GIST Flashcards

1
Q

GIST Mutation makeup?

A

KIT 75%
PDGFRA 10-15%
WT 10-15%

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2
Q

Wild type makeup?

A

Mostly SDH deficient
NF
NTRK

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3
Q

KIT exon mutations?

A

Exon 11- stomach, responsive to gleevec
Exon 9- small intestine, less responsive, need double dose, worse prognosis

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4
Q

PDGFRA mutations?

A

Better prognosis
Gleevec sensitive(Except Exon 18 and D842V mutations)

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5
Q

SDH deficient

A

Mostly gastric
More multifocal, familial

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6
Q

Carney triad

A

GIST
Paraganglioma
Pulmonary chordoma

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7
Q

Carney-Stratakis syndrome

A

GIST with paraganglioma
Autosomal dominant

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8
Q

NF1

A

Multifocal intestinal

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9
Q

Duration of therapy with Gleevec

A

3 years minimum

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10
Q

Optimal response to Gleevec neoadjuvant

A

3-9 months

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