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NICH

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.

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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