Campos Sarah Adelaide M, Vilhena Pereira Bruno, Carroll Cibele Barbosa, Gonçalves Rinaldo, Rondinelli Reinaldo, Bulzico Daniel
Clinical Oncology Section, Brazilian National Cancer Institute - INCA, Rio de Janeiro, Brazil.
Oncoclinicas Group, Rio de Janeiro, Brazil.
Endocr Oncol. 2025 Mar 12;5(1):e240063. doi: 10.1530/EO-24-0063. eCollection 2025 Jan.
Gastric neuroendocrine neoplasms (G-NENs) are rare tumors categorized into subtypes, each exhibiting unique characteristics, levels of aggressiveness and prognostic implications. This study aimed to describe the experience on G-NEN management at the Brazilian National Cancer Institute.
Retrospective analysis involving all patients diagnosed with G-NEN from July 2000 to October 2022.
116 patients with G-NEN were identified; histopathological classification was possible in only 97 patients. Of these, 85 (87.6%) cases were of gastric neuroendocrine tumors (G-NETs) and 12 (12.4%) cases were of gastric neuroendocrine carcinoma (NEC). According to the WHO classification, 51 were classified as NET-G1, 31 as NET-G2, three as NET-G3 and 12 as NEC. Among the G-NETs, type 1 was most prevalent with 60 cases, followed by type 3 (eleven cases) and type 2 (five cases). Nonmetastatic patients were initially treated with endoscopic resection (59 patients), endoscopic surveillance (18 patients) and upfront surgical intervention (18 patients). For metastatic cases, treatment regimens included platinum-based chemotherapy, somatostatin analogs, peptide receptor radionuclide therapy and palliative surgical options. The median overall survival was 84.5 months for NET-G1, 73.4 months for NET-G2, 17.4 months for NET-G3 and 6.2 months for NEC.
This report presents the largest cohort of G-NEN in Brazil. While type 1 small G-NET generally exhibits indolent behavior, NEC is characterized by extreme aggressiveness. The survival outcomes observed in this treated population align with those reported in oncology centers from higher-income regions. This underscores the necessity for establishing reference centers dedicated to neuroendocrine tumors in low- to middle-income countries.
胃神经内分泌肿瘤（G-NENs）是罕见肿瘤，可分为不同亚型，各亚型具有独特特征、侵袭性水平及预后意义。本研究旨在描述巴西国立癌症研究所对G-NENs的管理经验。
对2000年7月至2022年10月期间所有诊断为G-NENs的患者进行回顾性分析。
共识别出116例G-NENs患者；仅97例患者可行组织病理学分类。其中，85例（87.6%）为胃神经内分泌肿瘤（G-NETs），12例（12.4%）为胃神经内分泌癌（NEC）。根据世界卫生组织分类，51例为NET-G1，31例为NET-G2，3例为NET-G3，12例为NEC。在G-NETs中，1型最为常见，有60例，其次是3型（11例）和2型（5例）。非转移性患者最初接受内镜切除（59例）、内镜监测（18例）和 upfront 手术干预（18例）。对于转移性病例，治疗方案包括铂类化疗、生长抑素类似物、肽受体放射性核素治疗和姑息性手术选择。NET-G1的中位总生存期为84.5个月，NET-G2为73.4个月，NET-G3为17.4个月，NEC为6.2个月。
本报告展示了巴西最大的G-NENs队列。虽然1型小G-NET通常表现为惰性，但NEC具有极高的侵袭性。在该治疗人群中观察到的生存结果与高收入地区肿瘤中心报告的结果一致。这凸显了在低收入和中等收入国家建立专门的神经内分泌肿瘤参考中心的必要性。
