LAUSR

A 32-year-old woman was admitted because she was incidentally found to have pancreatic tumors during medical evaluation. She had no complaints. Her abdomen was soft, non-tender, and no palpable masses were detected. Carcinoembryonic antigen, carbohydrate antigen 19-9, and hormone levels were within normal limits. Enhanced computed tomography (CT) showed a hypervascular mass, approximately 30 mm in diameter, in the head of the pancreas and several cystic lesions in the head, body, and tail of pancreas (Fig. 1a,b). The tail of pancreas had developed calcification. Renal cyst was also observed. Magnetic resonance imaging (MRI) revealed a hyperintensity on T2 and diffusion weighted imaging in the head of the pancreas (Fig. 1c). Magnetic resonance cholangiopancreatography (MRCP) showed multiple pancreatic cysts (Fig. 1d). Endoscopic ultrasound revealed a hypoechoic mass, approximately 30 × 21 mm in the head of pancreas, and multiple cystic lesions in the head, body, and tail of the pancreas. Mural nodules were not observed in the cyst. Brain computed tomography was within normal limits. Endoscopic ultrasound-guided fine needle aspiration biopsy was performed on the mass in the head of the pancreas, which revealed cells from a neuroendocrine tumor (NET). The patient underwent subtotal stomach-preserving pancreaticoduodenectomy. Pathologically, the mass in the head of the pancreas was reported as NET (G1); it was positive for the expression of synaptophysin and Ki67 (index <2%), and the cystic lesions were all reported as serous cystadenoma (SCN) (Fig. 2). She had no other abnormalities found apart from NET and SCN of the pancreas, She, however, had a family history of von Hippel–Lindau (VHL) disease and renal cell carcinoma. Therefore, she was diagnosed with VHL-associated mixed serous neuroendocrine neoplasm. VHL disease is an autosomal dominant genetic disorder affecting various organs such as retina, central nervous system, kidneys, adrenal glands, and pancreas. Pancreatic lesions were reported in 70% of VHL disease. Various pancreatic lesions such as pancreatic cysts, SCNs, adenocarcinomas, and NETs have been described in patients with VHL disease. Rarely, components of SCN and NET occur in mixed or solitary forms. Such mixed tumors are termed mixed serous neuroendocrine neoplasms (MSNNs) and are more frequently seen in association with VHL disease. The prognosis of MSNN depends on the malignant component in it. MSNN has been reported to have a higher malignant potential than SCN or NET alone. Therefore, close clinical follow up and aggressive surgical treatment is recommended.