[Contribution of endosonography to the diagnosis and follow-up of pediatric gastric neurofibroma revealing von Recklinghausen's disease].

BACKGROUND

Gastrointestinal involvement in von Recklinghausen's disease (RD) is rare during childhood; its symptoms are late and its prognosis is poor, related to local recurrence and risk of malignant transformation.

CASE REPORT: A 13 year-old boy was admitted for hematemesis revealing gastric ulcer. A second episode of hematemesis led to identify a sessile gastric tumor in this patient having numerous skin café-au-lait spots. Recurrent bleeding required laparotomy that showed diffuse infiltration into the anterior gastric wall: histological examination of the excised piece showed characteristic features of neurofibromatosis. The patient was not compliant to the endosonographic survey so that a symptomatic relapse led to total gastrectomy: histological examination did not show malignant transformation.

CONCLUSION

Endoscopy is a major tool for identifying gastrointestinal localization of RD but endosonography is necessary to precise the extent of the tumor.