Surgical management of malignant islet cell tumors

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.