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F3 E4 Shea > 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
Q

Succinate Dehydrogenase (SDHx)- related pheos:

  • What 2 catecholamines do SDHB pheos secrete?
A

NE and Dopamine

26
Q

Do SDHB or SDHD pheos come from Dads side?

A

SDHD

27
Q

Succinate Dehydrogenase (SDHx)- related pheos: SDHD or SDHB PHEOS?

  • most present as head and neck PGLS
  • Most don’t secrete catecholamines
  • >99% benign
A

SDHD pheos

28
Q

What are the 3 locations of neuroendocrine tumors?

A
  1. respiratory tract
  2. Pancreas
  3. Anywhere along GI tract
29
Q

What are the 6 neuroendocrine tumors?

A
  1. Gastrinoma
  2. Insulinoma
  3. Glucagonoma
  4. VIPoma
  5. Somatostatinoma
  6. Carcinoid syndrome
30
Q

What syndrome causes the following:

  • Flushing
  • Diarrhea
  • Wheezing
  • R-sided valvular disease
A

Carcinomid syndrome

31
Q

Carcinoid syndrome is caused by tumor secretion of what

A

seretonin and other vasoactive peptides (histamine, prostaglandins, etc)

32
Q

What is the MC location of tumors in carcinoid syndrome

A

lungs

33
Q

How do you diagnose carcinoid syndrome?

A

urinary excretion of 5-HIAA

34
Q

Which NET has the follwing clinical presentation?

Zollinger-Ellison Syndrome:

  • Abd pain (Peptic ulcer disease, GERD)
  • Diarrhea
A

Gastrinoma

35
Q

What does a Gastrinoma secrete

A

gastrin

36
Q

Which NET would you use the Secretin test to diagnose? What would you see

A

Gastrinoma

Would see Gastrin rise

37
Q

Which NET would you see:

Elevated gastrin and low stomach pH

A

Gastrinoma

38
Q

What is the MC functioning islet cell tumor of pancreas

A

Insulinoma

39
Q

How will a patient with an insulinoma present?

A

Severe hypoglycemia

(must have whipple triad for it to be true hypoglycemia: Fasting hypoglycemia, neuroglycopenic sxs, improvement after glucose replacement)

40
Q

What is the Whipple triad? Which NET is this a common presentation of

A
  • Whipple triad:
    1. Fasting hypoglycemia
    2. Neuroglycopenic sxs (confusion, blurr. vision, etc)
    3. Improvement after glucose replacement
  • Seen in Insulinoma
41
Q

How do you dx Insulinoma

A

72 hr fast

Insulin, C-peptide and proinsulin levels NOT suppressed with hypoglycemia

42
Q

What 2 tests can be used to localize insulinoma

A

Triphasic CT of abd

Endoscopic US

43
Q

What test is important to do before removing insulinoma

A

Calcium-stimulated arteriogram

(to see if tumor is causing sxs)

44
Q

How do you treat an insulinoma

A
  • Surgery
    • enucleation (for small/benign tumors)
  • If can’t do sx: Medical
    • Frequent snacking
    • Diazoxide
    • Octreotide
    • Verapamil (CCB)
    • etc
45
Q

Presentation of which NET?

  • Watery diarrhea
  • hypokalemia
  • achlorhydria
A

VIPoma

(this produces cholera like sxs)

46
Q

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

A

VIPoma

47
Q

How do you diagnose VIPoma

A

elevated VIP levels

48
Q

presentation of which NET?

  • Hyperglycemia
  • Migratory necrolytic erythema (pic)
  • Diarrhea
A

Glucagonoma

49
Q

If you have a patient with “uncontrolled diabetes” and the following skin presentation on his leg, what NET should you be thinking?

A

Glucagonoma

50
Q

How do you dx glucagonoma

A

Elevated glucagon levels

51
Q

Presentation of what:

  • Diabetes
  • Gallbladder disease
  • Diarrhea
  • hyperglycemia
A

Somatostatinoma

(these are all side effects of Octreotide so should make sure they are not taking this medication)

52
Q

After having a positive biochemical test for any of the NETs (Carcinoid syndrome, gastrinoma, insulinoma, VIPoma, Glucagonoma, Somatostatinoma) what should you order?

A

CT or MRI to localize tumor

(could also do PET or octreoscan)

53
Q

What endocrine syndrome is associated with Insulinomas and glucagonomas

A

MEN1

F3 E4 Shea (12 decks)

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