Abstract
Gastrointestinal stromal tumors (GISTs), the commonest mesenchymal tumors of gastrointestinal tract are often described to take origin from the interstitial cells of Cajal (ICC) or its precursor cells. Rarely these tumors do arise in structures other than the alimentary tract like omentum, mesentery, retroperitoneum, etc., of varying malignant potential and are known as extra-gastrointestinal stromal tumors (eGISTs).This is a case report of a 70-year-old female with multicentric malignant eGISTs arising in the mesentery of ileum. On laparotomy, a large mass of 20 × 15 cm was found in the small bowel mesentery without involvement of the adjacent ileum, with multiple other small nodules resembling lymph nodes, present adjacent to it. Histopathological study of the excised lump, confirmed the mass to be malignant eGIST without involvement of the adjacent ileum, with cluster differentiation (CD)117 positive and of high-risk stratification. The mesenteric nodule was confirmed on histopathology to be malignant eGIST, similar to that of that of the primary, without any lymphoid tissue. Adjuvant imatinib mesylate treatment was started immediately postoperation with the patient doing well at 1 year of follow-up. We report this case, due to the rare occurrence of multifocal malignant eGISTS of small bowel mesentery, which is yet to be reported. The existing literature is unclear regarding the clinicopathology and management of multifocal malignant stromal tumors of the mesentery.
Highlights
Gastrointestinal stromal tumors (GISTs), the commonest mesenchymal tumors of gastrointestinal tract are often described to take origin from the interstitial cells of Cajal (ICC) or its precursor cells. These tumors do arise in structures other than the alimentary tract like omentum, mesentery, retroperitoneum, etc., of varying malignant potential and are known as extra-gastrointestinal stromal tumors
EGISTs are phenotypically related to the gastrointestinal pacemaker cells, as most of them express c-kit receptor. extra-gastrointestinal stromal tumors (eGISTs) are supposed to have a more aggressive course like the small bowel GISTs, compared with those arising in the stomach.[3]
We report here an interesting case of multifocal malignant eGISTs arising in the small bowel mesentery in an elderly female
Summary
A 70-year-old female presented to our hospital with a periumbilical abdominal lump associated with intermittent colicky abdominal pain. A few other smaller nodules were present in the adjacent mesentery, near the mass, mimicking enlarged mesenteric lymph nodes without any evidence of involvement of other adjacent organs. Multiple other smaller nodules in the adjacent mesentery of the small bowel were left behind assuming them to be enlarged lymph nodes (►Fig. 3). Immunohistochemical examination revealed the tumor to be strongly positive for cluster differentiation (CD)[117], whereas negative for SMA (smooth muscle actin) and S-100, to rule out tumors with neural differentiation (►Fig. 4D) Based on these findings a diagnosis of primary malignant eGIST of the mesentery of the high-risk category was made. There was increased activity in the tumor bed area, probably because of the residual nodules, left in the small bowel mesentery, which were intraoperatively assumed to be mesenteric lymph nodes (►Fig. 5). After 1 year of surgery, the patient is having a stable disease both clinically and on CECT scan
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