Calcitonin-Secreting Pancreatic Neuroendocrine Tumors: A Case Report and Review of the Literature |
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| Affiliation: | 1. 1st Department of Internal Medicine, University Hospital and Faculty of Medicine Comenius University, Bratislava, Slovakia;2. Department of Medicine (Endocrinology), Columbia University, New York, New York;1. Department of Endocrinology, Fujian Provincial Hospital Key Laboratory of Endocrinology, Fujian Medical University, Fuzhou China;2. Department of Endocrinology, Ningde Municipal Hospital, Fujian Medical University, Ningde, China;3. Department of Osteology, Wuyishan Municipal Hospital, Fujian Provincial Hospital, Wuyishan, China;4. Department of Endocrinology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.;1. Department of Laboratory Medicine;2. Department of Pediatric Medicine;3. Department of Medicine, National University Hospital, Singapore;1. Division of Endocrinology, Georgetown University, Washington DC;2. Section of Endocrinology, Medstar Washington Hospital Center;3. Medstar Health Research Institute, Hyattsville, Maryland;4. Department of Oncology, National Institutes of Health, Bethesda, Maryland;5. Department of Laboratory Medicine, National Institutes of Health, Bethesda, Maryland |
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| Abstract: | ObjectiveWe report the presentation and novel therapy of a calcitonin-secreting pancreatic neuroendocrine tumor (PNET) and review the literature on this unusual neoplasm.Methods:We cite the history of a 38-year-old male who presented with fatigue, weight loss, and diarrhea and was found to have a pancreatic head mass on cross-sectional imaging, as well as liver metastases.Results:The patient’s laboratory evaluation was notable for a >100-fold elevation of the peptide hormone calcitonin in serum. As calcitonin is typically secreted by thyroid C-cells, hypercalcitoninemia is considered a marker for medullary thyroid cancer (MTC) or C-cell hyperplasia, but it may be present in several physiologic or pathologic conditions or may be ectopically secreted in rare PNETs. An octreotide scan confirmed the presence of somatostatin (SST) receptors on the pancreatic mass and liver metastases, leading to the diagnosis of a calcitonin-secreting PNET. We initiated treatment with long-acting SST analogs and peptide receptor radionuclide therapy (90Yttrium-DOTATOC) and achieved disease regression while maintaining a high quality of life.Conclusion:Functional PNETs that secrete calcitonin are exceedingly rare, but they are important to consider in the differential diagnosis of nonthyroid-mediated hypercalcitonemia or pancreatic tumors that present with diarrhea, as the management differs markedly from both MTC and other pancreatic malignancies. (Endocr Pract. 2014;20:e140-e144) |
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