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MJ- F3 E4: Endocrine > Rare Endocrine Tumors > Flashcards

Rare Endocrine Tumors Flashcards

1
Q

Is MEN1 familial?

A

yes

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2
Q

What are the 3 tumor locations in MEN1?

A
  1. Parathyroid glands
  2. Anterior pituitary
  3. Enteropancreatic endocrine cells (e.g. gastrinoma, insulinoma)
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3
Q

MEN1 tumors result from ______ loss of function at the MEN1 gene on what chromosome?

A

two-hit loss

Chromosome 11

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4
Q

POPCORN

Which syndrome is associated with dermal tumors (facial angiofibromas, collagenomas, lipomas)

A

MEN1

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5
Q

Which syndrome is associated with leiomyomas (uterine, esophageal)?

A

MEN1

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6
Q

POPCORN

What gene is affected in MEN2?

A

RET proto-oncogene

(an activated suppressor gene- the other conditions are caused by tumor suppressor genes)

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7
Q

If there is a FHx of MEN2, what must you do

A

Screen

if + then prophylactic thyroidectomy (due to risk of thyroid cancer)

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8
Q

T/F: both MEN1 and MEN2 are germline inactivating mutations

A

true

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9
Q

POPCORN

What type of cancer is associated with both types (A and B) of MEN2?

A

Medullary thyroid cancer

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10
Q

What is the main difference b/w MEN2 Type 2A and 2B

A

Both have medullary thyroid cancer and pheochromocytomas

  • 2A has hyperparathyroidism
  • 2B has neuromas
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11
Q

What type of catacholamines are produced in Pheos in MEN2?

A

EPI or EPI and NE

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12
Q

Which syndrome is associated with increased risk of parathyroid cancer?

A

Hyperparathyroidism- Jaw Tumor Syndrome (HPT-JT

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13
Q

Which syndrome is associated with hyperparathyroidism** and **jaw tumors (cemento-ossifying fibromas)

A

HPT-JT

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14
Q

Which syndrome?

  • Germline inactivating mutation of the CDC73/HRPT2 tumor suppressor gene
A

HPT-JT

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15
Q

MEN1 vs HPT-JT:

  • Multiple or single parathyroid tumors
A
  • MEN1- multiple tumors
  • HPT-JT- Single adenoma, freq. cystic tumors
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16
Q

Is parathyroid cancer MC in MEN1 or HPT-JT syndrome?

A

HPT-JT

(rare in MEN1)

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17
Q

MEN1 vs HPT-JT:

  • Locations of neuroendocrine tumors (NET)
A
  • MEN1: parathyroid, pituitary, enteropancreatic, carcinoid
  • HPT-JT: Parathyroid only
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18
Q

What are the 2 non-endocrine findings in MEN1 vs HPT-JT

A

MEN1: dermal tumors, leiomyoma

HPT-JT: Jaw fibromas, renal cysts

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19
Q

Which syndrome has a NE producing pheochromocytoma?

A

VHL (Von HIppel-Lindau) syndrome

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20
Q

Which syndrome:

  • Autosomal dominant
  • germline
  • CNS/retinal hemangioblastoma
  • pancreatic cysts
  • Kidney cancer and cysts (renal cell carcinoma)
A

VHL syndrome

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21
Q

Other than a PHEO, what other organs are affected in VHL?

A
  • CNS/retinal hemangioblastoma
  • pancreatic cysts
  • Kidney cancer and cysts (renal cell carcinoma)
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22
Q

Which syndrome may have the following:

  • Pheochromocytoma (Epi and NE producing)
  • Neurofibromas (2+)
  • Cafe-au-lait spots (6+)
A

Neurofibromatosis type 1

23
Q

What should you consider if you find the following:

  • Pheo at younger age
  • Pheo that is extra-adrenal, multiple and metastatic
  • Pheo secreing only DOPAMINE
A

Succinate Dehydrogenase (SDHx)- related pheos

24
Q

Succinate Dehydrogenase (SDHx)- related pheos:

  • Is SDHB or SDHD malignant?
A

SDHB

(B=bad)

25
Succinate Dehydrogenase (SDHx)- related pheos: * What 2 catecholamines do SDHB pheos secrete?
NE and Dopamine
26
Do SDHB or SDHD pheos come from Dads side?
SDH**_D_**
27
Succinate Dehydrogenase (SDHx)- related pheos: **SDHD or SDHB PHEOS?** * most present as **head** and **neck** PGLS * Most don't secrete catecholamines * \>99% **_benign_**
SDHD pheos
28
What are the 3 locations of neuroendocrine tumors?
1. **respiratory tract** 2. **Pancreas** 3. Anywhere along **GI tract**
29
What are the 6 neuroendocrine tumors?
1. Gastrinoma 2. Insulinoma 3. Glucagonoma 4. VIPoma 5. Somatostatinoma 6. Carcinoid syndrome
30
What syndrome causes the following: * **Flushing** * Diarrhea * **_Wheezing_** * **R-sided valvular disease**
Carcinomid syndrome
31
Carcinoid syndrome is caused by tumor secretion of what
**seretonin** and other vasoactive peptides (**histamine, prostaglandins**, etc)
32
What is the MC location of tumors in carcinoid syndrome
lungs
33
How do you diagnose carcinoid syndrome?
urinary excretion of **_5-HIAA_**
34
_Which NET has the follwing clinical presentation?_ Zollinger-Ellison Syndrome: * Abd pain (Peptic ulcer disease, GERD) * Diarrhea
Gastrinoma
35
What does a Gastrinoma secrete
gastrin
36
Which NET would you use the **Secretin test** to diagnose? What would you see
**Gastrinoma** Would see **Gastrin rise**
37
Which NET would you see: Elevated gastrin and low stomach pH
Gastrinoma
38
What is the MC functioning islet cell tumor of pancreas
Insulinoma
39
How will a patient with an **insulinoma** present?
**Severe hypoglycemia** (must have whipple triad for it to be true hypoglycemia: Fasting hypoglycemia, neuroglycopenic sxs, improvement after glucose replacement)
40
What is the Whipple triad? Which NET is this a common presentation of
* Whipple triad: 1. Fasting hypoglycemia 2. Neuroglycopenic sxs (confusion, blurr. vision, etc) 3. Improvement after glucose replacement * Seen in Insulinoma
41
How do you dx Insulinoma
72 hr fast Insulin, C-peptide and proinsulin levels NOT suppressed with hypoglycemia
42
What 2 tests can be used to localize insulinoma
Triphasic CT of abd Endoscopic US
43
What test is important to do before removing insulinoma
Calcium-stimulated arteriogram | (to see if tumor is causing sxs)
44
How do you treat an insulinoma
* Surgery * enucleation (for small/benign tumors) * If can't do sx: Medical * Frequent snacking * **Diazoxide** * Octreotide * Verapamil (CCB) * etc
45
Presentation of which NET? * **_Watery diarrhea_** * hypokalemia * achlorhydria
**VIPoma** | (this produces cholera like sxs)
46
If you have a patient with **watery diarrhea** (similar presentation to cholera) but **DENIES recent travel** or **eating raw fish**, what should you be thinking
VIPoma
47
How do you diagnose VIPoma
elevated VIP levels
48
presentation of which NET? * **Hyperglycemia** * **Migratory necrolytic erythema** (pic) * Diarrhea
Glucagonoma
49
If you have a patient with "uncontrolled diabetes" and the following skin presentation on his leg, what NET should you be thinking?
Glucagonoma
50
How do you dx glucagonoma
Elevated glucagon levels
51
Presentation of what: * **Diabetes** * **Gallbladder disease** * **Diarrhea** * hyperglycemia
**Somatostatinoma** (these are all side effects of Octreotide so should make sure they are not taking this medication)
52
After having a positive biochemical test for any of the NETs (Carcinoid syndrome, gastrinoma, insulinoma, VIPoma, Glucagonoma, Somatostatinoma) what should you order?
**CT or MRI** to localize tumor | (could also do PET or octreoscan)
53
What endocrine syndrome is associated with Insulinomas and glucagonomas
MEN1

MJ- F3 E4: Endocrine (4 decks)

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