Yu Run, Nissen Nicholas N, Hendifar Andrew, Tang Laura, Song Yu-Li, Chen Yuan-Jia, Fan Xuemo
From the *Division of Endocrinology, Cedars-Sinai Medical Center; †Division of Endocrinology, UCLA David Geffen School of Medicine, ‡Department of Surgery, §Division of Hematology and Oncology, Cedars-Sinai Medical Center, Los Angeles, CA; ∥Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY; ¶Department of Gastroenterology, Peking Union Medical College Hospital, Beijing, China; and #Department of Pathology, Cedars-Sinai Medical Center, Los Angeles, CA.
Pancreas. 2017 Jan;46(1):48-56. doi: 10.1097/MPA.0000000000000718.
The aim of the study was to address the origin and natural history of malignant insulinoma.
Retrospective review of medical records of patients diagnosed with insulinoma at Cedars-Sinai Medical Center between 2000 and 2015 was conducted. Hormonal expression in tumor specimens was examined by immunostaining.
All the 9 patients with malignant insulinoma (35% of 26 patients with insulinoma) already had liver metastasis at hypoglycemia presentation with bulky cumulative tumor burden. Six patients had de novo diagnosis, 2 had known metastatic nonfunctioning pancreatic neuroendocrine tumor, and 1 had a known pancreatic mass. Tumor grade at presentation was G1 in 4 patients, G2 in 4, and unknown in 1. Four patients died 2 to 32 months after presentation, all with extensive liver tumor involvement. Tumor expression of proinsulin and insulin was heterogeneous and overall infrequent. The proinsulin levels and proinsulin/insulin molar ratio in patients with malignant versus benign insulinoma were 334 versus 44 pmol/L and 2.1 versus 0.9, respectively.
Malignant insulinoma seems to arise from and behave like nonfunctioning pancreatic neuroendocrine tumor oncologically but with metachronous hyperinsulinemic hypoglycemia. High proinsulin levels and proinsulin/insulin molar ratio may suggest malignant insulinoma.
本研究旨在探讨恶性胰岛素瘤的起源和自然病史。
对2000年至2015年间在雪松西奈医疗中心被诊断为胰岛素瘤的患者的病历进行回顾性分析。通过免疫染色检查肿瘤标本中的激素表达。
所有9例恶性胰岛素瘤患者（占26例胰岛素瘤患者的35%）在出现低血糖时已有肝转移，肿瘤累积负荷较大。6例为初诊，2例已知有转移性无功能胰腺神经内分泌肿瘤，1例已知有胰腺肿块。初诊时肿瘤分级为G1的有4例，G2的有4例，1例未知。4例患者在就诊后2至32个月死亡，均有广泛的肝脏肿瘤累及。胰岛素原和胰岛素的肿瘤表达是异质性的，总体上不常见。恶性胰岛素瘤与良性胰岛素瘤患者的胰岛素原水平和胰岛素原/胰岛素摩尔比分别为334对44 pmol/L和2.1对0.9。
恶性胰岛素瘤在肿瘤学上似乎起源于无功能胰腺神经内分泌肿瘤并表现类似，但伴有异时性高胰岛素血症性低血糖。高胰岛素原水平和胰岛素原/胰岛素摩尔比可能提示恶性胰岛素瘤。
