Von Hippel-Lindau syndrome from the urologist and oncologist perspective - literature review

被引：0

作者：

Kowalik, Krzysztof ^{[1
]}

Naroznicki, Pawel ^{[2
]}

Hetman, Katarzyna ^{[3
]}

Kolaczyk, Katarzyna ^{[4
]}

Smogor, Artur ^{[5
]}

Kasperowicz, Krystian ^{[6
]}

Dutkiewicz, Grazyna ^{[7
]}

Gruszczynska, Anna ^{[8
]}

Modrzejewski, Andrzej ^{[1
]}

机构：

[1] Pomeranian Med Univ, Clin Dept Gen Surg, Ul Piotra Skargi 7?11, PL-70965 Szczecin, Poland

[2] Med Univ Lodz, Med Fac, Coll Mil Med, Fac Med, Lodz, Poland

[3] West Pomeranian Oncol Ctr, Dept Clin Oncol, Szczecin, Poland

[4] Pomeranian Med Univ, Univ Clin Hosp 1, Dept Imaging Diagnost & Intervent Radiol, Szczecin, Poland

[5] Med Off ARTIMED, Szczecin, Poland

[6] Northumbria Univ, Fac Hlth & Life Sci, Newcastle Upon Tyne, England

[7] Pomeranian Med Univ, Univ Clin Hosp 2, Dept Nephrol Transplantol & Internal Med, Szczecin, Poland

[8] Pomeranian Med Univ, Dept Forens Med, Szczecin, Poland

来源：

ONCOLOGY IN CLINICAL PRACTICE | 2024年

关键词：

von Hippel-Lindau syndrome; familiar angiomatosis of the retina and cerebellum; clear cell renal cell carcinoma; polycystic kidney disease; RENAL-CELL CARCINOMA; MALIGNANT PHEOCHROMOCYTOMAS; PRACTICE GUIDELINE; DISEASE; HEMANGIOBLASTOMAS; PARAGANGLIOMAS; CABOZANTINIB; MUTATION; FEATURES; THERAPY;

D O I：

10.5603/ocp.101438

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Von Hippel-Lindau (VHL) syndrome is a rare genetic disorder characterized by multiple retinal and cerebellar hemangioblastomas that tend to occur in a familial setting. The pattern of inheritance is autosomal dominant. Patients with VHL syndrome have an increased risk of clear cell renal cell carcinoma (ccRCC) as well as pancreatic and testicular cysts and cystadenomas. Tumors associated with VHL syndrome manifest at a young age. This article presents diagnostic methods and discusses the treatment approaches for this syndrome based on a global literature review.

引用

页数：8

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