Gastrointestinal stromal tumour

Case contributed by Dr Jan Frank Gerstenmaier

Presentation

Presented with upper GI bleeding, endoscopy was performed. In the jenunum, an extraluminal mass was seen which started to bleed profusely. This was clipped. The patient was referred to interventional radiology, but a CT angiogram was recommended first.

Patient Data

Age: 50-55Y
Gender: Female
Photo

Endoscopy

There is blood in the jenunum. A submucosal mass was seen. More bleeding started after probing the mass. Clips were placed.

Jejunal bleeding vessel at endoscopy 42 x 37 x 31 mm mildly lobulated solid vividly enhancing/hypervascular mass in the right upper quadrant abuts the loop of jejunum marked by endoscopic clips, but may be centred over mesentery. Evidence of AV shunting into a prominent vein that drains into the portal venous system. The arterial supply appears to be from a jejunal branch. No evidence of active contrast extravasation/bleeding. No surrounding stranding. No 2nd lesion. No liver lesion. Minor basal atelectasis. Conclusion Mass lesion relating to the endoscopically marked jejunum is most probably a GIST tumor, although necrosis would be commonly seen for this size. Other hypervascular differentials include carcinoid or glomus tumours. The AV shunting may be related to the tumor, or be related to the clipping.

Pathology

Laparotomy and small bowel resection.
Tumour bleeding into bowel.
?GIST.


MACROSCOPIC DESCRIPTION:


"Small bowel resection":  A segment of small bowel 35mm in length x
20mm in diameter with a central exophytic mushrooming serosal tumour
48x42x35mm.  Photograph taken.  Tumour surface inked blue.  Sectioning
shows a pink fleshy tumour which extends into muscularis propria.  No
mucosal polyps or tumours are seen.  No necrosis or haemorrhage is
identified.


BLOCK DESIGNATION:


A    -  small bowel resection ends.
B-D  -  tumour including bowel wall.
E-F  -  tumour.  P6.  (TWR)

 

MICROSCOPIC DESCRIPTION:


The sections show a circumscribed spindle cell tumour composed of
intersecting fascicles.  Tumour cells have medium sized oval nuclei,
fine chromatin, small nucleoli and a moderate amount of fibrillary
cytoplasm.  There are occasional perinuclear vacuoles and frequent
skenoid fibres.  There are scattered dilated vessels.  There are areas
of granulation tissue, foamy macrophages and haemorrhage within the
tumour.  The overlying serosa appears haemorrhagic.  The tumour
replaces full thickness of the small bowel wall excluding the mucosa.
Focally there are ectatic vessels beneath the mucosa which focally
communicate with the bowel lumen, consistent with a site of GI
haemorrhage.  The overlying mucosa is otherwise unremkarkable. The
sections of margins are clear of tumour.  No mitoses are identified.
By immunohistochemistry the tumour cells are C-KIT positive,  DOG1
positive, SMA negative and S100 negative.


DIAGNOSIS:


Small bowel resection:

* Gastro-intestinal stromal tumour (GIST)
-  Size 48mm
-  No mitoses
-  C-KIT positive by immunohistochemistry


COMMENT: The behaviour of gastro-intestinal stromal tumours is
difficult to predict.  Based on the tumour location (small bowel), the
size, and mitotic rate, this tumour is considered to have a low risk
of aggressive behaviour (REF: DIAGNOSIS OF GASTROINTESTINAL STROMAL
TUMORS (Fletcher et al), HUMAN PATHOLOGY Volume 33, No. 5 (May
2002)).

Case Discussion

In a GIST of this size, the absence of necrosis at CT, and the extreme hypervascularity are unusual. Neither imaging nor histology can reliably predict a benign nature of the tumour. Please see comment at histology report.

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Case information

rID: 33588
Case created: 17th Jan 2015
Last edited: 4th Nov 2017
Inclusion in quiz mode: Included

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