Pancreatic mixed serous neuroendocrine neoplasm with clear cells leading to diagnosis of von Hippel Lindau disease

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• 作者：Aanchal Kakkar^a ; Mehar C. Sharma^a ; ^{sharmamehar@yahoo.co.in" class="auth_mail" title="E-mail the corresponding author} ; Rajni Yadav^a ; Rajesh Panwar^b ; Sandeep R. Mathur^a ; Venkateswaran K. Iyer^a ; Peush Sahni^b
• 关键词：Serous cystadenoma ; Neuroendocrine ; Mixed tumor ; Pancreas ; Von Hippel Lindau disease
• 刊名：Pathology - Research and Practice
• 出版年：2016
• 出版时间：August 2016
• 年：2016
• 卷：212
• 期：8
• 页码：747-750
• 全文大小：2187 K

文摘

Mixed serous neuroendocrine neoplasms are extremely rare tumors that are usually seen in female patients and are often associated with von Hippel Lindau (VHL) disease. We describe the case of a 38-year-old male who presented with complaints of anorexia, weight loss, and abdominal pain. CT abdomen showed a mass in the head of the pancreas, multiple small nodules in the body of pancreas, and bilateral adrenal masses. Fine needle aspiration cytology (FNAC) from the mass showed features of a neuroendocrine tumor, with many of the cells demonstrating abundant clear cytoplasm. Histopathological examination of the pancreaticoduodenectomy specimen showed a mixed serous neuroendocrine neoplasm with two components viz. serous cystadenoma and neuroendocrine tumor (NET) World Health Organization (WHO) grade 2. In addition, he was diagnosed to have bilateral pheochromocytomas and a paraganglioma. The synchronicity of these tumors suggested the possibility of VHL disease. Thus, identification of a NET with clear cells or of a mixed serous neuroendocrine neoplasm should raise suspicion of VHL disease. In a mixed tumor, FNAC may identify only one of the two components. Thorough processing of all pancreatic serous tumors for pathological examination is recommended, as NET may occur as a small nodule within the serous cystadenoma.