Wednesday 9 June 2004
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.
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
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
GLUT1- (glucose transporter-1)
RICH (rapidly involuting congenital hemangioma)
RETURN TO: vascular tumors
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