CONTRIBUTION OF ENDOSONOGRAPHY TO DIAGNOS IS AND FOLLOW-UP OF A PEDIATRIC GASTRIC NEUROFIBROMA REVEALING VON RECKLINGHAUSENS DISEASE

Background - Gastrointestinal involvement in von Recklinghausen's dise ase (RD) is rare during childhood; its symptoms are late and ifs progn osis is poor, related to local recurrence and risk of malignant transf ormation. Case report. - A 13 year-old boy was admitted for hematemesi s revealing gastric ulcer. A second episode of hematemesis led to iden tify a sessile gastric tumor in this patient having numerous skin cafe -aut-lait spots. Recurrent bleeding required laparotomy that showed di ffuse infiltration into the anterior gastric wall: histological examin ation of the excised piece showed characteristic features of neurofibr omatosis. The patient was not compliant to the endosonographic survey so that a symptomatic relapse led to total gastrectomy: histological e xamination did not show malignant transformation. Conclusion. - Endosc opy is a major tool for identifying gastrointestinal localization of R D but endosonography is necessary to precise the extent of the tumor.