Infantile hemangioendothelioma of the pericranium presenting as an occipital mass lesion

Case report

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✓ The case of a newborn infant with a large midline mass in the occipital region is presented. Skull x-ray films demonstrated multiple radiolucent defects in the occipital bone. A computerized tomography scan revealed an extracranial mass lesion with marked contrast enhancement. A magnetic resonance image demonstrated that the venous drainage of the lesion passed through the occipital bone into the dural venous sinuses. The anatomy of the hindbrain was normal. The neonate was treated by complete surgical removal of the mass. The histopathological diagnosis was infantile hemangioendothelioma, a tumor commonly found in the liver but rarely in this location. The classification, histopathological characteristics, imaging studies, and treatment of this tumor are discussed.

Article Information

Address reprint requests to: James T. Rutka, M.D., Ph.D., Division of Neurosurgery, Suite 1502, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8 Canada. email: rutka@sickkids.on.ca.

© AANS, except where prohibited by US copyright law.

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Figures

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    Posterior (A) and lateral (B) preoperative photographs of the neonate's head, showing a midline occipital lesion.

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    Plain skull x-ray film demonstrating radiolucencies (arrow) in the region of the torcular herophili.

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    A: Unenhanced axial CT scan revealing a well-defined extracranial lesion in the occipital region without involvement of intracranial structures. B: Contrast-enhanced axial CT scan demonstrating marked enhancement of the lesion.

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    A: Unenhanced sagittal T1-weighted MR image revealing a well-defined occipital mass and normal intracranial anatomy except dilation of the torcular herophili. B: Sagittal T2-weighted MR image demonstrating flow voids within the tumor vasculature (straight arrow) in communication with large dural venous sinuses located intracranially (curved arrow). C: Axial T1-weighted MR image demonstrating the extensive nature of the lesion. Note the vascular pedicle located laterally (arrow).

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    Magnetic resonance venography with two-dimensional time-of-flight reconstruction revealing the tumor's veins (curved arrow) draining into the tranverse sinus (straight arrow).

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    Preoperative photograph showing the patient in the prone position on the operating room table, following preparation of the skin.

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    Photomicrographs of the lesion. H & E, original magnification × 500. Upper: Photomicrograph showing a vascular tumor with multiple vascular channels of variable sizes. At this medium-power magnification, vascular spaces surrounded by nests of endothelial cells are seen. Occasional mitoses are evident in this cellular component of the lesion. Lower: Immunostaining for U. europeus lectin reveals positivity in endothelial cells and highlights the vascular nature of this soft-tissue lesion.

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