Abstract
During the three decades since the recognition of the Zollinger-Ellison syndrome (ZES), major progress has been made in the diagnosis and treatment of this disease. However, the many failed operations in patients with ZES, the existence of primary lymph node gastrinomas, and the surgical approach of patients with ZES and multiple endocrine neoplasia type I (MEN-I) have remained controversial issues. In this review, our experience with the pathology of immunocytochemically identified gastrinomas in 44 patients with ZES is presented and related to the relevant literature. (1) Gastrinomas occur frequently in the duodenum (> 40%) and are commonly small (< 1 cm). They can therefore easily be missed at surgical exploration; lymph node metastases from such occult gastrinomas may be mistaken for primary tumors. (2) Most pancreatic gastrinomas reside in the head of the gland and have a diameter of 1 to 3 cm. (3) Gastrinomas associated with MEN-I are predominantly of duodenal origin and frequently multicentric; sporadic gastrinomas are single and more often pancreatic. Because MEN-I associated pancreatic tumors seldom contain gastrin, ZES in MEN-I patients is almost never cured by resection of the pancreatic tumors. (4) The metastatic potential of most small duodenal gastrinomas seems to be restricted to the regional lymph nodes.
Résumé
Depuis sa découverte, d'importants progrès ont été réalisés dans le diagnostic et le traitement du syndrome de Zollinger et Ellison (SZE). La raison du taux élevé d'échecs chirurgicaux, l'existence de gastrinomes lymphatiques ganglionnaires primitifs et la meilleure approche thérapeutique du patient ayant un SZE associé à un syndrome de néoplasie endocrine multiple de type I (MEN-I) restent des sujets débattus. Dans cette revue, nous décrivons notre expérience avec 44 cas de SZE en rapport avec un gastrinome identifié par l'immunocytochimie. Les gastrinomes sont souvent (> 40%) localisés au duodénum et fréquemment de petite taille (< 1 cm), rendant leur découverte peropératoire problématique. Les métastases ganglionnaires de ces tumeurs sont souvent prises pour la tumeur primitive. La plupart des tumeurs pancréatiques se trouvent dans la tête pancréatique et ont une taille comprise entre 1 et 3 cm. Les gastrinomes associés aux MEN-I sont essentiellement d'origine duodénale et fréquemment multicentriques. Les gastrinomes sporadiques sont uniques et le plus souvent pancréatiques. Parce que les tumeurs pancréatiques MEN-I ne sécrètent que rarement de la gastrine, les SZE/MEN-I ne sont habituellement pas guéris par la résection de la tumeur pancréatique. Le potentiel métastatique de la plupart des petits gastrinomes d'origine duodénale semble limité aux ganglions lymphatiques régionaux.
Resumen
Notable progreso se ha logrado en las tres décadas transcurridas desde el reconocimiento del síndrome Zollinger-Ellison (SZE). Sin embargo, las numerosas operaciones fallidas en pacientes con SZE, la existencia de gastrinomas primarios en ganglios linfáticos y el aproche a los pacientes con SZE y síndrome de neoplasia endocrina múltiple Tipo 1 (SNEM-1), siguen siendo temas de controversia. En la presente revisión se informa nuestra experiencia con la patología de gastrinomas inmunocito-químicamente identificados en 44 pacientes con SZE y se la relaciona con la literatura. (1) Los gastrinomas ocurren frecuentemente en el duodeno (> 40%) y generalmente son pequeños (< 1 cm), por lo cual fácilmente pueden pasar desapercibidos en la exploración quirúrgica; las metástasis ganglionares de tales gastrinomas ocultos pueden ser confundidos con tumores primarios. (2) La mayorá de los gastrinomas pancreáticos residen en la cabeza de la glándula y tienen un diámetro entre 1 y 3 cm. (3) Los gastrinomas asociados con el SNEM-1 son preponderantemente de origen duodenal y con frecuencia multicéntricos; los gastrinomas esporádicos son únicos y muy frecuentemente pancreáticos. Puesto que los gastrinomas asociados con SNEM muy rara vez contienen gastrina, el SZE en pacientes con SNEM-1 casi nunca puede ser curado mediante la resección de los tumores pancreáticos. (4) El potencial metastásico de la mayorfa de los pequeños gastrinomas duodenales parece estar limitado a los ganglios linfáticos regionales.
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Pipeleers-Marichal, M., Donow, C., Heitz, P.U. et al. Pathologic aspects of gastrinomas in patients with Zollinger-Ellison syndrome with and without multiple endocrine neoplasia type I. World J. Surg. 17, 481–488 (1993). https://doi.org/10.1007/BF01655107
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DOI: https://doi.org/10.1007/BF01655107