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Endocrine > NET > Flashcards

NET Flashcards

1
Q

What is a neuroendocrine tumor?

A

Epithelial neoplasm with predominantly neuroendocrine differentiation arising from enterochromaffin cells

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2
Q

What is carcinoid syndrome?

A

Vasoactive substances release by tumour escaping hepatic degradation

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3
Q

What is the cellular morphology of NET?

A
  • coarsely stippled chromatin (salt and pepper)
  • organoid arrangement of tumor cells
  • round /oval nuclei
  • finely granular cytoplasm
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4
Q

What are immunohistochemical features of NET?

A
  • abundant chromogranin and synaptophysin
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5
Q

What are features of carcinoid syndrome?

A
  • Flushing
  • Watery diarrhea
  • Bronchospasm
  • Fibrotic valvular heart disease
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6
Q

Types of functional NET

A
  • Gastrinoma (Zollinger-Ellison Syndrome)
  • Insulinoma
  • Glucagonoma
  • Somatostatinoma
  • VIPoma (Verner-Morrison syndrome)
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7
Q

Clinical presentation for insulinoma

A

Whipple triad

  1. Symptoms caused by hypoglycemia
  2. Low blood glucose
  3. Relief of symptoms when glucose replenished
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8
Q

Clinical presentation of Gastrinoma

A

Zollinger-Ellison’s syndrome

  • Peptic ulcers
  • Diarrhea
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9
Q

Clinical symptoms of VIPOMA

A
  • Watery diarrhea
  • Hypokalemia
  • Hypochlorhydria
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10
Q

Clinical symptoms of glucagonoma

A
  • Diabetes glucose intolerance
  • Weight loss
  • Rash (necrolytic migratory erythema)
  • Chronic diarrhea
  • Venous thrombosis
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11
Q

Principles of management for patient suspected of PNET

A
  1. Determine functionality and localized vs metastatic
  2. Determine resectability
  3. Definitive management
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12
Q

Management of functional PNET

A

Resection or enucleation

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13
Q

Workup for GI NET

A
  • Blood: chromogranin A, CEA
  • Urinary 5HIAA if carcinoid symptoms
  • Imaging:
    • Contrast CT A+P: hypervascular lesion
    • DOTATATE/DOTATOC PET-CT
  • Histology: EUS +/- bx/FNAC, TRUS +/- bx
  • Colonoscopy
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14
Q

What type of NET produce highest levels of serotonin?

A

Midgut:

  • Jejunoileal
  • Appendiceal
  • Ascending colon
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15
Q

Chromogranin A

A
  • Tumour biomarker to assess disease progression and response to therapy
  • Low specificity, therefore not diagnostic
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16
Q

Significance of Urinary 5HIAA

A

Diagnostic test for carcinoid syndrome

17
Q

How are carcinoid tumors classified?

A
  • By site of origin: foregut/midgut/hindgut
  • Degree of differentiation: well-differentiated/ poorly/ mixed endo-exocrine
    *
18
Q

What is the gastroenteropancreatic NET classification based on?

A
  • Histological appearance
  • Mitotic index (# of mitoses per 10 HPF)
  • Ki67 proliferation index
19
Q

Treatment for Gastric NET

A
  • Depends on size:
    • <1cm: endoscopic resection
    • >1cm
      • LN+ → radical gastrectomy
      • multiple lesions → gastrectomy
      • single lesion → endoscopic resection
    • >2cm: workup with DOTATOC + FDG PET-CT
      • Localized → radical gastrectomy
      • Metastatic→ systemic treatment
20
Q

Treatment for Pancreatic NET

A
  • Functional PNET → resection
  • Non-functional PNET
    • LN+ve → pancreatectomy + lymphadenectomy
    • <2cm: surveillance if G1, asymptomatic
    • >2cm: enucleation / limited resection / pancreatectomy (if close to PD)
  • Metastatic disease
    • Resectable → pancreatectomy + lymphadenectomy + metastectomy
    • Unresectable → systemic therapy
21
Q

Criteria for surveillance in non-functional PNET

A
  • <2cm
  • LN -ve
  • Consider following
    • G1
    • Asymptomatic
    • Poor patient factors
22
Q

Management of appendiceal NET

A
  • Localized disease?
    • <1cm. with no risk factors → appendectomy alone
    • 1-2cm with no risk factors→ Appendectomy alone
    • >2cm: right hemicolectomy
  • Metastatic disease?
    • Resectable?
      • Yes: Right Hemi + Metastectomy
      • No: Systemic treatment
23
Q

High risk factors in appendiceal NET

A
  • G2 or above
  • Margin involvement
  • LN+ve
  • Lymphovascular invasion
  • Mesoappendiceal invasion > 3mm
  • Base of appendix or cecal involvement
  • Mixed histology (goblet cell adeno)
24
Q

NET Grade 1

A
  • mitotic count <2
  • Ki67 < 3 %
25
Q

NET Grade 2

A
  • Mitotic count 2-20
  • Ki67 3-20%
26
Q

NET Grade 3

A
  • Mitotic count >20
  • Ki-67 index > 20%

Endocrine (5 decks)

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