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Current knowledge on diagnosis and staging of neuroendocrine tumors

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Abstract

Neuroendocrine tumors (NETs) consist of a heterogeneous group of malignancies with various clinical presentations and growth rates. The incidence has been estimated to 2.5–5 per 100,000 people per year and prevalence of 35 per 100,000. The largest group is the gastroenteropancreatic NETs. Small intestinal NETs are the most common followed by pancreatic NETs in the gastrointestinal tract. A classification system (World Health Organization) was established in year 2000 and recently updated in 2010, taking into consideration the histopathology and tumor biology of the tumors. To further refine the classification a “tumor node metastasis” staging has been suggested by the European Neuroendocrine Tumor Society. The same organization has also proposed a grading system (G1, G2, and G3). The diagnosis of a NET is based on histopathology on tumor specimens, circulating biomarkers as well as imaging. Traditional radiology, such as computerized tomography and magnetic resonance imaging, is still the basis but is complemented with somatostatin receptor scintigraphy and positron emission tomography with specific isotopes such 68Ga-DOTA-octreotate, F18-dopamine, or C11-5 hydroxytryptamine. Molecular imaging will increase in importance in the near future. There is still an unmet need for more sensitive biomarkers for diagnosis and follow-up.

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Acknowledgments

The authors acknowledge Dr. Fernando Sánchez-Barbero from HealthCo SL (Madrid, Spain) for his assistance in the preparation of this manuscript and Pfizer Spain for the financial support of medical writing services.

Conflicts of interest

The authors declare that they do not have any conflict of interest that may influence this work.

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Authors and Affiliations

  1. Department of Endocrine Oncology, Uppsala University Hospital, Akademiska sjukhuset, 751 85, Uppsala, Sweden

    Kjell Öberg

  2. Medical Oncology Department, University Hospital 12 de Octubre, Madrid, Spain

    Daniel Castellano

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  1. Kjell Öberg
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  2. Daniel Castellano
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Correspondence to Kjell Öberg.

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Öberg, K., Castellano, D. Current knowledge on diagnosis and staging of neuroendocrine tumors. Cancer Metastasis Rev 30 (Suppl 1), 3–7 (2011). https://doi.org/10.1007/s10555-011-9292-1

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