THORAX ABDOMEN AND PELVIS

MEDICAL

KIDNEY

Question [CLICK ON ANY CHOICE TO KNOW THE RIGHT ANSWER]
In hereditary renal cell carcinoma (RCC), the following syndrome is implicated in carcinogenesis:
A
Polycystic kidney disease
B
Alports syndrome
C
VHL syndrome
D
Fabrys disease
Explanation: 

Detailed explanation-1: -There are four major hereditary renal cell carcinoma (RCC) syndromes. These syndromes are summarized in detail in the following PDQ summaries and in Table 1 below: Von Hippel-Lindau Disease (VHL). Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC). Hereditary Papillary Renal Carcinoma (HPRC).

Detailed explanation-2: -More than 55% of von Hippel-Lindau disease (VHL)-affected individuals only develop multiple renal cell cysts. The VHL-associated renal cell carcinomas (RCCs) that occur are characteristically multifocal and bilateral; these RCCs present as combined cystic and solid masses.

Detailed explanation-3: -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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