Rare Hematology News

Disease Profile

Pelvic lipomatosis

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.


US Estimated

Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

Excess of mature unencapsulated fatty tissue in the pelvis


Pelvic lipomatosis is a rare disease of unknown cause, characterized by overgrowth of mature, non-cancerous fat cells in the pelvic region. The symptoms of pelvic lipomatosis are related to the compression of the urinary system (urinary frequency, dysuria, nocturia, and hematuria), lower intestinal tract (constipation, tenesmus, rectal bleeding, and ribbon-like stools with mucus), and vascular system (edema of lower extremities). Pelvic lipomatosis has been linked to chronic lower urinary tract infections, obesity, and endocrine dysfunction. Treatment usually seeks to alleviate symptoms, especially kidney obstruction and end stage renal disease. While most cases are not progressive, upper-tract urinary surgery may be needed for those with severe symptoms and/or evidence of urinary obstruction with deterioration of the kidney function.[1]


To date, weight-loss has not been shown to be effective in managing pelvic lipomatosis. Treatment options for pelvic lipomatosis may include watchful waiting or surgery. Unfortunately, less invasive treatments such as antibiotics, steroids, and radiation therapy are not helpful.[2]

Currently there are no well established treatment guidelines for pelvic lipomatosis. It can likewise be difficult to find doctors who have clinical experience with this rare condition. The following organization has a medical advisory board, physician locator services, and patient networks which may help you find doctors who are experienced in treating pelvic lipomatosis. While the group does not offer information on pelvic lipomatosis currently, they do advocate for all people with rare fat disorders.

Fat Disorders Research Society
2305 Robincrest Ln
Glenview, IL 60025
E-mail: https://www.fatdisorders.org/contact-fdrs/
Website: https://www.fatdisorders.org


Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Social Networking Websites

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Pelvic lipomatosis. Click on the link to view a sample search on this topic.


        1. Rodriguez, JR & Malik, A. Pelvic Lipomatosis. Applied Radiology. 2003; 32(4):https://www.medscape.com/viewarticle/452493. Accessed 6/10/2011.
        2. Ali A, Swain S, Manoharan M. Pelvic lipomatosis: Bladder sparing extirpation of pelvic mass to relieve bladder storage dysfunction symptoms and pelvic pain. Cent European J Urol. 2014; 67(3):287-8. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4165674/#CIT0001. Accessed 2/16/2016.

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