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

Hemangiopericytoma


See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.


Your Question

A family member has recently been diagnosed with hemangiopericytoma of the skull and a lesion in the lung and possibly the kidney.  We are interested in learning more about this condition, including the standard course of treatment, since we have not been able to obtain much information about this diagnosis.  Can you assist us?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is hemangiopericytoma?

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]
Last updated: 11/19/2013

What is known about "hemangiopericytomas of the skull?"

Hemangiopericytomas that occur in the brain typically arise from the meninges. These types of hemangiopericytomas are called meningeal hemangiopericytomas and are usually attached to the dura mater, not surrounded by a membrane, and sometimes invade the deep tissues of the brain. These tumors are made up of many blood vessels, and are often prone to bleeding. Meningeal hemangiopericytomas are rare, with an incidence of 2-4% of meningiomas. They occur more commonly in men who are in their 30s or 40s.[2]

Like other brain tumors, hemangiopericytomas may cause peripheral neuropathy or signs of increased intracranial pressure, such as headache, vertigo, nausea and vomiting, and/or visual disturbances.[3]
Last updated: 7/9/2013

What is known about hemangiopericytomas of the lung and near the kidneys?

Hemangiopericytomas in the lung, pelvis, and near the kidneys are generally discovered when a tumor has grown to a size that causes symptoms. Hemangiopericytoma of the lung is usually discovered when a patient has chest discomfort or shortness of breath with or without a cough. Hemangiopericytoma of the pelvis or near the kidneys cause urinary retention, hydroureter, or hydronephrosis; rare cases of constipation, abdominal swelling, or vomiting have been reported.[3]
Last updated: 7/9/2013

What causes hemangiopericytoma?

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.[3]
Last updated: 7/9/2013

What treatment is available for meningeal hemangiopericytoma?

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.[2] 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.[3]
Last updated: 11/19/2013

References
  • Kumar: Robbins and Cotran: Pathologic Basis of Disease, 7th ed.. Saunders, An Imprint of Elsevier; 2005;
  • 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;
  • Hogle WP. Malignant Hemangiopericytoma. Clinical Journal of Oncology Nursing. January/February 2003;