Endoscopic ultrasound-guided fine-needle aspiration diagnosis of clear-cell pancreatic endocrine neoplasm in a patient with von Hippel-Lindau disease: a case report.

摘要: The cytologic findings of a clear-cell pancreatic endocrine neoplasm (PEN) diagnosed by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) occurring in 29-year-old man with von Hippel-Lindau (VHL) syndrome are described. Imaging studies showed multiple masses and cysts, single liver lesion, pulmonary renal cysts. Cytologic features this PEN included the presence large sheets rounded clusters polygonal neoplastic cells relatively abundant cytoplasm containing numerous, small, sharply-demarcated vacuoles that occasionally indented nuclei gave "frothy" appearance. Mild anisonucleosis was present nucleoli were visible. Rare stripped seen. Small vessels transgressed tumor cell sheets. These distinct from those typical neoplasms, bear resemblance to carcinoma metastatic pancreas. To best our knowledge, is first detailed report EUS-FNA associated VHL syndrome. We believe distinctive characteristic features, together immunohistochemical studies, can allow preoperative diagnosis highly unusual lesion avoid possible confusion other neoplasms pancreas, particularly carcinoma.