Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Hereditary leiomyomatosis and renal cell cancer


Other Names for this Disease
  • Familial leiomyomatosis
  • HLRCC
  • LRCC
  • MCL
  • MCUL
More Names
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Treatment


Newline Maker

How might hereditary leiomyomatosis and renal cell cancer be treated?

Skin growths (cutaneous leiomyomas) associated with hereditary leiomyomatosis and renal cell cancer (HLRCC) should be examined by a dermatologist. Treatment of these may include surgery to remove a painful growth; cryoablation and/or lasers; and/or medications such as calcium channel blockers, alpha blockers, nitroglycerin, antidepressants, and/or antiepileptic drugs (AEDs), which have been reported to reduce pain.[1] Uterine fibroids should be evaluated by a gynecologist. These are typically treated in the same manner as those that occur in the general population. However, most women with HLRCC need medication and/or surgical removal of the fibroids (myomectomy) at a younger age. Medications may include gonadotropin-releasing hormone agonists (GnRHa), antihormonal drugs, and pain relievers. Hysterectomy should be performed only when necessary.[1] Early detection of kidney tumors in HLRCC is important because they grow aggressively. Total nephrectomy may be strongly considered in individuals with a detectable renal mass.[1]
Last updated: 1/9/2014

References
  1. Pithukpakorn M, Toro JR. Hereditary Leiomyomatosis and Renal Cell Cancer . GeneReviews. 2007; http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=hlrcc. Accessed 7/6/2009.


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

  • Orphanet lists clinical trials, research studies, and patient registries enrolling people with this condition. Click on Orphanet to view the list.

Medical Products

The medication(s) listed in the table(s) below have been approved by the Food and Drug Administration (FDA) for treatment of this condition. The FDA Office of Orphan Products Development designates "orphan products" for those that treat rare diseases affecting fewer than 200,000 Americans. The table(s) below may not be an exhaustive list of drugs or products used to treat this condition. There may be other products available that are not considered orphan products. To search for all FDA approved drugs, visit Drugs@FDA. You can find orphan products used to treat other conditions by searching the Orphan Drug Product Designation database.


Generic Name Sorafenib
Trade Name
(Manufacturer Name)
Nexavar
(Bayer HealthCare Pharmaceuticals)
Indication
The FDA has approved this product to be used in this manner.
Treatment of patients with advanced renal cell carcinoma

Generic Name Aldesleukin
Trade Name
(Manufacturer Name)
Proleukin®
(Novartis)
Indication
The FDA has approved this product to be used in this manner.
Treatment of adults (>18 years old) with metastatic renal cell carcinoma.
More Information about this product Drug Information Portal
Medline Plus Health Information

Generic Name Temsirolimus
Trade Name
(Manufacturer Name)
Torisel®
(Wyeth Pharmaceuticals, Inc.)
Indication
The FDA has approved this product to be used in this manner.
Treatment of advanced renal cell carcinoma
More Information about this product Drug Information Portal
Medline Plus Health Information