Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC, USA.
Radiographics. 2010 Oct;30(6):1445-64. doi: 10.1148/rg.306105523.
Pancreatic endocrine tumors (PETs) are primarily well-differentiated tumors composed of cells that resemble normal islet cells but that arise from pancreatic ductal cells. They are classified as functioning or nonfunctioning according to their associated clinical symptoms; insulinoma, gastrinoma, and glucagonoma are the most common functioning PETs. They also are classified according to their biologic behavior, although all PETs have malignant potential. Most are sporadic, but some are associated with familial syndromes such as multiple endocrine neoplasia type 1, von Hippel-Lindau syndrome, and neurofibromatosis type 1. At imaging, PETs typically appear as well-defined hypervascular masses, a finding indicative of their rich capillary network. Cystic change, calcification, and necrosis are common in large tumors, which are associated with a poorer prognosis and a higher prevalence of local and vascular invasion and metastases than are smaller tumors. Even when metastases are present, many well-differentiated PETs have an indolent course. Poorly differentiated PETs are rare and have an infiltrative appearance; patients with such tumors have a poor prognosis. Knowledge of the characteristic clinical, pathologic, and radiologic features of PETs is important in the evaluation and management of patients with a suspected clinical syndrome or a pancreatic mass.
胰腺内分泌肿瘤（PET）主要是分化良好的肿瘤，由类似于正常胰岛细胞的细胞组成，但起源于胰腺导管细胞。根据其相关的临床症状，它们被分为功能性或无功能性；胰岛素瘤、胃泌素瘤和胰高血糖素瘤是最常见的功能性 PET。它们还根据其生物学行为进行分类，尽管所有 PET 都具有恶性潜能。大多数是散发性的，但有些与家族综合征有关，如多发性内分泌瘤 1 型、von Hippel-Lindau 综合征和神经纤维瘤病 1 型。在影像学上，PET 通常表现为界限清楚的富血管性肿块，这一发现表明其毛细血管网络丰富。囊性变、钙化和坏死在大肿瘤中很常见，与较小肿瘤相比，这些肿瘤预后较差，局部和血管侵犯及转移的发生率较高。即使存在转移，许多分化良好的 PET 也具有惰性病程。分化不良的 PET 很少见，呈浸润性外观；此类肿瘤患者预后不良。了解 PET 的特征性临床、病理和影像学特征对于评估和管理疑似临床综合征或胰腺肿块的患者非常重要。
