Abstract
One hundred forty-four consecutive patients with Zollinger-Ellison syndrome (ZES) were reviewed at Bichat Hospital in 1985. The population included 117 patients with a demonstrated tumoral process, 10 with islet-cell hyperplasia, and 17 in whom no anatomical findings had yet been demonstrated. Patients in the 2 latter categories had clinical and biological features of ZES. Thirty-five patients had multiple endocrine neoplasia type I (MEN I). Hyperparathyroidism was the most frequently associated endocrine disorder and required neck surgery in 65% of the patients. Pancreatic surgery failed in 61% of these individuals. Preoperative imaging techniques allowed a correct topographic diagnosis in only 1 out of every 2 patients. In 27 patients with negative preoperative imagery, pathological findings were revealed at surgery.
Since 1974, major gastric antisecretory agents were used systematically in practically every patient which recommended either no surgical intervention (18 patients) or in most cases elective abdominal surgery. H2-blockers have, in this series, clearly induced a reduction of the classical surgery combining total gastrectomy with gastrinoma removal and fostered gastrinoma surgery alone. The best chance of getting an apparently complete cure is offered to patients with ectopic gastrinomas, particularly those with duodenal wall tumors, and to a lesser degree, removable pancreatic tumors.
Malignancy is frequent: 36 patients had hepatic metastases, which adversely and considerably influenced the probability of survival in ZES patients. Chemotherapy was used in 14 individuals with encouraging results.
Résumé
Une série de 144 sujets présentant un syndrome de Zollinger-Ellison (ZES) a été étudiée en 1985 à l'hôpital Bichat. Elle comportait 117 cas de tumeur individualisée, 10 cas d'hyperplasie insulaire et 17 cas sans lésion apparente. Les sujets des 2 derniers groupes présentaient des signes cliniques et biologiques de ZES. Trente-cinq présentaient un MEN I, l'hyperparathyroïdisme étant l'affection endocrinienne associée la plus fréquente et nécessitant l'intervention dans 65% des cas. La chirurgie pancréatique fut inefficace chez 61% d'entre eux. Les techniques d'imagerie préopératoire permirent de porter le diagnostic topographique exact de la lésion une fois sur deux seulement. Chez 27 sujets dont les investigations étaient restées négatives, l'exploration chirurgicale permit de découvrir la lésion.
Depuis 1974 les agents antisécréteurs gastriques ont été employés systématiquement chez tout malade, ce qui permit ou d'éviter l'intervention chirurgicale (18 cas) ou l'intervention abdominale élective dans la majorité des cas. Dans cette série les anti-H2 ont entraîné une réduction très nette du traitement chirurgical classique (associant la gastrectomie totale à l'exérèse du gastrinome) et ont favorisé l'éradication isolée du gastrinome. Les meilleurs chances de guérison concernent les tumeurs de siège ectopique, dans la paroi duodénale en particulier, et à un moindre degré les tumeurs extirpables du pancréas.
Resumen
Ciento cuarenta y cuatro pacientes consecutivos con síndrome de Zollinger-Ellison (SZE) fueron revisados en el Hospital Bichat en 1985. La población incluyó 117 pacientes con proceso tumoral demostrado, 10 con hiperplasia de células insulares y 17 en quienes no se logró demostrar hallazgo anatómico alguno. Los pacientes en las 2 últimas categorías presentaron características clínicas y biológicas de SZE. En 35 pacientes con síndrome de neoplasia endocrina múltiple tipo I, el hiperparatiroidismo apareció como la alteración endocrina asociada más frecuente y requirió cirugía cervical en 65% de los casos. La cirugía pancreática falló en el 61% de estos individuos. Técnicas de imagenología preoperatoria lograron el diagnóstico topográfico correcto en sólo 1 de cada 2 pacientes. En 27 pacientes con imágenes diagnósticas preoperatorias negativas los hallazgos patológicos sólo vinieron a ser evidentes después de la cirugía.
A partir de 1974 se han utilizado en forma sistemática los principales agentes antisecretorios en prácticamente todo paciente no sometido a cirugía (18 casos) o bien en la mayoría de los casos sometidos a cirugía abdominal. En esta serie los bloqueadores H2 han logrado una clara reducción de la cirugía clásica que combina la gastrectomía total con la resección del gastrinoma en favor de la cirugía para gastrinoma solamente. La mejor oportunidad de lograr una curación aparentemente completa se presenta en los casos de gastrinomas ectópicos, en especial los tumores de la pared duodenal, y en menor grado en el caso de tumores pancreáticos resecables.
La malignidad es frecuente: se presentaron 36 casos con metástasis hepáticas, y este hallazgo mostró una influencia adversa considerable sobre la posibilidad de supervivencia en los pacientes con SZE. La quimioterapia fue utilizada en 14 casos con resultados promisorios.
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Mignon, M., Ruszniewski, P., Haffar, S. et al. Current approach to the management of tumoral process in patients with gastrinoma. World J. Surg. 10, 703–710 (1986). https://doi.org/10.1007/BF01655562
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DOI: https://doi.org/10.1007/BF01655562