title: Pancreatic mixed serous neuroendocrine neoplasm with clear cells leading to diagnosis of von Hippel Lindau disease.
abstract: 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.
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India.
authors.forename: Aanchal
authors.initials: A
authors.lastname: Kakkar
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India. Electronic address: sharmamehar@yahoo.co.in.
authors.forename: Mehar C
authors.initials: MC
authors.lastname: Sharma
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India.
authors.forename: Rajni
authors.initials: R
authors.lastname: Yadav
authors.affiliation: Department of Gastrointestinal Surgery, All India Institute of Medical Sciences, New Delhi 110029, India.
authors.forename: Rajesh
authors.initials: R
authors.lastname: Panwar
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India.
authors.forename: Sandeep R
authors.initials: SR
authors.lastname: Mathur
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India.
authors.forename: Venkateswaran K
authors.initials: VK
authors.lastname: Iyer
authors.affiliation: Department of Gastrointestinal Surgery, All India Institute of Medical Sciences, New Delhi 110029, India.
authors.forename: Peush
authors.initials: P
authors.lastname: Sahni
mesh_terms: D000328:Adult; D018293:Cystadenoma, Serous / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008297:Male; D018358:Neuroendocrine Tumors / Q000209:etiology* / Q000473:pathology; D010179:Pancreas / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000209:etiology* / Q000473:pathology; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000175:diagnosis* / Q000473:pathology
keywords: Mixed tumor; Neuroendocrine; Pancreas; Serous cystadenoma; Von Hippel Lindau disease