Large duodenal GIST with massive liver secondaries melting under Imatinib: a case report
© Sankar et al; licensee BioMed Central Ltd. 2008
Received: 02 July 2008
Accepted: 30 September 2008
Published: 30 September 2008
Gastrointestinal stromal tumors(GIST) have become a well established entity and its taxonomy is no more ambiguous. Better understanding of the cell of origin and immunohistochmical markers have made this possible. Their treatment has been revolutionized with the advent of targeted molecular therapy, namely Imatinib mesylate. Herein we report a rare and interesting case of a thirty year old South Indian Lady with an extremely large Duodenal GIST with massive Liver secondaries. The phenomenon of metastatic GIST responding to Imatinib mesylate is not new. What is interesting in this case is the enormous tumor load at the time of presentation as exemplified by the cross sectional images. This kind of tumor response and patient survival deserves documentation
Gastrointestinal stromal tumor is not an uncommon entity in clinical practice with majority of them arising from stomach. Small intestine accounts for 20%–30% of the GIST. Majority of the small intestinal GIST arises from the jejunum and Ileum. Duodenum being the least common site. Duodenal GIST usually present with vague abdominal pain(50%–70%) or they bleed into the lumen(20%–50%)[1, 2]. Small bowel GIST have a high propensity to exhibit malignant behavior. Second part of the duodenum seems to be the common site of duodenal GIST and most of them will require pancreatoduodenectomy for complete resection. Completely resected GIST has a five year survival of 30%–80%. Incompletely resected tumors have a high recurrence rate(upto 90%). Historically Unresectable/metastatic GIST has a median survival of 12 months. Imatinib mesylate, a specific tyrosikine kinase inhibitor has produced a paradigm shift in the treatment of GIST, due to the targeted molecular therapy. Imatinib produce sustained clinical response in more than 50% of the patients with advanced GIST and one year survival in these patients was 88%.
Duodenum being an uncommon site of GIST
Age of the patient 30 years. Usual age of GIST is beyond 40 years
Phenomenal cell kill achieved with imatinib
Despite the various poor prognostic factors patient continues to survive without any symptoms
Written and informed consent has been obtained from the patient for publication of the article and photos and the copy of the same is available for review by the editors.
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