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Genetic and Rare Diseases Information Center (GARD)

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Non-involuting congenital hemangioma


Other Names for this Disease

  • NICH
  • Noninvoluting congenital hemangioma
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

My son was born with a vascular lesion in the right groin area.  A biopsy of the lesion suggested it is likely to be a non-involuting congenital hemangioma (NICH), but there is a possibility it is a kaposiform hemangioendothelioma.  I would like to know why it has not been possible to determine which of the two it actually is and why surgical excision will be necessary in the future.

Our Answer

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

What is a non-involuting congenital hemangioma (NICH)?

Non-involuting congenital hemangioma (NICH) is a rare type of infantile hemangioma, which is a tumor that forms from the abnormal growth of blood vessels in the skin.  NICH looks like an oval, purplish mark or bump that can occur on any part of the body.  NICH is present from birth (congenital) and increases in size as the child grows.  Unlike other hemangiomas, NICH do not disappear spontaneously (involute).[1]
Last updated: 12/11/2011

How is non-involuting congenital hemangioma diagnosed?

Non-involuting congenital hemangioma (NICH) is diagnosed by taking a biopsy of the skin mark and examining the tissue under a microscope.  NICH looks different under the microscope than most infantile hemangiomas because the blood vessels are arranged more irregularly.  Also, the cells in an NICH do not have glucose receptors, whereas the cells of almost all hemangiomas do have glucose receptors.  Finally, NICH is different from more common types of hemangiomas because NICH does not spontaneously disappear (involute). Instead, NICH remains stable over time.[1]
Last updated: 12/11/2011

Can non-involuting congenital hemangioma be distinguished from kaposiform hemangioendothelioma?

Confusion in the diagnosis of a non-involuting congenital hemangioma (NICH) may arise because NICH has similar characteristics to a type of hemangioendothelioma known as kaposiform hemangioendothelioma (KH), which is a rare cancer that forms from blood vessels in the skin.  Like NICH, the cells of a KH do not have glucose receptors and KH does not spontaneously disappear (involute).[2]  Because NICH and KH differ from infantile hemangiomas in the same ways, it may be difficult to diagnosis one or the other with certainty.
Last updated: 12/11/2011

How might non-involuting congenital hemangioma treated?

Because non-involuting congenital hemangioma (NICH) is quite rare, there are no established guidelines for the treatment of this condition.  However, the authors of one article on NICH suggest that there is no risk for excessive bleeding during the removal of an NICH and it is unlikely to regrow after surgery.[1]  Because NICH is a benign skin mark, surgery isn't necessary but can be considered to improve appearance of the skin.
Last updated: 12/11/2011

References
  • Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE. Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly. Plastic and Reconstructive Surgery. 2001; 107:1647-1654. http://www.ncbi.nlm.nih.gov/pubmed/11391180. Accessed 12/6/2011.
  • Lyons LL, North PE, Mac-Moune Lai F, Stoler MH, Folpe AL, Weiss SW. Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. American Journal of Surgical Pathology. 2004; 28:559-568. http://www.ncbi.nlm.nih.gov/pubmed/15105642. Accessed 12/6/2011.
Other Names for this Disease
  • NICH
  • Noninvoluting congenital hemangioma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.