Infantile hemangiomas involving the neuraxis: clinical and imaging findings

Abstract

Background and purpose: 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.

Materials and 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. 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.

Results: 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.

Conclusions: CNS involvement by infantile hemangiomas is an unusual occurrence, which, when recognized, can help optimize patient management.

Fig 1.

Gross appearance of cutaneous lesions overlying infantile hemangiomas involving the neuraxis (patient 12). A, A 4-month-old female infant presented with multiple bleeding scalp hemangiomas and anemia (patient 3). B, Another 4-month-old female infant presented with a large flat pink stain over the lower back, with a macular network-like structure consistent with the reticular pattern of spinal hemangioma. This appearance has been associated with underlying ventral-caudal structural anomalies.

Fig 2.

Typical MR imaging findings of neuraxis hemangioma (patient 3). Sagittal precontrast (A) and postcontrast (B), and axial postcontrast T1- (C) and T2-weighted (D) images demonstrate a well-circumscribed lesion in the fourth ventricle with extension into the left foramen of Luschka. The lesion is isointense on T1-weighted imaging, hyperintense on T2-weighted imaging, and enhances avidly and uniformly. Note the lack of normal enhancement within the torcular herophili, consistent with thrombosis.

Fig 3.

Infantile spinal hemangioma (patient 14). Sagittal T1 precontrast (A), postcontrast (B), and T2-weighted (C) images and an axial postcontrast T1-weighted image (D) demonstrate avidly enhancing hemangioma within the extradural compartment of the lower thoracic and upper lumbar spine. Note the prominent flow voids indicative of the proliferative phase of infantile hemangioma.

Fig 4.

Typical CT findings of neuraxis hemangioma (patient 2). Precontrast (A) and postcontrast (B−D) images demonstrate an isoattenuated lobulated intensely enhancing mass within the left periorbital/orbital soft tissues with posterior extension to the left cavernous sinus and the left cerebellopontine angle (B). In addition, focal enhancement is seen within the left internal auditory canal (D). C, Tortuous vessels are noted adjacent to the left internal carotid artery terminus.

Fig 5.

Infantile hemangioma in the right cerebellopontine angle cistern (patient 6). Axial T1 precontrast (A), axial T2 (B), and axial T1 postcontrast (C) images demonstrate a lobulated well-circumscribed avidly enhancing hemangioma in the fourth ventricle with extension through the foramen of Luschka to the right cerebellopontine angle cistern.

Fig 6.

Concomitant regression of extracranial (right orbital/periorbital soft tissues) and intracranial (right lateral ventricle) components of infantile hemangioma (patient 9). A, Postcontrast axial T1-weighted pretreatment image. B, Postcontrast axial T1-weighted image after 3 months of corticosteroid treatment.

Fig 7.

Regression of the prespinal and extradural extension of hemangioma in response to corticosteroid treatment (patient 14). Paramedian sagittal and coronal T2-weighted pretreatment images (A, B, and D) and midine sagittal and coronal T2-weighted images after 3 months of corticosteroid treatment (C and E).