NICH

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