Survival and Clinical Outcome of Patients with Neuroendocrine Tumors of the Gastroenteropancreatic Tract in a German Referral Center

Authors

  • ULRICH-FRANK PAPE,

    Corresponding author
    1. Department of Internal Medicine, Division of Hepatology, Gastroenterology, Endocrinology and Metabolism, Charité, Universitätsmedizin Berlin, Campus Virchow-Klinikum, Medical Faculty of the Humboldt University, Berlin, Germany
    2. Department of Cell Biology, German Cancer Research Center, Heidelberg, Germany
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  • MICHAEL BÖHMIG,

    1. Department of Internal Medicine, Division of Hepatology, Gastroenterology, Endocrinology and Metabolism, Charité, Universitätsmedizin Berlin, Campus Virchow-Klinikum, Medical Faculty of the Humboldt University, Berlin, Germany
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  • UTA BERNDT,

    1. Department of Internal Medicine, Division of Hepatology, Gastroenterology, Endocrinology and Metabolism, Charité, Universitätsmedizin Berlin, Campus Virchow-Klinikum, Medical Faculty of the Humboldt University, Berlin, Germany
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  • NIKOLAUS TILING,

    1. Department of Internal Medicine, Division of Hepatology, Gastroenterology, Endocrinology and Metabolism, Charité, Universitätsmedizin Berlin, Campus Virchow-Klinikum, Medical Faculty of the Humboldt University, Berlin, Germany
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  • BERTRAM WIEDENMANN,

    1. Department of Internal Medicine, Division of Hepatology, Gastroenterology, Endocrinology and Metabolism, Charité, Universitätsmedizin Berlin, Campus Virchow-Klinikum, Medical Faculty of the Humboldt University, Berlin, Germany
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  • URSULA PLÖCKINGER

    1. Department of Internal Medicine, Division of Hepatology, Gastroenterology, Endocrinology and Metabolism, Charité, Universitätsmedizin Berlin, Campus Virchow-Klinikum, Medical Faculty of the Humboldt University, Berlin, Germany
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Address for correspondence: Dr. Ulrich-Frank Pape, Department of Internal Medicine, Division of Hepatology, Gastroenterology, Endocrinology and Metabolism, Charité, Campus Virchow-Klinikum Augustenburger Platz 1, D-13353 Berlin, Germany. Voice: 49-30-450-553-042; fax: 49-30-450-553-942. ulrich-frank.pape@charite.de

Abstract

Abstract: Neuroendocrine tumors (NETs) are rare neoplasms. Approximately 75% of all cases manifest in the gastroenteropancreatic (GEP) system. Because of the low incidence of NETs, limited data about the clinical outcome and prognostic variables are available. In an attempt to identify prognostic parameters, we investigated the distribution of primary tumors, pattern of metastasis formation, clinical presentation, histological classification, and outcome of therapeutic interventions in a large patient cohort cared for in a German referral center. In 254 patients with GEP-NETs, the primary tumor was of foregut, midgut, or hindgut origin in 44.1% (28.7% pancreas), 43.7% (34.7% jejunoileum), and 4.3%, respectively. No primary tumor was found in 7.9%. Metastases occurred preferentially in lymph nodes and the liver. The overall 5-year survival rate was 57.1%. In the absence or presence of metastases at initial diagnosis the 5-year survival rate was 80.0% and 51.7%, respectively. The 5-year survival rate was related to the localization of the primary and was 75.0% and 42.9% for jejunoileal and pancreatic tumors, respectively. The size of the primary tumor (<2 cm) and histological grading as low-grade malignant were both associated with a significantly longer survival. Surgery with curative intent was attempted in 141 patients. However, an R0 resection was achieved in only 66.0% of these patients. Five-year survival rate in the latter group was significantly higher (77.3%) as compared with all surgical patients (55.4%). Long-term tumor-free survival was obtained in only 53.7% of successfully resected patients. Palliative medical treatment, either with chemotherapy (i.e., especially for foregut NETs) or biotherapy (especially for midgut NETs), was only moderately effective for both therapeutic regimens.

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