Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant PotentialFrom the Radiologic Pathology Archives

Almost all neoplasms of the pancreas are derived from pancreatic epithelial components, including the most common pancreatic mass, primary pancreatic ductal adenocarcinoma (PDAC). Nonepithelial neoplasms comprise only 1%-2% of all pancreatic neoplasms. Although some may arise directly from intrapancreatic elements, many originate from mesenchymal, hematopoietic, or neural elements in the retroperitoneal peripancreatic space and grow into the pancreas. Once these tumors reach a certain size, it can be challenging to identify their origin. Because these manifest at imaging as intrapancreatic masses, awareness of the existence and characteristic features of these nonepithelial neoplasms is crucial for the practicing radiologist in differentiating these tumors from primary epithelial pancreatic tumors, an important distinction given the vastly different management and prognosis. In part 1 of this article, the authors reviewed benign nonepithelial neoplasms of the pancreas. This article focuses on malignant nonepithelial neoplasms and those of uncertain malignant potential that can be seen in the pancreas. The most common malignant or potentially malignant nonepithelial pancreatic tumors are of mesenchymal origin and include soft-tissue sarcomas, solitary fibrous tumor, and inflammatory myofibroblastic tumor. These tumors commonly manifest as large heterogeneous masses, often containing areas of necrosis and hemorrhage. The clinical features associated with these tumors and the imaging characteristics including enhancement patterns and the presence of fat or calcification help distinguish these tumors from PDAC. Hematopoietic tumors, including lymphoma and extramedullary plasmacytoma, can manifest as isolated pancreatic involvement or secondarily involve the pancreas as widespread disease. Hyperenhancing paragangliomas or hypervascular metastatic disease can mimic primary pancreatic neuroendocrine tumors or vascular anomalies.