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Von Hippel-Lindau(VHL)disease is a rare autosomaldominant disorder caused by germ line mutations ofthe VHL tumour suppressor gene.It predisposes affectedindividuals to develop a variety of neoplasms,includinghaemangioblastomas of the central nervous system,retinal angiomas,renal cell carcinomas(RCCs),pheochromocytomas and cysts of the kidneys andepididymis.Germ line VHL mutations have been foundin all VHL disease families.RCC occurs in 25% to 45%of patients with VHL disease and is one of the leadingcauses of death.As the RCCs in VHL disease tend to bemultifocal and bilateral,the management of thembecomes complex.Here,we share our experience aboutthe management of solid renal tumours in 18 Chinesepatients with VHL disease.
Von Hippel-Lindau (VHL) disease is a rare autosomaldominant disorder caused by germ line mutations of the VHL tumor suppressor gene. It predisposes affected individuals to develop a variety of neoplasms, including haemangioblastomas of the central nervous system, retinal angiomas, renal cell carcinomas (RCCs) , pheochromocytomas and cysts of the kidneys andepididymis. Germ line VHL mutations have been foundin all VHL disease families. RCC occurs in 25% to 45% of patients with VHL disease and is one of the leading culses of death. As the RCCs in VHL disease tend to bemultifocal and bilateral, the management of thembecomes complex. Here, we share our experience about the management of solid renal tumors in 18 Chinesepatients with VHL disease.