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Disease Profile
Disseminated peritoneal leiomyomatosis
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.
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Age of onset
Adult
ICD-10
D20.1
Inheritance
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.
X-linked
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.
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.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
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)
Diffuse peritoneal leiomyomatosis; Leiomyomatosis peritonealis disseminate; DPL;
Categories
Digestive Diseases; Rare Cancers
Summary
Disseminated peritoneal leiomyomatosis (DPL) is a rare condition which is characterized by nodules or small lumps of smooth muscle
Symptoms
- Abdominal and
pelvic pain which is often associated with abnormal menstrual bleeding (dysmenorrhia) - Rectal bleeding
- Abnormally heavy bleeding during menstruation (menorrhagia)
- Constipation
- Intestinal obstruction
DPL may be discovered incidentally during a physical exam when masses may be felt in the abdomen. Since DPL usually does not produce any symptoms, the condition may also be unexpectedly found during a cesarean section (C-section) or abdominal surgery of another reason.[1][2][3]
Cause
Most cases occur sporadically in people with no
Diagnosis
Treatment
Related diseases
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
Conditions with similar signs and symptoms from Orphanet
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Differential diagnosis includes parasitic leiomyoma, intravenous leiomyomatosis and other primitive or secondary peritoneal carcinomatoses.
Visit the Orphanet disease page for more information.
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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
- 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.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Disseminated peritoneal leiomyomatosis. Click on the link to view a sample search on this topic.
References
- Bisceglia M, Galliani CA, Pizzolitto S, Ben-Dor D, Giannatempo G, Bergoli AL, & Aieta M. Selected case from the Arkadi M. Rywlin International Pathology Slide Series: Leiomyomatosis peritonealis disseminata: report of 3 cases with extensive review of the literature. Adv Anat Pathol. Adv Anat Pathol; 21(3):201-15. https://www.ncbi.nlm.nih.gov/pubmed/24713991. Accessed 12/21/2015.
- Zyla MM, Dzieniecka M, Kostrzewa M, Stetkiewicz T, Wilamowska A, Ksiezakowska-Lakoma K, & Wilczynki J R. Leiomyomatosis peritonealis disseminata of unusual course with malignant transformation: case report. Acta Obstet Gynecol Scand. 2015; 94:220-223. https://www.ncbi.nlm.nih.gov/pubmed/25546607. Accessed 12/21/2015.
- Bayrak S, Pasaoglu E, Cakar E, Bektas H, Colak S, Sevinc MM & Kinaci E. Disseminated peritoneal leiomyomatosis with chronic constipation: a case report. Journal of Medical Case Reports. 2014; 8:114-118. https://www.jmedicalcasereports.com/content/pdf/1752-1947-8-114.pdf. Accessed 12/21/2015.
- Stewart EA & Quade BJ. Variants of uterine leiomyomas (fibroids0. UpToDate. February 18, 2014; https://www.uptodate.com/contents/variants-of-uterine-leiomyomas-fibroids. Accessed 12/21/2015.
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