision, seizures, amnesia, coma) 3. Improves after glucose replacement
34
Elevated labs seen in Insulinomas
Insulin, c-peptide, proinsulin levels are not suppressed with hypoglycemia
35
How are Insulinomas diagnosed?
72 hour fast NPO except non-caloric beverages and essential medications End test when patient has plasma glucose < 50 mg/dl with neuroglycopenic symptoms or 72 hours elapse
36
How can insulinomas be detected with imaging
1. Triphasic CT Abdomen | 2. Endoscopic ultrasound (wire with light)
37
If you can't locate an insulinomas what other imaging tool can you use to detect the tumor
Calcium Stimulated arteriogram injection of calcium stimulates release of insulin so you can detect where insulin is secreted the highest in the pancreas
38
Treatment for Insulinomas?
Surgical removal
39
What medical agents can be used to treat insulinomas?
Frequent snacking to avoid prolonged fasting Diazoxide- acts directly on beta cells to suppress insulin secretion Ocreatide- somatostatin analog, use if hypglycemia refractory to diazoxide
40
What is characteristic of VIPoma (Vasoactive Intestinal Peptide tumor)?
WHDA syndrome Watery diarrhea Hypokalemia Achlorhydria (hypochlorhydria)
41
How are VIPoms tested?
VIP levels
42
What is a clinical manifistation of a Glucagonoma
Migratory necrolytic erthema Hyperglyemia
43
What test can be used to diagnose Glucagona
Test glucagon levels
44
What is being secreted in Somatostatinomas? How can this be tested for?
Somatostatin Somatostatin levels
45
If biochemical tests are positive for Somatostatins what must you do next?
``` Pursue imaging: CT scan or MR or PET Octreoscan ```
46
What syndromes can occur from Somatostatinomas
Diabetes Gallbladder disease Diarrhea
47
Which cells can MEN 1 tumors disperse through?
Gastrin cells (located in duodenal submucosa maybe?)

Foundations 3 (25 decks)

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