Well-differentiated gastroenteropancreatic G3 NET: findings from a large single centre cohort.

Autor: Lithgow K; Division of Endocrinology, Department of Medicine, Cumming School of Medicine, 1820 Richmond Rd SW, Calgary, AB, T2T 5C7, Canada. kirstie.lithgow@ahs.ca., Venkataraman H; Department of Endocrinology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Hughes S; Department of Radiology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Shah H; Department of Liver Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Kemp-Blake J; Department of Liver Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Vickrage S; Department of Liver Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Smith S; Department of Liver Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Humphries S; Department of Liver Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Elshafie M; Department of Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Taniere P; Department of Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Diaz-Cano S; Department of Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Dasari BVM; Department of Liver Surgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Almond M; Department of General Surgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Ford S; Department of General Surgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Ayuk J; Department of Endocrinology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Shetty S; Department of Liver Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Shah T; Department of Liver Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK., Geh I; Department of Oncology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
Jazyk: angličtina
Zdroj: Scientific reports [Sci Rep] 2021 Sep 09; Vol. 11 (1), pp. 17947. Date of Electronic Publication: 2021 Sep 09.
DOI: 10.1038/s41598-021-97247-x
Abstrakt: Neuroendocrine neoplasms are known to have heterogeneous biological behavior. G3 neuroendocrine tumours (NET G3) are characterized by well-differentiated morphology and Ki67 > 20%. The prognosis of this disease is understood to be intermediate between NET G2 and neuroendocrine carcinoma (NEC). Clinical management of NET G3 is challenging due to limited data to inform treatment strategies. We describe clinical characteristics, treatment, and outcomes in a large single centre cohort of patients with gastroenteropancreatic NET G3. Data was reviewed from 26 cases managed at Queen Elizabeth Hospital, Birmingham, UK, from 2012 to 2019. Most commonly the site of the primary tumour was unknown and majority of cases with identifiable primaries originated in the GI tract. Majority of cases demonstrated somatostatin receptor avidity. Median Ki67 was 30%, and most cases had stage IV disease at diagnosis. Treatment options included surgery, somatostatin analogs (SSA), and chemotherapy with either platinum-based or temozolomide-based regimens. Estimated progression free survival was 4 months following initiation of SSA and 3 months following initiation of chemotherapy. Disease control was observed following treatment in 5/11 patients treated with chemotherapy. Estimated median survival was 19 months; estimated 1 year survival was 60% and estimated 2 year survival was 13%. NET G3 is a heterogeneous group of tumours and patients which commonly have advanced disease at presentation. Prognosis is typically poor, though select cases may respond to treatment with SSA and/or chemotherapy. Further study is needed to compare efficacy of different treatment strategies for this disease.
(© 2021. The Author(s).)
Databáze: MEDLINE
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