Elsevier

World Neurosurgery

Volume 104, August 2017, Pages 1044.e1-1044.e3
World Neurosurgery

Clinical Images
Status Epilepticus Secondary to Pseudonodular Hemorragic Occipital Lesion with Edema: “Non Semper Ea Sunt, Quae Videntur, Decipit Frons Prima Multos” (Things Are Not Always What They Seem; The First Appearance Deceives Many)

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We report a case in which common radiologic images masked a rare case of supratentorial hemangioblastoma (HBL). Other peculiarities of this case are the clinical presentation with status epilepticus and the occurrence of a supratentorial HBL unrelated to von Hippel–Lindau syndrome. Based on clinical and radiologic findings, including massive cerebral edema and hemorrhagic presentation, our preoperative diagnosis was a cerebral metastasis. In this scenario, physicians must take into account the words of the Roman fabulist Phaedrus: “Non semper ea sunt, quae videntur, decipit frons prima multos” (things are not always what they seem; the first appearance deceives many).

Introduction

A 61-year-old man was admitted to our clinic for continuous tonic-clonic seizures. A computed tomography (CT) scan showed a hyperdense hemorrhagic lesion located in the left occipital lobe with significant cerebral edema and homogeneous contrast enhancement (Figure 1A and B). Fluid-attenuated inversion recovery magnetic resonance imaging (MRI) revealed an intra-axial pseudonodular lesion in the left occipital lobe surrounded by a hypointense rim of hemosiderin and edema (Figure 1C). T1-weighted post-gadolinium coronal MRI confirmed homogeneous contrast enhancement (Figure 1D). A sagittal susceptibility-weighted MRI sequence revealed a draining hypertrophied vein (Figure 1E). Spinal MRI demonstrated no other lesions, and ophthalmologic evaluation revealed homonymous hemianopsia.

Based on clinical and radiologic findings, the most likely diagnosis was cerebral metastasis, even though a total body CT scan showed no primary tumors. Another possible diagnosis considered was cavernous angioma.

A left occipital craniotomy was performed to resect the mass with the aid of a neuronavigation system. During surgery, a well-circumscribed lesion with abnormal vascular structures both inside and around was found (Figure 2A). An arterialized draining vein was localized on the superior pole of the lesion (Figure 2A). After disconnection of vascular feeders (Figure 2B), a gross total resection was achieved (Figure 2C). Interestingly, after resection of the hemangioblastoma (HBL), the cortical draining vein was no longer arterialized (Figure 2D).

The main steps of surgery are illustrated in Video 1. The postoperative course was uneventful, and a CT scan confirmed the total resection of the mass (Figure 2E). Histopathological examination of the surgical specimen demonstrated characteristic features of HBL (World Health Organization grade I). Further radiologic investigations and genetic testing excluded von Hippel–Lindau syndrome (VHL). Follow-up at 3 months after surgery revealed marked improvement in the patient's visual field.

HBLs of the CNS are rare vascularized neoplasms often associated with VHL syndrome.1, 2, 3 A supratentorial location is particularly uncommon.3 To the best of our knowledge, status epilepticus secondary to HBL has not been reported previously. Other peculiarities of this case included the neoplasm's supratentorial location, the presence of massive peritumoral edema, and the hemorrhagic presentation, which are not typically characteristic of HBLs.1, 2, 3

This report also demonstrates our mistaken initial diagnosis of a supretantorial HBL, which we misinterpreted as a cerebral metastasis or cavernous angioma.4 In this scenario, physicians must take into account the words of the Roman fabulist Phaedrus: “Non semper ea sunt, quae videntur, decipit frons prima multos” (things are not always what they seem; the first appearance deceives many).

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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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