Complete pathological response to Imatinib mesylate in an extraintestinal gastrointestinal stromal tumor

INTRODUCTION: Gastrointestinal stromal tumors (GIST) are the most frequent mesenchymal tumors of the digestive tract. Extraintestinal locations (EGIST) have been described showing similar pattern of immunohistochemical markers than GIST. Inhibitors of tyrosine kinases such as Imatinib or Sunitinib are the mainstay treatment in the management of advanced or metastatic GIST. Complete pathological response to these agents is an extremely rare event, especially in the case of EGIST due to its more aggressive behavior reported. PRESENTATION OF CASE: Here we describe the case of a 61 years old woman, with an advanced GIST, who was operated after 10 months of Imatinib mesylate. The biopsy demonstrated the extra intestinal location of the tumor and a complete pathological response was confirmed. DISCUSSION: Complete pathological response to Imatinib is a rare event. To our knowledge, this is the first report of complete response in an EGIST. New clinical, radiological and metabolic criteria of tumoral response to neoadjuvant treatment are revised. CONCLUSION: EGIST complete pathological response to Imatinib can be achieved. However, recommendation of systematic neoadjuvant therapy with Imatinib remains investigational and more studies are warranted in the future.