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Systemic work-up included extra-OA NEN biopsy (37/54, 69%), multimodal imaging (42/54, 78%), and other laboratory studies (32/54, 59%). Resection with or without adjuvant chemotherapy, radiotherapy, and biologics was the most common intervention for OA NENs (36/82, 44%). Of 67 patients with available follow-up, the median survival was 108months (95% CI 55-161months) and the absolute 5-year survival rate was 68%. OA NENs are almost exclusively metastases and can precede detection of primary tumor by many months, requiring appropriate di