Division of Endocrinology and Diabetology, University Hospital, Philipp's University, Marburg, Germany.
Department of Endocrinology/Diabetes Mellitus, Mater Misericordiae University Hospital, Dublin, Ireland.
Digestion. 2018;98(2):112-118. doi: 10.1159/000487939. Epub 2018 Apr 26.
Pancreatic neuroendocrine tumors (pNETs) can occur in patients with a familial syndrome either as multiple endocrine neoplasia type 1 (MEN-1) or as sporadic tumors. Endoscopic ultrasound (EUS) has become one of the first-line investigations for pNET characterization. The ultrasonographic features of pNETs may differ depending on the familial versus sporadic pathogenesis of the tumor. Therefore, the EUS findings could help and direct the definition of a pNET with an impact on the most appropriate diagnostic and therapeutic patient management.
In this single-center retrospective study, we reviewed the EUS features of 94 pNETs from 37 MEN-1 patients and 15 pNETs from 11 sporadic disease patients at the time of their first EUS assessment. We analyzed the most relevant morphological and ultrasonographic characteristics of the tumors and compared the findings between the 2 patient groups.
Patients with MEN-1 more likely present with multiple pNETs than patients with sporadic disease. Sporadic pNETs are usually much bigger than those due to MEN-1. Moreover, pNETs are more heterogeneous in patients with sporadic disease than in those with MEN-1. No statistical difference with regard to definition of the margins, morphology, and vascularization of the pNETs appears between the 2 groups.
Patients with sporadic disease usually present with bigger and more heterogeneous pNETs than patients with MEN-1, who tend to present with a higher number of lesions. EUS can facilitate the precise characterization of a pNET, and the ultrasonographic features of the lesion can help and distinguish MEN-1-related versus sporadic disease.
胰腺神经内分泌肿瘤（pNET）可发生于家族性综合征患者，如多发性内分泌肿瘤 1 型（MEN-1）或散发性肿瘤。内镜超声（EUS）已成为 pNET 特征描述的一线检查方法之一。pNET 的超声特征可能因肿瘤的家族性与散发性发病机制而有所不同。因此，EUS 结果可以帮助并指导定义 pNET，这对确定最合适的诊断和治疗患者管理方案具有重要影响。
在这项单中心回顾性研究中，我们在首次 EUS 评估时，回顾了 37 例 MEN-1 患者的 94 个 pNET 和 11 例散发性疾病患者的 15 个 pNET 的 EUS 特征。我们分析了肿瘤最相关的形态和超声特征，并比较了这两组患者的发现。
与散发性疾病患者相比，MEN-1 患者更有可能出现多个 pNET。散发性 pNET 通常比 MEN-1 引起的 pNET 大得多。此外，与 MEN-1 患者相比，散发性疾病患者的 pNET 更为异质。两组患者的 pNET 边界、形态和血管化的定义没有统计学差异。
与 MEN-1 患者相比，散发性疾病患者通常会出现更大和更异质的 pNET，而 MEN-1 患者往往会出现更多的病变。EUS 可以促进 pNET 的精确特征描述，并且病变的超声特征可以帮助区分 MEN-1 相关疾病与散发性疾病。
