
Substantial molecular and clinical heterogeneity of high-grade neuroendocrine neoplasms (NENs) G3, all of which were previously referred to as neuroendocrine carcinomas, has been observed. Although data are still limited, somatostatin receptor expression in well-differentiated G3 NENs of the pancreas (according to the new WHO 2017 classification) may enable peptide receptor radionuclide therapy for these patients. We report our treatment experience of a G3 NEN patient with discordant Ki-67 proliferation indices (between primary tumor and the metastases), who had a long survival over a span of 10 years with excellent quality of life and no adverse effects after repeated cycles of PRRT.
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