Short answer · Medically reviewed summary · Last updated: 2023-07-13

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.

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

Prevalence of Hereditary Leiomyomatosis and Renal Cell Carcinoma: how many people are affected worldwide, differences by sex and region, with sources.

Prevalence of Hereditary Leiomyomatosis and 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.


Author: DiseaseMaps Editorial Team
Reviewed against authoritative medical sources (NIH GARD, Orphanet, OMIM)
Last updated: 2023-07-13
Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
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