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Microscopic pathology image showing Angiomatosis cutis. 4X.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Angiomatosis (Morphologic Abnormality)
  • Vascular Proliferation

What is Angiomatosis? (Definition/Background Information)

  • An Angiomatosis is a benign condition, wherein there is a growth of small blood vessels (capillaries) involving multiple tissue types (soft tissues), organs, muscles, and bones. They are known to appear, spread across a large section of the body
  • Angiomatosis frequently affects the lower limbs. Other regions where it occurs, are the abdomen, chest, and arms
  • They can cause a variety of health concerns, depending on the tumor location and size

Who gets Angiomatosis? (Age and Sex Distribution)

  • Angiomatoses usually form during the first twenty years of life, and are definitely noticeable before the mid-adult age of 40 years
  • Though it affects both males and females; there is a slight predilection for the female sex
  • There is no known ethnic/racial preference

What are the Risk Factors for Angiomatosis? (Predisposing Factors)

Risk factors are currently unknown; but, Angiomatosis are found to be present with the following disorders:

  • Bacillary Angiomatosis
  • Klippel-Trenaunay-Weber syndrome
  • Sturge-Weber syndrome
  • Von Hippel-Lindau disease

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Angiomatosis? (Etiology)

  • The exact cause of Angiomatosis occurrence is unknown. It may be caused by genetic factors
  • All lesions become evident by/after adulthood; the inception of which is thought to occur, either at the fetal stage, or after birth
  • It has been reported that all lesions are manifested before the age of 20 years

What are the Signs and Symptoms of Angiomatosis?

The presentations are based on the location of Angiomatosis. Signs and symptoms include:

  • Angiomatosis grows at a slow rate, but are known to alternatively recede and expand in size
  • Most common locations are the legs; other locations include the abdomen, chest wall, and hands/arms
  • Generally large Angiomatosis cause more explicit signs and symptoms than smaller tumor growths

How is Angiomatosis Diagnosed?

Diagnostic tests vary, based on location of the tumor. Angiomatosis is diagnosed by:

  • Physical exam with evaluation of medical history
  • CT, MRI scan of affected region
  • Biopsy of tissue: Histopathological studies conducted on a biopsy specimen by a pathologist

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Angiomatosis?

The complications from Angiomatosis could include:

  • If Angiomatosis occurs in brain tissues, then headaches, vision problems, difficulty in walking, and frequent falls, may occur
  • Recurrence of the tumor after surgery
  • Severe pain, depending on the tumor formation location
  • Damage to vital nerves, blood vessels, and surrounding structures during surgery
  • Side effects from chemotherapy (such as toxicity), radiation therapy: if these methods are employed for the treatment

How is Angiomatosis Treated?

Treatment measures for Angiomatosis include:

  • Wide surgical excision of Angiomatosis with removal of the entire lesion is the preferred method. If the tumor is not fully removed, then it might recur
  • When the lesion is not at an accessible location, or is unsafe for surgical intervention; other therapy modes may be employed
  • Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
  • Post-operative care is important: Minimum activity level is to be ensured until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Angiomatosis be Prevented?

Current medical research has not established a way of preventing Angiomatosis occurrence.

What is the Prognosis of Angiomatosis? (Outcomes/Resolutions)

  • Angiomatoses are benign, but symptomatic; hence, they may require some form of treatment. The treatment method adopted is based on the location of the lesion
  • Prognosis is generally excellent when the lesions are small. In such situations, their recurrence risk is also completely avoided
  • The probability of recurrence is high at nearly 50% (in some cases), after surgical removal of the tumor
  • If Angiomatosis occurs in the brain, then depending on the size and location, the prognosis will vary from excellent to possible death

Additional and Relevant Useful Information for Angiomatosis:

Venous Malformations and Arteriovenous Malformations used to be synonyms of Angiomatosis previously, but have now been redefined.

What are some Useful Resources for Additional Information?

Genetic Alliance     
4301 Connecticut Avenue NW Suite 404 Washington, DC 20008-2369    
Phone: (202) 966-5557    
Fax: (202) 966-8553    
Email: info@geneticalliance.org   
Website: http://www.geneticalliance.org

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue Danbury, CT 06810
Phone: (203) 744-0100
Toll-Free: (800) 999-6673
Fax: (203) 798-2291
Email: orphan@rarediseases.org
Website: http://www.rarediseases.org

References and Information Sources used for the Article:

Pathology & Genetics: Tumours of Soft Tissue and Bone; WHO Classification of Tumours; Edited by Christopher D.M. Fletcher, K. Krishnan Unni, Fredrik Mertens; IARC Press, Lyon, 2002

Helpful Peer-Reviewed Medical Articles:

Ghosh, S. B., Mala, Y. M., Tripathi, R., & Singh, A. (2011). Pelvic angiomatosis: an unusual cause of recurrent obstructed labor: a case report and review of literature. Arch Gynecol Obstet, 283(1), 127-129. doi: 10.1007/s00404-010-1420-6

Malik, R., Malik, R., Tandon, S., & Tandon, P. (2008). Skeletal angiomatosis - rare cause of bone destruction: a case report with review of literature. Indian J Pathol Microbiol, 51(4), 515-518.   

Pavanello, M., Piatelli, G., Ravegnani, M., Consales, A., Rossi, A., Nozza, P., . . . Cama, A. (2007). Cystic angiomatosis of the craniocervical junction associated with Chiari I malformation: case report and review of the literature. Childs Nerv Syst, 23(6), 697-700. doi: 10.1007/s00381-006-0274-5

Schmidt, D. (2005). [Retinal angiomatosis]. Klin Monbl Augenheilkd, 222(2), 90-109. doi: 10.1055/s-2005-857911

Schmidt, D., & Agostini, H. T. (2007). [Retinal angiomatosis- an ophthalmological challenge]. Klin Monbl Augenheilkd, 224(12), 905-921. doi: 10.1055/s-2007-963770

Kempf, V. A., Lebiedziejewski, M., Alitalo, K., Wälzlein, J. H., Ehehalt, U., Fiebig, J., ... & Grassl, G. (2005). Activation of hypoxia-inducible factor-1 in bacillary angiomatosis: evidence for a role of hypoxia-inducible factor-1 in bacterial infections. Circulation, 111(8), 1054-1062.

Schmidt, D., Natt, E., & Neumann, H. P. H. (2000). Long-term results of laser treatment for retinal angiomatosis in von Hippel-Lindau disease. European journal of medical research, 5(2), 47-58.

Wilson, C. L., Song, L. M. W. K., Chua, H., Ferrara, M., Devine, R. M., Dozois, R. R., & Nehra, V. (2001). Bleeding from cavernous angiomatosis of the rectum in Klippel-Trenaunay syndrome: report of three cases and literature review. The American journal of gastroenterology, 96(9), 2783.