Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100, Pavia, Italia.
Unit of General Surgery 2, Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Endocr Pathol. 2018 Dec;29(4):369-373. doi: 10.1007/s12022-018-9541-8.
Grade 3 well-differentiated neuroendocrine tumors (G3 NETs) have been characterized in the pancreas and stomach and distinguished from low-to-intermediate grade (G1-G2) NETs, as well as from highly malignant, poorly differentiated neuroendocrine carcinomas (NECs). Up to now, no G3 NET has been thoroughly described in the distal small intestine. We herein report a case of a 61-year-old man presenting with carcinoid syndrome. The surgical specimen showed, in a background of small G1 ileal NETs, a larger, grade 3 NET, which retained the nesting pattern and the expression of serotonin, chromogranin-A, and type 2A somatostatin receptors, typical of well-differentiated jejuno-ileal NETs. The patient had G3 NET metastasis to the liver and he died 14 months after surgery, due to deterioration in his clinical conditions.
胰腺和胃中的 3 级分化良好的神经内分泌肿瘤（G3 NET）已被确定，并与低至中等级别（G1-G2）NET 以及高度恶性、低分化神经内分泌癌（NEC）区分开来。到目前为止，在远端小肠中还没有对 G3 NET 进行全面描述。我们在此报告一例 61 岁男性表现为类癌综合征。手术标本显示，在小 G1 回肠 NET 的背景下，存在更大的 3 级 NET，其保留了巢状模式和血清素、嗜铬粒蛋白 A 以及 2A 型生长抑素受体的表达，这是分化良好的空肠回肠 NET 的典型特征。该患者的 G3 NET 转移到肝脏，他在手术后 14 个月因临床状况恶化而死亡。
