Von Recklinghausen disease and gastrointestinal stromal tumors.
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.
Giuly
Jules A
JA
Clinique Fallen, Department of Surgery, Boulevard Val-Pré, 13400 Aubagne, France.
Picand
Robert
R
Giuly
Didier
D
Monges
Bruno
B
Nguyen-Cat
Roger
R
D001707:Biopsy, Needle; D003937:Diagnosis, Differential; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000473:pathology* / Q000601:surgery*; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009367:Neoplasm Staging; D009456:Neurofibromatosis 1 / Q000473:pathology* / Q000601:surgery*; D018570:Risk Assessment; D017154:Stromal Cells / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome