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Foundations-Endocrine > Rare Endocrine Tumors > Flashcards

Rare Endocrine Tumors Flashcards

1
Q

MC etiology in MEN1

A

Familial= 90%

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2
Q

Name the gene mutation in MEN1

A

LOSS of menin tumor suppressor protein

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3
Q

When is it considered that you have the DISEASE in MEN1

A

2nd hit

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4
Q

List the MC tumors in MEN1

A
  1. Parathyroid Hyperplasia
  2. Pancreatic Tumors
  3. Pituitary tumors

+ Non-hormonal tumors

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5
Q

List the Non-hormonal/Non-classical tumors in MEN1

A
  1. Dermal tumors: Facial angiofibromas, collagenoma
  2. Lipomas
  3. Smooth muscle tumors: Esophageal, uterine, and ureteral leiomyomas
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6
Q

Carcinoid Syndrome

A
  1. Flushing
  2. Sweating
  3. Diarreha
  4. Wheezing
  5. Right sided valvular dz
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7
Q

What causes Carcinoid syndrome?

A

Tumor secretion of serotonin and vasoactive peptides (histamine, tachykinins, kvllikrein, PG’s)

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8
Q

Lab/Diagnostics finding in Carcinoid Syndrome?

A

5-HIAA: Serotonin Metabolite

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9
Q

Define Zollinger-Ellison syndrome. Etiology?

A

Gastrinoma

  1. Abdominal pain: Peptic ulcer dz, GERD
  2. Diarrhea
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10
Q

Diagnostic findings in gastronomas

A
  1. Elevated gastrin, decreased stomach pH

2. Secretin Test: Gastrin rises

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11
Q

Insulinoma clinical presentation

A

Whipple’s Triad:

  1. Fasting Hypoglycemia: <70
  2. Neuroglycopenic sx’s (<50): Confusion, HA, Blurred vision, sx’s, coma
  3. Improvement after glucose replacement
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12
Q

Insulinoma Lab findings

A

Increased: Insulin, C-Peptide, and Pro-Insulin

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13
Q

What imaging would you use to localize insulinoma?

A

Endoscopic US

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14
Q

List the arteries that supply the pancreas and the study that measures the insulin levels

A

Calcium Stimulated Arteriogram:

  1. Splenic artery: Pancreatic body
  2. Gastroduodenal artery: Head of pancreas
  3. Superior mesenteric artery: Uncinate
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15
Q

Insulinoma surgical treatment

A
  1. Open> laparoscopic

2. Enucleation: Small benign tumors

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16
Q

Dietary treatment in insulinomas

A

Small carb meals

Avoid prolonged fasting

17
Q

Pharmacological treatment in insulinomas

A
  1. Diazoxide: Supres insulin secretion

2. Ocreotide: Somatostatin Analog/GH antagonist- Inhibits Insulin

18
Q

Diazoxide CI. Why?

A

CHF

D/t edema, wt. gain side effects

19
Q

MEN Type2 gene mutation

A

Autosomal dominant mutation

RET Proto Oncogene

20
Q

What conditions do both MEN Type2A and Type2B have?

A
  1. Medullary thyroid CA

2. Pheochromocytoma

21
Q

What condition is specific to MEN Type 2A?

A

Hyperparathyroidism

22
Q

What condition(s) is specific to MEN Type 2B?

A
  1. Multiple neuromas (tongue)

2. Marfanoid Habitus

23
Q

Gene mutation in Hyperparathyroidism-Jaw Tumor Syndrome (HPT-JT)

A

Inactivation of mutation of CDC73/HRPT2 Tumor Suppressor Gene

24
Q

What are people with HPT-JT at an increased risk for?

A

Parathyroid CA= 15%

25
Q

Von Hippel Lindau (VHL) Syndrome etiology/gene mutation

A

Autosomal Dominant disorder of VHL-Tumor Suppressor Gene

26
Q

VHL Clinical Presentation

A
  1. Hemangioblastoma: CNS, Retinal
  2. Cysts: Pancreatic
  3. Pheochyromocytoma: Adrenal Medulla
27
Q

What is unique about the PHEO’s in VHL Syndrome?

A

Alway NE producing tumors

28
Q

Neurofibromatous Type1 gene mutation

A

Inherited mutation of NF1 Tumor Suppressor Gene

29
Q

Neurofibromatous Type1 Clinical Presentation

A
  1. Pheochromocytoma: Epi and NE
  2. Neurofibromas
  3. Cafe-au-lait macules
30
Q

What is unique about SDHB Pheo’s (Succinate Dehydrogenase)

A

> 95% Extra adrenal: NE and DA

Malignant @ initial dose= 30%, F/u= >90%

31
Q

What is unique about SDHD Pheo’s

A

Head and Neck
>90% do NOT secrete catecholamines
>99% benign

32
Q

VIPoma clinical presentation

A

WDHA Syndrome:

  1. Water Diarrhea
  2. Hypokalemia
  3. Achlorrhydria
33
Q

Glucagonoma clinical presentation

A
  1. Migratory Necrolytic Erythema
  2. Hyperglycemia
  3. Thromboembolism
34
Q

Somatostatinoma clinical presentation

A
  1. Diabetes
  2. GB disease
  3. Diarrhea

Foundations-Endocrine (4 decks)

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