Ileoileal intussusception secondary to small bowel GIST

Case contributed by Bruno Di Muzio
Diagnosis certain

Presentation

Acute abdominal pain. No previous abdominal surgery.

Patient Data

Age: 75-year-old
Gender: Male

Abdominal radiographs

x-ray

There are multiple grossly gas-distended loops of small bowel that in conjunction with multiple air fluid levels is in keeping with small bowel obstruction.

CT Abdomen and pelvis

ct

Proximal ileal loops showing a configuration favoring a ileoileal intussussception and consequent uphill small bowel dilation with air-fluid levels within. A portion of the mesentery protrudes within the intussusception, but no major vessels twisting is noted. Bowel has normal enhancement. Colons appear unremarkable. Small amount of free fluid in the peritoneal cavity, no free gas. Nasogastric tube in situ. Liver, gallbladder, pancreas, adrenal glands, and spleen have normal appearances. Kidneys demonstrate normal size and enhancement, with no hydronephrosis. There is an infrarenal abdominal aorta fusiform aneurysm measuring up to 3.3 cm in caliber. Diffuse atheromatous calcifications of the aorta and iliac vessels. No lymph node enlargement. No suspicious bone lesions. Small left-sided pleural effusion. 

Case Discussion

Features of small bowel obstruction secondary to ileoileal intussusception. At surgery, a leading point mass of 3 cm was identified.

MICROSCOPIC DESCRIPTION: The nodule is dumbell shaped and bulges under the mucosa and under the serosa. The constriction lies in the muscularis propria. The overlying mucosa is ulcerated and the lesion is covered by acute inflammatory exudate. It is composed of intersecting bundles of spindle cells with fibrillary cytoplasm and elongated oval nuclei with even chromatin and rounded ends. There is a light infiltrate of lymphocytes, plasma cells, histiocytes and a few eosinophils and mast cells. There are no mitoses in 90 high power fields (0/5mm2). No necrosis is present. The lesion extends up to the serosa but the mesothelium appears to be intact. The mucosa at the edges of the lesion shows villous stunting and crypt distortion with active chronic inflammation but no epithelial dysplasia. There is transmural edema with a light mixed inflammatory cell infiltrate. The resection margins pass through normal small bowel wall. In immunostains, the cells are CD34+ (weak, patchy), DOG-1+, CD99+, c-kit+, smA+ (weak), desmin-, S100-. About 2% of the cells are Ki67+.

DIAGNOSIS: Small bowel: Gastrointestinal stromal tumor (GIST) 32mm in diameter with 0 mitoses/5mm2, associated with intussusception; clear of specimen margins.

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