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Disease Profile

Intravascular papillary endothelial hyperplasia

Prevalence
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.

Unknown

Age of onset

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ICD-10

#N/A

Inheritance

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

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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

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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.

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

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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.

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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.

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Not applicable

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Other names (AKA)

Masson's tumor; Masson's vegetant intravascular hemangio-endothelioma; Masson's pseudoangiosarcoma

Summary

Intravascular papillary endothelial hyperplasia (IPEH) is a non-cancerous tumor that occurs due to the overgrowth of the cells that line the blood vessels. Symptoms of IPEH may include a soft mass under the skin and reddish-blue coloring on the skin. Other symptoms may depend on the location of the IPEH. The most common areas of the body for IPEH to occur are the head, neck, lower limbs, and fingers. If left untreated, the tumor can cause damage to the tissue around it and become infected. The cause is unknown. Diagnosis is made by imaging studies and a biopsy to remove a piece of the tumor for examination by a pathologist. Treatment usually includes surgery to remove the tumor.[1][2][3][4]

Symptoms

The following list includes the most common signs and symptoms in people with intravascular papillary endothelial hyperplasia (IPEH). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.

Symptoms may include:[1][3]

  • Painless slow growing mass
  • Red-blue color on the skin

These tumors can grow anywhere on the body. They most often appear on the head, neck, lower limbs, and fingers. They can also grow on bone. If the IPEH is untreated, it may cause damage to the tissue around it and become infected. IPEH is a noncancerous type of tumor, and once it is removed, it rarely comes back.[2]

Cause

The cause of intravascular papillary endothelial hyperplasia is unknown. It may be due to a combination of factors, including previous trauma or a history of a vein or artery condition.[2][4]

Diagnosis

Intravascular papillary endothelial hyperplasia (IPEH) is diagnosed based on the symptoms, clinical exam, imaging studies, and a biopsy to look at a small piece of the tumor under the microscope.[1][3] It may be necessary to exclude other more common conditions before IPEH can be diagnosed.

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.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Intravascular papillary endothelial hyperplasia. Click on the link to view a sample search on this topic.

References

  1. Sasso SE, Naspolini AP, Milanez TB, Suchard G. Masson's tumor (intravascular papillary endothelial hyperplasia). An Bras Dermatol. Sep-Oct 2019; 94(5):620-621. https://pubmed.ncbi.nlm.nih.gov/31777368.
  2. Mirmohammadsadeghi H, Mashhadiabbas F, Latifi F. Huge central intravascular papillary endothelial hyperplasia of the mandible: a case report and review of the literature. J Korean Assoc Oral Maxillofac Surg. Aug 2019; 45(4):180-185. https://pubmed.ncbi.nlm.nih.gov/31508349.
  3. Shim HK, Kim MR. Intravascular Papillary Endothelial Hyperplasia of the Vocal Cord: A Case Report and Review of the Literature. Am J Case Rep. Nov 12, 2019; 20:1664-1668. https://pubmed.ncbi.nlm.nih.gov/31712548.
  4. Han JW, Lee JH, Kim EK. Intravascular papillary endothelial hyperplasia (Masson's hemangioma) of the face. Arch Craniofac Surg. Apr 2019; 20(2):109-111. https://pubmed.ncbi.nlm.nih.gov/30703866.

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