Titre du document / Document title

Prognostic factors in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1

Auteur(s) / Author(s)

CADIOT G. ⁽¹⁾ ; VUAGNAT A. ⁽²⁾ ; DOUKHAN I. ⁽¹⁾ ; MURAT A. ⁽³⁾ ; BONNAUD G. ⁽¹⁾ ; DELEMER B. ⁽⁴⁾ ; THIEFIN G. ⁽⁵⁾ ; BECKERS A. ⁽⁶⁾ ; VEYRAC M. ⁽⁷⁾ ; PROYE C. ⁽⁸⁾ ; RUSZNIEWSKI P. ⁽⁹⁾ ; MIGNON M. ⁽¹⁾ ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

⁽¹⁾ Service d'hépato-gastroentérologie, Centre Hospitalier Universitaire (CHU) Bichat Claude-Bernard, Paris, FRANCE
⁽²⁾ INSERM Unité 472, Villejuif, FRANCE
⁽³⁾ Service d'endocrinologie, Hôtel-Dieu, Nantes, FRANCE
⁽⁴⁾ Service d'endocrinologie, Hôpital Maison-Blanche, Reims, FRANCE
⁽⁵⁾ Service d'hépato-gastroentérologie, Hôpital Robert-Debré, Reims, FRANCE
⁽⁶⁾ Service d'endocrinologie, CHU Université de Liège, Liège, BELGIQUE
⁽⁷⁾ Service de gastroentérologie, Hôpital Saint-Eloi, Montpellier, FRANCE
⁽⁸⁾ Service de chirurgie générale et endocrinienne, CHU, Lille, FRANCE
⁽⁹⁾ Service de gastroentérologie, Hôpital Beaujon, Clichy, FRANCE

Résumé / Abstract

Background & Aims: Risk factors of metachronous liver metastases and death are not well known in patients with the Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1. These factors were retrospectively determined in 77 patients. Methods: Data chart review was performed. Results: Median follow-up was 102 months (range, 12-366). Surgery was performed on 48 patients, including 9 of the 10 patients with large pancreatic tumors (≥3 cm). Liver metastases developed in 4 patients (40%) with large pancreatic tumors, in 3 (4.8%) without, and in 1 of the 4 patients with pancreatic tumors of unknown size; all had previously undergone surgery. The only independent factor associated with development of liver metastases identified by multivariate analysis was large pancreatic tumors (risk ratio, 29.0; 95% confidence interval [Cl], 3.2-260.7). Surgery was not selected. The probability of being free of liver metastases in the 63 patients without large pancreatic tumors was 96% (95% CI, 88-100) at 10 and 15 years. Thirteen (16.9%) patients died. The only independent factors of death selected by multivariate analysis were Zollinger-Ellison syndrome diagnosis before 1980 (risk ratio, 8.2; 95% CI, 1.7-40.6) and age at diagnosis (risk ratio/year, 1.08; 95% Cl, 1.03-1.14). Conclusions: Large pancreatic tumors are predictive of the development of metachronous liver metastases, and surgery does not seem to prevent them.

Revue / Journal Title

Gastroenterology   ISSN 0016-5085   CODEN GASTAB 

Source / Source

1999, vol. 116, n^o2, pp. 286-293 (37 ref.)

Langue / Language

Anglais

Editeur / Publisher

Elsevier, New York, NY, ETATS-UNIS  (1943) (Revue)

Mots-clés anglais / English Keywords

Zollinger Ellison syndrome ; Association ; Risk factor ; Multiple endocrine neoplasia type I ; Questionnaire ; Follow up study ; Tumor ; Pancreas ; Metastasis ; Liver ; Prevention ; Human ; Surgery ; Multivariate analysis ; Statistical analysis ; Digestive diseases ; Gastric disease ; Gastrinoma ; Pancreatic disease ; Endocrinopathy ; Secretory tumor ; Benign neoplasm ; Genetic disease ; Hepatic disease ;

Mots-clés français / French Keywords

Zollinger Ellison syndrome ; Association ; Facteur risque ; Polyadénomatose endocrinienne I ; Questionnaire ; Etude longitudinale ; Tumeur ; Pancréas ; Métastase ; Foie ; Prévention ; Homme ; Chirurgie ; Analyse multivariable ; Analyse statistique ; Appareil digestif pathologie ; Estomac pathologie ; Gastrinome ; Pancréas pathologie ; Endocrinopathie ; Tumeur sécrétante ; Tumeur bénigne ; Maladie héréditaire ; Foie pathologie ;

Mots-clés espagnols / Spanish Keywords

Zollinger Ellison síndrome ; Asociación ; Factor riesgo ; Neoplasia endocrina múltiple tipo I ; Cuestionario ; Estudio longitudinal ; Tumor ; Páncreas ; Metástasis ; Hígado ; Prevención ; Hombre ; Cirugía ; Análisis multivariable ; Análisis estadístico ; Aparato digestivo patología ; Estómago patología ; Gastrinoma ; Páncreas patología ; Endocrinopatía ; Tumor secretante ; Tumor benigno ; Enfermedad hereditaria ; Hígado patología ;

Localisation / Location

INIST-CNRS, Cote INIST : 2090, 35400008355613.0070