PATHOLOGY MCQ
TUMOURS GENERAL FEATURES
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Question
[CLICK ON ANY CHOICE TO KNOW THE RIGHT ANSWER]
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In hereditary renal cell carcinoma (RCC), the following syndrome is implicated in carcinogenesis:
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Polycystic kidney disease
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Alports syndrome
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VHL syndrome
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Fabrys disease
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Explanation:
Detailed explanation-1: -Close to 60% of all patients with VHL will develop RCC or renal cysts. von Hippel-Lindau (VHL) disease is an autosomal dominant syndrome caused by a mutation in VHL, a tumor suppressor gene. VHL is found on chromosome 3 and, when mutated, can lead to uncontrolled cellular growth and oncogenesis.
Detailed explanation-2: -People with von Hippel-Lindau syndrome commonly develop cysts in the kidneys, pancreas, and genital tract. They are also at an increased risk of developing a type of kidney cancer called clear cell renal cell carcinoma and a type of pancreatic cancer called a pancreatic neuroendocrine tumor.
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