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Familial islet cell tumors in von Hippel‐Lindau's disease
Author(s) -
Hull Meredith T.,
Warfel Kathleen A.,
Muller Jans,
Higgins James T.
Publication year - 1979
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(197910)44:4<1523::aid-cncr2820440452>3.0.co;2-0
Subject(s) - von hippel–lindau disease , medicine , pheochromocytoma , pancreas , pathology , multiple endocrine neoplasia , renal cell carcinoma , clear cell , islet , disease , endocrinology , diabetes mellitus , biology , genetics , gene
Von Hippel‐Lindau's Disease is an hereditary disorder characterized by the development of hemangioblastomas of the cerebellum and retina and a variety of cystic and neoplastic lesions of other organs such as renal cell carcinoma and pheochromocytoma. In a single generation of a family with Von Hippel‐Lindau's disease, all four siblings developed lesions classically associated with the complex. Additionally, two of the four developed islet cell tumors of the pancreas, one in one patient and five in the other. While a familial incidence of islet cell tumors is known in multiple endocrine adenomatosis, type I and Zollinger‐Ellison syndrome, such a familial occurrence has been heretofore unrecorded in the Von Hippel‐Lindau complex.