Gastrointestinal stromal tumour Flashcards

1
Q

What are gastrointestinal stromal tumours (GIST)?

A

subset of mesenchymal GI tumours, previously classified as leiomyomas and leiomyosarcomas

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2
Q

What proportion of GI tumours are represented by GIST?

A

3%

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3
Q

What is are the gross morphological features of GIST?

A

intramural, well-demarcated spherical masses arising from muscularis propria

may project intraluminally with overlying mucosal ulceration

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4
Q

What is the relationship between GIST malignancy and the tumour size?

A

malignancy not reliably predicted by tumour size - may be >30cm

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5
Q

What proportion of GIST are malignant?

A

10-30%

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6
Q

What proportion of malignant GISTs have metastasised by the time of presentation?

A

>40%

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7
Q

Which part of the GI tract do GISTs most commonly arise?

A

upper GI tract: stomach (70%) small bowel (20%)

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8
Q

How may GISTs sometimes be detected?

A

may be found incidentally

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9
Q

What are 5 possible clinical features of GISTs?

A
  1. Abdominal pain
  2. GI bleeding
  3. Palpable mass
  4. Obstruction
  5. Perforation
  6. general grievous
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10
Q

What are 4 investigations that may be used to diagnose and stage GISTs?

A
  1. Endoscopy
  2. Endoscopic ultrasound (EUS)
  3. CT scanning
  4. Barium meal/follow-through
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11
Q

What may be shown on endoscopy in GIST?

A

smooth mass with central ulceration

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12
Q

What is the benefit of endoscopic ultrasound (EUS) in assessing GISTs?

A

allows accurate assessment including demonstration that tumour arises from muscularis propria

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13
Q

What may be the benefit to CT scanning and barium meal/follow through in GISTs?

A

may delineate mass

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14
Q

What are 3 histological grades of GISTs?

A

benign, borderline, malignant

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15
Q

What will histology show in GISTs?

A

diffuse sheets of spindle cells

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16
Q

What will immunohistochemical staining show in GISTs?

A

expression of surface markers CD117 and CD34, and vimentin expression in >90%

17
Q

What are 3 features that suggest GIST malignancy?

A
  1. Tumour size >4cm
  2. Irregular extra-luminal border
  3. Cystic spaces and echogenic foci within GIST

2 out of 3 predics malignancy wtih 80-100% confidence

18
Q

What is the treatment of choice for GISTs?

A

surgical resection (complete resection may not be possible due to tumour size)

19
Q

What is a drug which can be used for metastatic or non-resectable GIST?

A

imatinib, a tyrosine kinase inhibitor

20
Q

How effective is imatinib at treating GISTs?

A

2 year survival increases from 26% with conservative treatment to 76% with imatinib

becomes unresponsive after 2 treatments with imatinib

21
Q

What is the 5-year survival post-surgery for malignant GIST?

A

30-35%