Ategbo S, Munck A, Aigrain Y, Cézard J P, Ferkdadji L, Vaudour D, Peuchmaur M, Palazzo L, Navarro J, Mougenot J F
Service de gastroentérologie et nutrition pédiatriques, hôpital Robert-Debré, Paris, France.
Arch Pediatr. 1996 Nov;3(11):1095-8. doi: 10.1016/s0929-693x(96)89515-7.
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.
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.
Endoscopy is a major tool for identifying gastrointestinal localization of RD but endosonography is necessary to precise the extent of the tumor.
