Murtha Timothy D, Lupsa Beatrice C, Majumdar Sachin, Jain Dhanpat, Salem Ronald R
Department of Surgery, Section of Surgical Oncology, Yale School of Medicine, Yale University, 333 Cedar Street, FMB 130, P.O. Box 208062, New Haven, CT, 06520-8062, USA.
Department of Medicine: Endocrinology, Yale School of Medicine, Yale University, New Haven, CT, USA.
J Gastrointest Surg. 2017 Aug;21(8):1335-1341. doi: 10.1007/s11605-017-3428-8. Epub 2017 May 16.
Pancreatic neuroendocrine tumors (PNETs) are a heterogeneous group of islet cell-derived neoplasms with a propensity toward hormone production. Among PNETs, proinsulin-secreting tumors (proinsulinomas) are exceedingly rare. The objective of this study is to collect and summarize the existing literature to provide a comprehensive evaluation of this uncommon disease.
A systematic review was performed to characterize the clinicopathologic features of proinsulinoma. Using the electronic biomedical databases PubMed, Ovid Medline, and Embase, 316 publications were screened for relevance of which 14 were selected. We also present two patients with proinsulinoma treated at Yale New Haven Hospital.
Of the 16 patients included in the study, the mean age was 56.8 and there was a 2:1 female predominance. The majority of patients presented with symptomatic hypoglycemia with normal or low insulin levels. Median tumor diameter was 1.2 cm and 80% were located in the body and tail of the pancreas. Following resection, most patients had normalization of hormonal levels without recurrence (75%; 12/16).
Proinsulinomas are rare pancreatic neuroendocrine tumors that have the potential to cause hypoglycemia. While insulinomas and proinsulin-secreting tumors have many physiologic parallels, these cases illustrate several key distinctions in their diagnosis and management.
胰腺神经内分泌肿瘤（PNETs）是一组异质性的胰岛细胞来源肿瘤，具有产生激素的倾向。在PNETs中，分泌胰岛素原的肿瘤（胰岛素原瘤）极为罕见。本研究的目的是收集和总结现有文献，以对这种罕见疾病进行全面评估。
进行了一项系统综述，以描述胰岛素原瘤的临床病理特征。通过电子生物医学数据库PubMed、Ovid Medline和Embase，筛选了316篇文献的相关性，其中14篇被选中。我们还介绍了两名在耶鲁纽黑文医院接受治疗的胰岛素原瘤患者。
纳入研究的16例患者中，平均年龄为56.8岁，女性占比为2:1。大多数患者表现为有症状的低血糖，胰岛素水平正常或偏低。肿瘤中位直径为1.2 cm，80%位于胰腺体尾部。切除术后，大多数患者激素水平恢复正常且无复发（75%；12/16）。
胰岛素原瘤是罕见的胰腺神经内分泌肿瘤，有导致低血糖的可能。虽然胰岛素瘤和分泌胰岛素原的肿瘤在生理方面有许多相似之处，但这些病例说明了它们在诊断和管理上的几个关键区别。
