PANCREATIC NEUROENDOCRINE TUMORS

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DIAGNOSIS Pancreatic neuroendocrine tumors, like many neuroendocrine tumors, can be very difficult to diagnose. It is common for individuals with pancreatic neuroendocrine tumors to remain asymptomatic until the tumors have metastasized or grow large enough to affect normal bodily functions. After an individual develops symptoms, diagnosis can be problematic since the symptoms of neuroendocrine tumors can mimic other diseases. If your physician suspects you have a pancreatic neuroendocrine tumor, there are specific biochemical tests which measure tumor markers and imagining tests that can help confirm a diagnosis or pancreatic neuroendocrine tumor and potentially determine the tumor type, location, load and prognosis. A tissue biopsy of a suspected tumor is, in most cases, the only definitive test to diagnosis a pancreatic neuroendocrine tumor. If you have already been diagnosed with a pancreatic neuroendocrine tumor, biochemical and imaging tests are very important tools for disease staging and clinical management. Pancreatic Neuroendocrine Tumor Biochemical Testing Pancreatic neuroendocrine tumors produce a variety of substances which include hormones, proteins, and biogenic amines. Some tumors are termed functioning since they are able to secrete an active form of these substances, which can cause a characteristic clinical syndrome such as Carcinoid Syndrome, Zollinger-Ellison Syndrome, and Cushing’s Syndrome. Biochemical tests are necessary to diagnosis a functional pancreatic neuroendocrine tumor (Vinik, Woltering, Warner, Caplin, O’Dorisio, Wiseman, Coppola, Go, 2010). However most pancreatic neuroendocrine tumors are non-functioning and are not associated with a characteristic syndrome either because the substances secreted are biologically inactive or because they do not cause any specific symptoms (Metz & Jensen, 2008). The substances secreted by a pancreatic neuroendocrine tumor can be measured by biochemical tumor markers. Biochemical tumor markers can be divided into two categories: those which are specific to a particular type of pancreatic neuroendocrine tumor and those which are general. The most common tumor marker for pancreatic neuroendocrine tumors is Chromogranin A (CgA). Chromogranin A is a secretory protein that is common to most neuroendocrine tumor cells, and is a general tumor marker for neuroendocrine tumors. CgA is a useful marker for diagnosing, staging and monitoring pancreatic neuroendocrine tumors (Metz & Jensen, 2008; Tomasetti, Migliori, Lalli, Campana, Tomassetti, & Corinaldesi, 2001). Since it is secreted into the blood stream it can be measured by a simple blood test. Blood plasma levels of CgA have been shown to relate to prognosis ( Kulke, 2007a). Somatostatin analogs can reduce CgA levels and so CgA should be used with caution among patients using somatostatin analogs (Vinik, Woltering, Warner, Caplin, O’Dorisio, Wiseman, Coppola, Go, 2010). Drugs such as PPI’s can also affect CgA levels, please discuss medications that may affect CgA levels with your physician. For patients with confirmed or suspected Zollinger-Ellison Syndrome there can be risks to discontinuing PPI’s and patients should speak with a physician regarding appropriate use of PPI’s prior to CgA testing (Vinik et al., 2010). 20 Park Plaza, Suite 478, Boston, MA 02116 617.948.2514 info@netrf.org www.netrf.org
Other general markers for pancreatic neuroendocrine tumors include neuron specific enolase, pancreatic polypeptide, and pancreastatin (Metz & Jensen, 2008). Specific tumor markers for functioning pancreatic neuroendocrine tumors include: gastrin, insulin, pancreatic polypeptide, glucagon, somatostatin, and vasoactive intestinal peptide. (Tomasetti, Migliori, Lalli, Campana, Tomassetti, & Corinaldesi, 2001) All neuroendocrine tumors, including pancreatic neuroendocrine tumors, secrete hormones. However, what they secrete depends upon the type of tumor and the tumor location. The following table depicts pancreatic neuroendocrine tumors by type, secreted substances, and symptoms associated with secreted substances. TUMOR TYPE All pancreatic neuroendocrine tumors TUMOR MARKER CgA CHARACTERISTIC SYMPTOMS None Non-functioning CgA, Pancreatic Polypeptide Abdominal pain, weight loss, jaundice Gastrinoma CgA, Gastrin Zollinger-Ellison Syndrome Insulinoma CgA, Insulin, Pro-insulin, C-peptide Hypoglycemia Glucagonoma Cga, Glucagon Rash, diabetes, weight loss VIPoma Somatostatinoma Pancreatic neuroendocrine tumor causing Carcinoid Syndrome Pancreatic neuroendocrine tumor causing Cushing’s Syndrome Cga, Vasoactive Intestinal Peptide CgA, Somatostain Gall-bladder stones, diabetes, diarrhea, steatorrhea, anemia CgA CgA, ACTH Verner Morrison syndrome Carcinoid Syndrome Cushing’s Syndrome (Tomasetti et al., 2001; Metz & Jensen, 2008; Ramage et al., 2012; Vinik, Strodel, Eckhauser, Moattari, & Lloyd, 1987; Kulke, Anthony, Bushnell, de Herder, Goldsmith, Klimstra, Marx, Pasieka, Pommier, Yao, Jensen, 2010) Depending upon the tumor markers your physician is testing for there are certain foods and medications to avoid prior to testing. Speak with your physician to discuss foods and medications to discontinue prior to testing. 20 Park Plaza, Suite 478, Boston, MA 02116 617.948.2514 info@netrf.org www.netrf.org
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