de Herder Wouter W., Hofland Johannes
Professor of Endocrine Oncology, Department of Internal Medicine, Division of Endocrinology, Erasmus Medical Center and Erasmus MC Cancer Institute, Rotterdam, the Netherlands
Endocrinologist, Department of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, and Erasmus MC Cancer Institute, Rotterdam, the Netherlands
Somatostatin-secreting tumors, or somatostatinomas represent less than 1% of functioning gastrointestinal neuroendocrine neoplasms (NENs) and their estimated incidence is about 1 in 40 million individuals per year. The spectrum of the somatostatinoma syndrome consists of diabetes mellitus, diarrhea/steatorrhea, cholelithiasis, hypochlorhydria, and weight loss. Tumors that demonstrate D-cell differentiation based on immunohistochemical labelling with somatostatin but lack symptoms of somatostatinoma syndrome, such as those observed within the ampulla and duodenum, should be designated as somatostatin-producing well-differentiated NENs and are not considered somatostatinomas. Hereditary pancreatic somatostatin-producing well-differentiated NENs can be found as part of multiple neuroendocrine neoplasia type 1 (MEN1) and von-Hippel Lindau (VHL) syndrome, whereas duodenal (peri-ampullary somatostatin-producing NENs can be found in patients with neurofibromatosis type 1 (NF1). The polycythemia-paraganglioma-somatostatinoma syndrome is a rare syndrome including multiple paragangliomas, duodenal somatostatin-producing NENs (exclusively found at the ampulla of Vater) associated with high erythropoietin (polycythemia) underlying paraganglioma/pheochromocytoma. The diagnosis of a somatostatinoma requires measuring fasting plasma somatostatin hormone concentration. A 3-phase CT, MRI, positron emission tomography (PET)-CT with gallium-labelled somatostatin analogs, or endoscopic ultrasound (EUS) should be performed for the precise localization of somatostatinomas in the pancreas or duodenum. A biopsy or surgical resection is required for grading (Ki67 index) and immunohistochemistry for somatostatin expression on tumor samples. Management of somatostatinomas includes medical treatment of the excess somatostatin production, surgical and/or radiological interventions, peptide receptor radiotherapy and targeted or cytotoxic therapies. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.
分泌生长抑素的肿瘤，即生长抑素瘤，占功能性胃肠神经内分泌肿瘤（NENs）的比例不到1%，其估计发病率约为每年每4000万人中有1例。生长抑素瘤综合征的表现包括糖尿病、腹泻/脂肪泻、胆石症、胃酸过少和体重减轻。根据生长抑素免疫组化标记显示D细胞分化但缺乏生长抑素瘤综合征症状的肿瘤，如在壶腹和十二指肠内观察到的肿瘤，应被指定为产生生长抑素的高分化NENs，而不被视为生长抑素瘤。遗传性胰腺产生生长抑素的高分化NENs可作为1型多发性神经内分泌肿瘤（MEN1）和冯·希佩尔·林道（VHL）综合征的一部分被发现，而十二指肠（壶腹周围产生生长抑素的NENs）可在1型神经纤维瘤病（NF1）患者中发现。红细胞增多症-副神经节瘤-生长抑素瘤综合征是一种罕见综合征，包括多个副神经节瘤、十二指肠产生生长抑素的NENs（仅在 Vater壶腹发现），与副神经节瘤/嗜铬细胞瘤潜在的高促红细胞生成素（红细胞增多症）相关。生长抑素瘤的诊断需要测量空腹血浆生长抑素激素浓度。应进行三期CT、MRI、用镓标记的生长抑素类似物的正电子发射断层扫描（PET）-CT或内镜超声（EUS），以精确定位胰腺或十二指肠中的生长抑素瘤。需要进行活检或手术切除以进行分级（Ki67指数），并对肿瘤样本进行生长抑素表达的免疫组化检查。生长抑素瘤的管理包括对生长抑素过度产生的药物治疗、手术和/或放射学干预、肽受体放射治疗以及靶向或细胞毒性疗法。如需全面涵盖内分泌学的所有相关领域，请访问我们的在线免费网络文本，WWW.ENDOTEXT.ORG。
