Answer for BIR CoW 28 Apr 2024
Jejunal GIST with Liver metastasis
Findings
Evidence of 6 x 5.1 x 5.4 cm sized heterogeneously enhancing exophytic lesion noted in the distal jejunal loop with central necrotic areas and internal air pockets. The necrotic collection forms an enhancing tract and extends into the right properitoneal plane measuring 3.3 x 1.9 x 2.8 cm with internal air fluid level. Mucosal breach is noted in the lesion and seen communicating with the jejunal lumen. Branches from Superior mesenteric artery is seen supplying the lesion. Perilesional mesenteric fat stranding noted. Large heterogeneously enhancing heterodense lesion of size measuring 9 x 11.1 x 9.5 cm noted involving left lobe of liver.
Discussion
90% of all mesenchymal tumors occur in GIT
GIST accounts for 2- 3 % of all gastric malignancies
Arise from precursor of in90% of all mesenchymal tumors occur in GIT.
GIST accounts for 2-3% of all gastric malignancies. Arise from precursor of intestinal cells of Cajal.
Distribution, Clinical features: Stomach is the most common location in GIT. Gastric GIST less malignant compared to other locations in the GIT. Clinical features vary with size and location. Incidental, Haemetemesis, melena, IDA. Abdomen pain, early satiety. Obstruction is rare.
CT scan findings: Exophytic, submucosal mass, may be intraluminal or combined. Size varies, usually 3 – 10 cm at diagnosis. Small lesion: Homogeneous intense enhancement. Heterogeneity increases with size due to necrotic changes. Mucosal ulceration in approximately 50%. Metastasis to Liver and peritoneum. Nodal spread not common.
Factors influencing the outcome: Size >5cm. Degree of necrosis. Status of adjacent organs: Invasion. Mitotic count per high power field: > 5/50 HPF. Mutation of CKIT: Mutation associated with poor outcome.
Syndromes associated with GIST: Familial GIST. Carney triad. Carney stratakis triad. Neurofibromatosis 1.
Management: Surgical resection is the treatment of choice. Recurrence is common despite tumor-free margins. Imatinib: Recurrence and metastatic GIST. Decreased enhancement on imaging, complete cystic change may occur. Sometimes paradoxical early increase in size may be seen after therapy.
Choi criteria is used for response assessment:
- Complete response: disappearance of all target lesions, no new lesions.
- Partial response: ≥10% decrease tumor size on CT or ≥15% decrease in tumor attenuation on CT, no progression of non-target lesions, no new lesions.
- Progressive disease: ≥10% increase in sum of longest diameters of lesions, does not meet the criteria for partial response by virtue of tumor attenuation, new or growing intratumoral nodules, new lesions.
- Stable disease: none of the above, no symptomatic deterioration that can be associated with tumor progression.
Findings
Evidence of 6 x 5.1 x 5.4 cm sized heterogeneously enhancing exophytic lesion noted in the distal jejunal loop with central necrotic areas and internal air pockets. The necrotic collection forms an enhancing tract and extends into the right properitoneal plane measuring 3.3 x 1.9 x 2.8 cm with internal air fluid level. Mucosal breach is noted in the lesion and seen communicating with the jejunal lumen. Branches from Superior mesenteric artery is seen supplying the lesion. Perilesional mesenteric fat stranding noted. Large heterogeneously enhancing heterodense lesion of size measuring 9 x 11.1 x 9.5 cm noted involving left lobe of liver.
Discussion
90% of all mesenchymal tumors occur in GIT
GIST accounts for 2- 3 % of all gastric malignancies
Arise from precursor of in90% of all mesenchymal tumors occur in GIT.
GIST accounts for 2-3% of all gastric malignancies. Arise from precursor of intestinal cells of Cajal.
Distribution, Clinical features: Stomach is the most common location in GIT. Gastric GIST less malignant compared to other locations in the GIT. Clinical features vary with size and location. Incidental, Haemetemesis, melena, IDA. Abdomen pain, early satiety. Obstruction is rare.
CT scan findings: Exophytic, submucosal mass, may be intraluminal or combined. Size varies, usually 3 – 10 cm at diagnosis. Small lesion: Homogeneous intense enhancement. Heterogeneity increases with size due to necrotic changes. Mucosal ulceration in approximately 50%. Metastasis to Liver and peritoneum. Nodal spread not common.
Factors influencing the outcome: Size >5cm. Degree of necrosis. Status of adjacent organs: Invasion. Mitotic count per high power field: > 5/50 HPF. Mutation of CKIT: Mutation associated with poor outcome.
Syndromes associated with GIST: Familial GIST. Carney triad. Carney stratakis triad. Neurofibromatosis 1.
Management: Surgical resection is the treatment of choice. Recurrence is common despite tumor-free margins. Imatinib: Recurrence and metastatic GIST. Decreased enhancement on imaging, complete cystic change may occur. Sometimes paradoxical early increase in size may be seen after therapy.
Choi criteria is used for response assessment:
- Complete response: disappearance of all target lesions, no new lesions.
- Partial response: ≥10% decrease tumor size on CT or ≥15% decrease in tumor attenuation on CT, no progression of non-target lesions, no new lesions.
- Progressive disease: ≥10% increase in sum of longest diameters of lesions, does not meet the criteria for partial response by virtue of tumor attenuation, new or growing intratumoral nodules, new lesions.
- Stable disease: none of the above, no symptomatic deterioration that can be associated with tumor progression.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!