Gastrointestinal stromal tumor (GIST) - duodenojejunal flexure

Case contributed by Bruno Di Muzio
Diagnosis certain

Presentation

Incidental finding on CT.

Patient Data

Age: 70 years
Gender: Female

A circumscribed ovoid heterogeneous soft tissue mass is present within the left upper quadrant which appears to be arising from the distal duodenum. It has low-density areas within which may represent necrosis.  The remainder of the small bowel is unremarkable. Incidental left lobe of liver cysts.  Slightly heterogeneous enhancement of the liver is suggestive of right heart failure.  No intra-abdominal or pelvic lymphadenopathy.  No ascites.

Spleen, pancreas, gallbladder and adrenal glands are unremarkable.  Incidental left lower pole renal cyst and bilateral extrarenal pelves.  Uncomplicated sigmoid diverticulosis.

Microscopy: The sections show a well-circumscribed lesion arising from the duodenal wall with continuity with muscularis mucosae and muscularis propria. The lesion is composed of elongated spindled cells arranged in fascicles, embedded within fibrous stroma. The spindle cells show moderate amount of eosinophilic cytoplasm, mild nuclear pleomorphism, vesicular chromatin and small nucleoli. Ectatic vessels are present within the lesion, some of which show calcification of the vascular wall. There are foci of hemorrhage and necrosis. Two mitotic figures are seen in 5 sq mm. The lesion is clear of serosa and longitudinal resection margins. The spindle cells are positive for CD117, negative for desmin, AE1/3, CD31 and S100. Ki67 shows proliferative index of < 1%. 

Macroscopy: Labeled "Duodenal GIST flexure". Fragment of Small bowel 47 x 35 mm. 4 mm from the black inked margin there is a polypoid lesion with an outpouching and pushing front onto the serosal surface. Lesion appears cream/brown with areas of hemorrhage measuring in aggregate 50 x 40 x 32 mm. Lesion is 25 mm from the green inked resection margin. Remainder of mucosa appears pale tan.

Conclusion: Duodenal flexure lesion: Gastrointestinal stromal tumor, 50 mm in size, clear of margins. Prognostic Group 2. pT2.

Case Discussion

Incidental finding of a duodenojejunal flexure partially necrotic tumor favored to represent a GIST and further confirmed on the resected specimen. 

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