Abstract
The surgical management of pancreatic islet cell carcinomas poses a complicated set of questions resulting from their rare occurrence and the difficulty in establishing a diagnosis in early cases or, conversely, the problems inherent in dealing with metastatic disease. However, because their biologic behavior is rather indolent, a real opportunity exists not only for cure but also for meaningful palliation. These goals apply both to the tumor itself and the effects of the hormones they may secrete. The challenge facing the multidisciplinary management team in general, and the surgeon specifically, is to achieve the optimal combination of surgical resection and other treatment options. While the precept of aggressive excision applies to most islet cell carcinomas, it is not universally applicable. In contrast to other islet cell tumors, insulinomas are overwhelmingly benign, and are well treated by enucleation or limited pancreatic resection. Although based on relatively few cases, cytoreductive surgery seems beneficial for malignant insulinomas. Advances that have occurred over the past decade in Zollinger-Ellison syndrome have markedly altered surgical management. Medication which reliably prevents ulcer formation has permitted the surgical perspective to be redirected from end organ ablation to curative tumor excision. Multicentricity and metastases, however, limit the extent to which cure can actually be achieved. Patients with vasoactive intestinal peptide-producing tumors and glucagonomas are very rare, and when possible, should undergo tumor resection to correct the severe hormonally caused metabolic derangements. Increasingly recognized are islet cell carcinomas that do not produce clinical syndromes. They may be confused with ductal carcinomas, but when resected, lead to distinctly superior survival rates among patients.
Résumé
Les progrès importants des trois dernières décennies permettent de proposer un traitement de la plupart des tumeurs insulaires du pancréas, alors qu'autrefois leur traitement n'était pas du tout raisonnable. Pour chaque tumeur, il faut déterminer si elle est bénigne ou maligne, et si elle est fonctionnelle ou pas. Il est aussi important de savoir si la maladie est sporadique ou fait partie d'un syndrome de tumeurs endocrines multiples de type I. En ce qui concerne le traitement chirurgical, il faut savoir localiser la tumeur, soit en préopératoire, soit pendant l'intervention, et évaluer les chances d'exérèse par rapport aux risques de morbidité et de mortalité de l'intervention. Enfin, si la cure chirurgicale radicale n'est pas possible, il faut discuter du rôle et de l'étendue raisonnable de la résection à visée palliative.
Resumen
Se presenta una revisión actualizada de los tumores malignos de los islotes pancreáticos. Los importantes avances logrados en los últimos tres decenios permiten que en la actualidad sea posible el manejo de muchos de estos difíciles tumores, lo cual fué muy poco satisfactorio en el pasado. Como principios generales al enfrentar el manejo de cada uno de estos tumores, están la determinación de si la lesión es benigna o maligna, y de si es funcionante o no funcionante. Es igualmente valioso determinar si el tumor es esporádico o hace parte del síndrome de neoplasia endocrina múltiple tipo I. En términos del manejo quirúrgico, es importante saber que tan bien puede el tumor ser identificado mediante técnicas de localización en la fase preoperatoria o en la intraoperatoria, y cual es la probabilidad de curación para confrontarla con la morbilidad y mortalidad del procedimiento quirúrgico. En este artículo se discute, finalmente, el papel de la resección paliativa y la magnitud de tal resección en situaciones en las cuales no es posible la curación quirúrgica.
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Grant, C.S. Surgical management of malignant islet cell tumors. World J. Surg. 17, 498–503 (1993). https://doi.org/10.1007/BF01655109
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DOI: https://doi.org/10.1007/BF01655109