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What is the prevalence of Hereditary Leiomyomatosis and Renal Cell Carcinoma?

How many people does Hereditary Leiomyomatosis and Renal Cell Carcinoma affect? Does it have the same prevalence in men and women? And in the different countries?

Prevalence of Hereditary Leiomyomatosis and Renal Cell Carcinoma

The prevalence of Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC) is relatively rare. It is estimated to affect approximately 1 in 1,000,000 individuals worldwide. HLRCC is an autosomal dominant genetic disorder caused by mutations in the FH gene. This condition is characterized by the development of leiomyomas (benign smooth muscle tumors) in the skin and uterus, and an increased risk of renal cell carcinoma (a type of kidney cancer). Early diagnosis and regular surveillance are crucial for individuals with a family history of HLRCC to detect and manage potential renal cell carcinoma.



Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC) is a rare genetic condition characterized by the development of benign smooth muscle tumors (leiomyomas) in the skin and uterus, and an increased risk of developing aggressive kidney cancer (renal cell carcinoma).


The prevalence of HLRCC is estimated to be very low, with only a few hundred families worldwide identified with this condition. It is inherited in an autosomal dominant manner, meaning that an affected individual has a 50% chance of passing the gene mutation to each of their children.


While the exact prevalence of HLRCC is difficult to determine due to its rarity, it is considered an extremely rare condition. The scarcity of reported cases suggests that it occurs in less than 1 in 200,000 individuals in the general population.


Given its rarity, it is crucial for individuals with a family history of HLRCC or symptoms associated with the condition to seek genetic counseling and testing. Early detection and appropriate management can significantly improve outcomes for those affected by HLRCC.


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