Infantile Hemangiomas Involving the Neuraxis: Clinical and Imaging Findings

V Viswanathan,D.B Orbach,J.B Mulliken,E.R Smith,H.P.W Kozakewich,S.J Fishman,P.E Burrows

doi:10.3174/ajnr.a1478

Abstract

The neuroradiology and neurosurgery literature is replete with references to "hemangioma" involving the central nervous system (CNS). However, the number of cases of true infantile hemangiomas in the CNS reported to date is 15. Our purpose was to delineate the definition of infantile hemangiomas, determine their prevalence in the neuraxis, and describe their imaging characteristics and associations in this location. We reviewed our Vascular Anomalies Center data base from 1999 through May 2008 to assess the prevalence of intracranial or intraspinal involvement within the total cohort of infantile hemangiomas. Fifteen patients were identified with infantile hemangiomas that involved the neuraxis. Two board-certified neuroradiologists reviewed the available imaging of these 15 patients, and a board-certified pathologist reviewed the available histopathology. Clinical records of all 15 patients were reviewed to identify the type of treatment and the treatment response. Of the 1454 patients listed with infantile hemangioma, 15 (approximately 1.0%) had involvement of the CNS. Eight patients had intracranial infantile hemangioma, 6 had intraspinal hemangioma, and 1 had both. In most instances, there was continuous extension into the neuraxis from an extracranial or extraspinal lesion. There were no cases of a CNS hemangioma without an accompanying extra-CNS tumor. Two patients had findings consistent with posterior fossa anomalies, cervicofacial hemangioma, arterial anomalies, cardiac defects, ocular abnormalities, and associated sternal or ventral defect. Of note, there were no brain or spinal parenchymal signal-intensity abnormalities, and there was no evidence of parenchymal invasion. CNS involvement by infantile hemangiomas is an unusual occurrence, which, when recognized, can help optimize patient management.

Highlights

AND PURPOSE: The neuroradiology and neurosurgery literature is replete with references to “hemangioma” involving the central nervous system (CNS)
Of the 1454 patients listed with infantile hemangioma, 15 (ϳ1.0%) had involvement of the CNS
The classification of vascular anomalies proposed by Mulliken and Glowacki in 19821 was adopted by the International Society for the Study of Vascular Anomalies (ISSVA) in 1996.2 There are 2 major categories in this system based on clinical and cellular behavior: vascular tumors and vascular malformations

Summary

Methods

We reviewed our Vascular Anomalies Center data base from 1999 through May 2008 to assess the prevalence of intracranial or intraspinal involvement within the total cohort of infantile hemangiomas. We reviewed all of the records of our Vascular Anomalies Center data base from 1999 through May 2008, most of which were referrals from outside sources, by using the search terms “hemangioma,” “head and neck,” “intracranial,” “periorbital,” “skull base,” “intraspinal,” “spinal,” “coccygeal,” “PHACE,” and “PHACES.” We identified a total of 1454 patients with a diagnosis of infantile hemangioma based on clinical and imaging characteristics. In this cohort, there were 15 patients in whom infantile hemangioma involved the intracranial or intraspinal compartments. All 15 patients’ clinical records were reviewed to identify the treatment each patient received and the response

Results

Discussion

Conclusion