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NICH

Wednesday 9 June 2004

A CLINICAL: NICH (noninvoluting congenital hemangioma) is a glut-1 negative congenital hemangioma that does not regress with time. RICH (rapidly involuting congenital hemangioma) and infantile hemangioma regress after birth. HISTOPATHOLOGY: distinctive features include 1) prominent arteries and abnormal veins with arteriolobular and arteriovenous fistulae and 2) hobnail endothelial cells. However, NICH removed early (2-4 years) is often similar or histologically indistinguishable to RICH. Synopsis - rare, congenital, cutaneous vascular anomaly - grows proportionately with the child and does not regress. - slightly more frequent occurence in male patients - always appeared alone - location in order of frequency: -* head/neck region -* extremities -* trunk - round-to-ovoid in shape - plaque-like or bossed - variable shades of pink to purple - average diameter of 5 cm - overlying skin frequently punctuated by coarse telangiectasia, often with central or peripheral pallor - warm on palpation - fast-flow by Doppler ultrasonography - lobular collections of small, thin-walled vessels - large, often stellate, central vessel - interlobular areas containing predominantly dilated, often dysplastic veins; - arteries also increased in number - small arteries "shunting" directly into lobular vessels or into abnormal extralobular veins - hobnailed endothelial cells lined the small intralobular vessels - mast cells increased Immunochemistry - GLUT1- (glucose transporter-1) DIFFERENTIAL: - RICH (rapidly involuting congenital hemangioma) - infantile hemangioma RETURN TO: vascular tumors References - Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE. Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr Surg. 2001 Jun;107(7):1647-54. PMID: 11391180

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