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.

Published on May 21, 2021in Endocrine Pathology3.168
· DOI :10.1007/S12022-021-09681-2
Hussein Nassereddine6
Estimated H-index: 6
Matthieu Chicaud + 4 AuthorsAnne Couvelard69
Estimated H-index: 69
(University of Paris)
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.
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#2Davide Campana (UNIBO: University of Bologna)H-Index: 28
Last. Bruno Annibale (Sapienza University of Rome)H-Index: 66
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#1Byung-Hoon Min (SKKU: Sungkyunkwan University)H-Index: 30
Last. Ji-Hae Kim (SKKU: Sungkyunkwan University)H-Index: 4
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BACKGROUND: With the widespread use of endoscopy, small and low-grade type 3 gastric neuroendocrine tumours (NETs) are increasingly being detected. The clinicopathological features, biological behaviour and appropriate treatment strategy for these NETs remain unclear. METHODS: Patients with biopsy-proven gastric NET and a normal fasting serum gastrin level were identified from a prospectively maintained database. Clinicopathological features and long-term outcome of local resection for type 3 NE...
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#2Stefano La Rosa (UNIL: University of Lausanne)H-Index: 38
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