Клинико-морфологическое наблюдение высокодифференцированной нейроэндокринной опухоли головки поджелудочной железы на фоне синдрома фон Гиппеля–Линдау

N.V Kretova,I.m Sechenov First Moscow State Medical University, Moscow ,A.A Kharlamova,O.A Lobanova,T.G Volodkina,N.B Paramonova,E.E Rudenko,E.A Kogan,T.A Demura,I.m Sechenov First Moscow State Medical University, Moscow ,I.m Sechenov First Moscow State Medical University, Moscow ,I.m Sechenov First Moscow State Medical University, Moscow ,I.m Sechenov First Moscow State Medical University, Moscow ,I.m Sechenov First Moscow State Medical University, Moscow ,I.m Sechenov First Moscow State Medical University, Moscow ,I.m Sechenov First Moscow State Medical University, Moscow

doi:10.31088/cem2024.13.2.61-66

Abstract

Von Hippel-Lindau (VHL) disease is a rare autosomal dominant genetic disease characterized by the formation of benign and malignant tumors. From 10 to 20% of patients develop a pancreatic neuroendocrine tumor (panNET). This article presents a clinical and morphological case that describes a well-differentiated neuroendocrine tumor of the pancreatic head accompanied by VHL syndrome. Positive Synaptophysin and Chromagranin A staining of tumor cells confirmed the diagnosis. The proliferation index (Ki-67) was less than 3%. In addition, serous cystadenoma without signs of neuroendocrine differentiation was found in the pancreatic tail, which was confirmed by positive reaction with antibodies to CA IX and MUC6 and a negative reaction with antibodies to Synapthophysin in the epithelial cells. Keywords: von Hippel-Lindau syndrome, neuroendocrine tumor, pancreas, immunohistochemical study

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