Rare Endocrinology News

Disease Profile

Hemangiopericytoma

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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Categories

Rare Cancers

Summary

Hemangiopericytoma is a term used to described a group of tumors that are derived from pericytes, the cells normally arranged along specific types of blood vessels called capillaries and venules. These types of tumors are typically slow-growing, may be either benign (non-cancerous) or malignant (cancerous), and may occur anywhere in the body.[1]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
5%-29% of people have these symptoms
Fatigue
Tired
Tiredness

[ more ]

0012378
Neoplasia of the pleura
0100527
Soft tissue neoplasm
0031459
Weight loss
0001824
1%-4% of people have these symptoms
Abnormality of the forehead
0000290
Abnormality of the mediastinum
0045026
Abnormality of the peritoneum
0002585
Constipation
0002019
Diplopia
Double vision
0000651
Fever
0001945
Hypoinsulinemia
0040216
Hypophosphatemic rickets
0004912
Loss of consciousness
Passing out
0007185
Low back pain
0003419
Neoplasm of the liver
Liver cancer
Liver tumor

[ more ]

0002896
Neoplasm of the lung
Lung tumor
0100526
Neoplasm of the nervous system
Tumor of the nervous system
0004375
Night sweats
0030166
Pelvic mass
0031501
Prostate cancer
0012125
Recurrent hypoglycemia
Recurrent low blood sugar levels
0001988
Reduced Cpeptide level
0030795
Urinary retention
0000016
Uterine neoplasm
Uterine tumor
0010784
Vaginal neoplasm
Vaginal tumor
0100650
Percent of people who have these symptoms is not available through HPO
Abnormality of the cardiovascular system
Cardiovascular abnormality
0001626
Autosomal recessive inheritance
0000007

Cause

The cause of the disease is unknown, and no strong clinical data exist to indicate a convincing link to specific causes. Some reports suggest a relationship between hemangiopericytoma and occupational vinyl chloride exposure, as well as exposure to herbicides.[2]

Treatment

Radical surgical resection with removal of all meningeal attachments is typically the preferred treatment. However this treatment option is generally possible in only 50-67% of patients who have meningeal hemangiopericytoma. Embolization prior to surgery is recommended because of the excessive bleeding associated with these tumors.[3] Embolization is a method of stopping the blood flow to the tumor. This can be done mechanicially or through the use of chemicals that cause blood vessels to close. If chemicals that kill cells are used during embolization the procedure is referred to as chemoembolization.[2]

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.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • 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 Hemangiopericytoma. Click on the link to view a sample search on this topic.

References

  1. Kumar: Robbins and Cotran: Pathologic Basis of Disease, 7th ed.. Saunders, An Imprint of Elsevier; 2005;
  2. Hogle WP. Malignant Hemangiopericytoma. Clinical Journal of Oncology Nursing. January/February 2003;
  3. Kaba SE, Kyritsis AP. Section 10: Neurological Malignancies. Chapter 58: Atypical and Malignant Malinomas. In: Raghavan D, Brecher ML, Johnson DH, Meropol NJ, Moots PL, Rose PG. Textbook of Uncommon Cancer Third Edition. England: John Wiley & Sons, Ltd; 2006;

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