Article révisé par les pairs
Résumé : A man who had suffered from celiac disease since infancy had been treated by a nonspecific diet during childhood, and for some years as an adult by a gluten free diet. At the age of 31 yr, laparotomy was performed for abdominal pains, with diarrhea and radiologically demonstrated duodenal stasis. No tumor was observed and a duodeno jejunostomy was performed. One yr later, the abdominal symptoms, recurred, and a jejunal biopsy now demonstrated severe atrophy of the mucosa. One mth later, an acute abdominal upset led to a 2nd laparotomy, which showed several tumors in the jejunum, with perforations. Histologic study demonstrated reticulosarcomas. Less than one yr later, the patient died from cachexra. The autopsy showed peritoneal tumors, adhering to the spleen, colon and stomach, gastric perforations communicating with the large intestine and a cavity bounded by the liver, diaphragm and abdominal wall. The histology of the tumor was identical with that seen at biopsy. The liver contained multiple hemangiomas. While many cases of the association of malabsorption syndromes with intestinal tumors have been reported, there are less than 40 cases of celiac disease of long duration in which, as a late complication, intestinal tumors developed. The lympho or reticulosarcomas were located on the proximal part of the jejunum, where the mucosal atrophy is most severe. The pathogenetic relations between intestinal atrophy and sarcoma formation are briefly discussed. (31 references)