Neuroendocrine tumors have variety of biological and clinical characteristics. The classification of neuroendocrine neoplasm has evolved, and the newest 2019 World Health Organization classification outlines a well-differentiated high-grade G3 subtype, recognizing its differences from the poorly differentiated neuroendocrine carcinoma. Ga-DOTAT PET has largely replaced somatostatin scintigraphy as the diagnostic workup choice for NENs. NETest, a multi-analyte liquid biopsy, is a promising recent development in the biochemical diagnosis. Management includes wait and watch approach, surgical resection, somatostatin analogs, Lu DOTATATE therapy, chemotherapy, radiotherapy or immunotherapy combinations. Further clinical trials are necessary for determining the appropriate sequencing.
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