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2013
von Hippel-Lindau (VHL) disease is an autosomal-dominant familial cancer syndrome associated with mutations of the VHL tumor suppressor gene (3p25-26). Its estimated incidence ranges from 1 in 36,000 to 1 in 53,000 with a penetrance of up to 95% by age 60. Genotype-phenotype correlation divides VHL into two broad clinical subtypes.
Angela, Chou +3 more
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von Hippel-Lindau (VHL) disease is an autosomal-dominant familial cancer syndrome associated with mutations of the VHL tumor suppressor gene (3p25-26). Its estimated incidence ranges from 1 in 36,000 to 1 in 53,000 with a penetrance of up to 95% by age 60. Genotype-phenotype correlation divides VHL into two broad clinical subtypes.
Angela, Chou +3 more
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Community Oncology, 2004
Von Hippel-Lindau syndrome (VHL) is an autosomal-dominant heritable neoplastic disorder in which multiple benign and malignant neoplasms and cysts of specific histopathologies develop in the kidney, adrenal gland, pancreas, brain, spinal cord, eye, inner ear, epididymis, and broad ligament, associated with germ-line mutations in the VHL tumor ...
Gladys M. Glenn +8 more
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Von Hippel-Lindau syndrome (VHL) is an autosomal-dominant heritable neoplastic disorder in which multiple benign and malignant neoplasms and cysts of specific histopathologies develop in the kidney, adrenal gland, pancreas, brain, spinal cord, eye, inner ear, epididymis, and broad ligament, associated with germ-line mutations in the VHL tumor ...
Gladys M. Glenn +8 more
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2010
Von Hippel-Lindau syndrome (VHLS) is an autosomal dominant familial cancer syndrome arising from germ-line inactivation of the VHL gene on the short arm of chromosome 3. VHLS manifests in a myriad of hyper-vascular tumors of both benign and malignant nature.
Jenny J, Kim +2 more
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Von Hippel-Lindau syndrome (VHLS) is an autosomal dominant familial cancer syndrome arising from germ-line inactivation of the VHL gene on the short arm of chromosome 3. VHLS manifests in a myriad of hyper-vascular tumors of both benign and malignant nature.
Jenny J, Kim +2 more
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Applied Radiology
Abstract VHL is a rare tumor predisposition syndrome characterized by tumors such as hemangioblastoma, renal cell carcinoma, and pheochromocytoma. Although patients are often diagnosed within the 2nd to 3rd decade of life, patients may present with tumors during childhood.
Natasha Gardiner +3 more
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Abstract VHL is a rare tumor predisposition syndrome characterized by tumors such as hemangioblastoma, renal cell carcinoma, and pheochromocytoma. Although patients are often diagnosed within the 2nd to 3rd decade of life, patients may present with tumors during childhood.
Natasha Gardiner +3 more
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2018
Von Hippel–Lindau syndrome (VHL) is a heritable neoplastic disease resulting in multisystem carcinoma, benign tumors, and cysts. VHL is inherited in an autosomal dominant manner and could result in benign or malignant tumors, with high penetrance affecting multiple organ systems, including the central nervous system (CNS) and visceral organs [1 ...
Jie Zhou, Xiaoran Li
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Von Hippel–Lindau syndrome (VHL) is a heritable neoplastic disease resulting in multisystem carcinoma, benign tumors, and cysts. VHL is inherited in an autosomal dominant manner and could result in benign or malignant tumors, with high penetrance affecting multiple organ systems, including the central nervous system (CNS) and visceral organs [1 ...
Jie Zhou, Xiaoran Li
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1998
Abstract von Rippel-Lindau syndrome (VHL) is a cancer-prone inherited disorder of adulthood involv ing many organs and organ systems (Figs 26.1 and 26.2, Table 26.1). The reported prevalence in different populations was 1: 38000-1: 53000 (Maher et al. 1991; Neumann and Wiestler 1991).
Hartmut P H Neumann +3 more
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Abstract von Rippel-Lindau syndrome (VHL) is a cancer-prone inherited disorder of adulthood involv ing many organs and organ systems (Figs 26.1 and 26.2, Table 26.1). The reported prevalence in different populations was 1: 38000-1: 53000 (Maher et al. 1991; Neumann and Wiestler 1991).
Hartmut P H Neumann +3 more
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Imaging Diagnosis of Von Hippel-Lindau Syndrome
Journal of Craniofacial Surgery, 2019Introduction: Von Hippel-Lindau (VHL) syndrome is a group of rare autosomal dominant hereditary diseases that involve multiple organs. Due to its high recurrence rate and complex and diverse clinical manifestations, VHL is prone to being either misdiagnosed or missed entirely.
Nan, Xu +3 more
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Calcified Pheochromocytoma in Von Hippel-lindau Syndrome
Journal of Urology, 1979AbstractUrologic manifestations of the von Hippel-Lindau syndrome include benign and malignant renal tumors and cysts, epididymal cysts and adenomas, adrenal cortical tumors and pheochromocytomas. A rare case of a calcified pheochromocytoma in a patient with the von Hippel-Lindau syndrome is reported.
D L, Harper +5 more
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Renal cell carcinoma in von hippel-lindau syndrome
The American Journal of Medicine, 1987Thirty-seven cases of von Hippel-Lindau syndrome are reported. The urinary tract was studied in 23 patients; 15 of them had the renal lesions. Eight of these 15 patients had renal cell carcinoma, which was bilateral in six. Because 80 percent of these patients are seen initially by neurologists or ophthalmologists, the potential for the development of ...
R S, Malek +3 more
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Klinische Monatsblatter fur Augenheilkunde, 1984
The von Hippel-Lindau syndrome is an autosomal dominant condition that comprises, apart from angiomas of the retina, the cerebellum, the spinal cord, and the cerebrum, also cystic and blastomatous dysplasias resulting from maldevelopment, namely cystic kidney and pancreas, hypernephroma, and pheochromocytoma.
H, Reich, F, Hollwich
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The von Hippel-Lindau syndrome is an autosomal dominant condition that comprises, apart from angiomas of the retina, the cerebellum, the spinal cord, and the cerebrum, also cystic and blastomatous dysplasias resulting from maldevelopment, namely cystic kidney and pancreas, hypernephroma, and pheochromocytoma.
H, Reich, F, Hollwich
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