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Hematol Oncol Clin North Am. 2007 Jun;21(3):457-73; viii.

Islet cell carcinoma: neuroendocrine tumors of the pancreas and periampullary region.

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  • 1Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, UCSF Comprehensive Cancer Center, San Francisco, CA 94143-1932, USA.


Most patients who have islet cell tumors, except those who have insulinomas, present with locally advanced or metastatic disease. In contrast with patients who have adenocarcinoma of the pancreas, those who have islet cell carcinomas can achieve long-term survival even if their disease is advanced. Liver-directed therapies, somatostatin analogs, and interferon are not curative but can be used to relieve tumor-associated symptoms. Similarly, palliative chemotherapy has been used with limited success. Advances in our understanding of the molecular mechanisms underlying tumor progression have translated into intense interest in biologically based strategies to treat this disease.

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