title: [Von Hippel-Lindau disease type 2-related pancreatic neuroendocrine tumor and adrenal myelolipoma].
abstract: The paper describes a case of von Hippel--Lindau-related pancreatic neuroendocrine tumor and adrenal myelolipoma in a 44-year-old woman. The pancreatic tumor and a left retroperitoneal mass were removed in the women in July 2014 and May 2015. Histological examination of the pancreatic tumor revealed that the latter consisted of clear cells forming tubular and tubercular structures showing the expression of chromogranin A, synaptophysin, and cytokeratins 18 and 19 and a negative response to CD10 and RCC. The adrenal medullary mass presented as clear-cell alveolar structures with inclusions of adipose tissue mixed with erythroid, myeloid, and lymphoid cells. The clear-cell component of the adrenal gland expressed neuroendocrine markers with a negative response to cytokeratins, CD10, and RCC. Molecular genetic examination yielded a signal corresponding to two copies of the VHL gene. No deletions or amplifications of the gene were detected. Cases of von Hippel--Lindau disease concurrent with adrenal pheochromocytoma and myelolipoma and simultaneous pancreatic involvement were not found in the literature.
authors.affiliation: Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow.
authors.forename: O V
authors.initials: OV
authors.lastname: Dolzhansky
authors.affiliation: Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow.
authors.forename: M M
authors.initials: MM
authors.lastname: Morozova
authors.affiliation: Research Center for Medical Genetics, Moscow, Russia.
authors.forename: S A
authors.initials: SA
authors.lastname: Korostelev
authors.affiliation: Research Center for Medical Genetics, Moscow, Russia.
authors.forename: I V
authors.initials: IV
authors.lastname: Kanivets
authors.affiliation: Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow.
authors.forename: N K
authors.initials: NK
authors.lastname: Chardarov
authors.affiliation: Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow.
authors.forename: G A
authors.initials: GA
authors.lastname: Shatveryan
authors.affiliation: Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow.
authors.forename: E M
authors.initials: EM
authors.lastname: Paltseva
authors.affiliation: Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow.
authors.forename: D N
authors.initials: DN
authors.lastname: Fedorov
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D000328:Adult; D002869:Chromosome Aberrations; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D018209:Myelolipoma / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D009363:Neoplasm Proteins / Q000096:biosynthesis / Q000235:genetics; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D057089:Pathology, Molecular; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*
chemical_list: D009363:Neoplasm Proteins