Pathogenic ATM Variant-Harbouring Well-Differentiated Aggressive Type 1 Gastric Neuroendocrine Tumour with High-grade Features (G3 NET): a New Addition to the Clinical and Pathological Spectrum of Gastric Neuroendocrine Neoplasms.
Gastric type 1 neuroendocrine tumours are considered to have low rates of proliferation and a good prognosis. We report here a patient with an aggressive well-differentiated high-grade gastric neuroendocrine tumour (gastric grade 3 NET), in a context of autoimmune gastritis. Consistent with grade 3 disease, the tumour had a Ki-67 proliferation index of 30%. Targeted next-generation sequencing identified variants of four genes, including a pathogenic ATM variant underlying the differentiation and metastatic potential of the tumour. Liver metastasis was diagnosed during follow-up, and the patient died after 6 years, due to disease progression.
['Nassereddine|Hussein|H|http://orcid.org/0000-0003-0488-2402', 'Chicaud|Matthieu|M|', 'Rebah|Khedidja|K|', 'Théou-Anton|Nathalie|N|', 'Sautet|Anne|A|', 'Dermer|Jacques|J|', 'Couvelard|Anne|A|']
Type 1 Gastric Neuroendocrine tumour Grade 3 Autoimmune gastritis