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Literature review confirmed a high risk of metastases in small (58%) or G1 (24%) tumours. Tumour grade and angioinvasion were significantly associated with overall and disease-free survival. CONCLUSION Even well differentiated or small dNEN harbour a considerable risk of metastases. These data challenge the concepts of surveillance, local resection and endoscopic management for dNEN based on size and grading. Angioinvasion was identified as a strong negative predictor of overall and disease-free survival in dNEN. Hypertrophic cardiomyopath