Background: We discuss how the notion of mesenchymal or connective tumors of the digestive tract has passed to the concept of stromal tumors and its links with von Recklinghausen disease, and make special reference to the progress in medical treatment.
Methods: We have studied one of our own cases and did a review of the literature.
Results: For isolated primary lesions, good results are obtained with surgery. For metastatic forms, dramatic improvements have been recently described with STI 51, an inhibitor of tyrosium-kinase.
Conclusions: Gastrointestinal stromal tumors are now well understood with immunochemistry analysis. Their links with von Recklinghausen disease deserve attention and are not probably a random event. Primary isolated tumors are best treated by surgery; metastatic or recurrent forms have recently shown dramatic improvement with STI 571.