Accepted Manuscript 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) Davide Nasi, M.D., Franco Servadei, M.D., Antonio Romano, M.D PII: S1878-8750(17)30813-6 DOI: 10.1016/j.wneu.2017.05.114 Reference: WNEU 5808 To appear in: World Neurosurgery Received Date: 26 March 2017 Revised Date: 15 May 2017 Accepted Date: 18 May 2017 Please cite this article as: Nasi D, Servadei F, Romano A, 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), World Neurosurgery (2017), doi: 10.1016/j.wneu.2017.05.114. This is a PDF file of an unedited manuscript that has been accepted for publication. 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ACCEPTED MANUSCRIPT Status epilepticus secondary to pseudonodular hemorragic occipital lesion with edema: “Non semper ea sunt, quae videntur, decipit frons prima multos” RI PT (things are not always what they seem; the first appearance deceives many) SC Davide Nasi M.D. 1, Franco Servadei M.D. 2, Antonio Romano M.D 2 M AN U 1 Department of Neurosurgery of Institute for Scientific and Care Research "ASMN" of Reggio Emilia, Reggio Emilia, Italy 2 Neurosurgery-Neurotraumatology Unit of University Hospital of Parma, Parma and Department of Neurosurgery of Institute for Scientific and Care Research "ASMN" of Reggio Emilia, Reggio Corresponding author: EP Davide Nasi, M.D. TE D Emilia, Italy AC C Department of Neurosurgery of Institute for Scientific and Care Research "ASMN" of Reggio Emilia, Reggio Emilia, Italy Viale Risorgimento 80, 42121, Reggio Emilia, Italy Telephone: +39 0522296070; Mobile: +39 3483887309; Fax: +39 522296070 Email: davidenasi83@gmail.com ACCEPTED MANUSCRIPT Clinical Image Conflict of interest: None. RI PT Abstract We report a common radiological images that hide an infrequent case of a supratentorial hemangioblastoma (HBL). Others peculiarities of this case are the clinical presentation with a SC status epilepticus and the occurrence of a supratentorial HBL unrelated with Von Hippel Linedeau Syndrome (VHL). Based on clinical and radiological findings including massive cerebral edema and M AN U hemorrhagic presentation, our preoperative diagnosis was a cerebral metastasis. In this scenario, physicians must be taken into account the words of Phaedrus, a Roman fabulis: “Non semper ea sunt, quae videntur, decipit frons prima multos” (things are not always what they Clinical Image TE D seem; the first appearance deceives many). EP 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 AC C with large cerebral edema and homogeneous contrast enhancement (Fig. 1ab). The MRI FLAIRweighted images showed intra-axial pseudo nodular lesion in left occipital lobe surrounded by a hypointense rim of hemosiderin and with edema (Fig 1c). T1-weighted post-gadolinium coronal image confirmed homogeneous contrast ehnacement (Fig 1d). MRI sagittal susceptibility weighted imaging (SWI) sequence revealed a draining hypertrophied vein (Fig. 1e). Spinal MRI showed no others lesions and ophthalmologic evaluation revealed homonymous hemianopsia vision loss. Based on clinical and radiological findings, the most likely diagnosis was cerebral metastasis, despite total-body CT scan was unsuccessful in detecting primary tumors. Other possible diagnosis ACCEPTED MANUSCRIPT was cavernous angioma. A left occipital craniotomy was performed to resect the mass with the aid of neuronavigation system. During surgery, a well-circumscribed lesion with abnormal vascular structures both inside and around was found (Fig 2a). An arterialized draining vein was localized on the superior pole of the lesion (Fig. 2a). After disconnection of vascular feeders (Fig. 2b), a cortical draining vein was no longer arterialized (Fig. 2d). RI PT gross total resection was achieved (Fig. 2c). Interesting, after resection of hemangioblatoma, the The main surgical aspects were illustrated in Video 1. Post-operative course was uneventful and CT SC scan confirmed the total resection of the mass (Fig 2e). Histopathological examination of the M AN U surgical specimen demonstrated characteristic features of hemangioblastoma (WHO grade I) [HBL]. Further radiological investigations and genetic test excluded Von Hippel Linedeau Syndrome (VHL). Patient’s follow-up revealed marked improvement in his visual field 3 months after surgery. TE D Hemangioblastomas (HBL) of the CNS are rare vascularized neoplasms often associated with VHL syndrome [1-3]. Supratentorial location is particularly uncommon [3]. To our best knowledge, status epilepticus secondary to a HBL has not been previously reported in EP the literature. Other peculiarities were supratentorial location, the presence of massive AC C peritumoral edema and hemorrhagic presentation which were not characteristic of HBL [1-3]. In conclusion, this report portrays also our pitfall in the initial diagnosis of a supretantorial HBL which we misinterpreted as a cerebral metastasis or cavernous angioma [4]. In this scenario, physicians must be taken into account the words of Phaedrus, a Roman fabulis: “Non semper ea sunt, quae videntur, decipit frons prima multos” (things are not always what they seem; the first appearance deceives many). Compliance with ethical standards ACCEPTED MANUSCRIPT Funding: No funding was received for this research. Conflict of interest: None. Informed consent: Informed consent was obtained from all individual participants included in the RI PT study. References SC 1. Mills SA, Oh MC, Rutkowski MJ, Sughrue ME, Barani IJ, Parsa AT. Supratentorial M AN U hemangioblastoma: clinical features, prognosis, and predictive value of location for von Hippel-Lindau disease. Neuro Oncol. 2012 Aug;14(8):1097-104. 2. Pandey S, Sharma V, Pandey D, Kumar V, Kumar M. Supratentorial haemangioblastoma without von Hippel-Lindau syndrome in an adult: A rare tumor with review of literature. TE D Asian J Neurosurg. 2016 Jan-Mar;11(1):8-14. 3. Lonser RR, Butman JA, Huntoon K, Asthagiri AR, Wu T, Bakhtian KD, Chew EY, Zhuang Z, Linehan WM, Oldfield EH. Prospective natural history study of central nervous system EP hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 2014 May;120(5):1055-62 AC C 4. Nasi D, Somma Ld, Iacoangeli M, Liverotti V, Zizzi A, Dobran M, Gladi M, Scerrati M. Calvarial bone cavernous hemangioma with intradural invasion: An unusual aggressive course-Case report and literature review. Int J Surg Case Rep. 2016;22:79-82. RI PT ACCEPTED MANUSCRIPT Figure Legend SC Fig. 1 Preoperative neuroimaging studies. M AN U a,b. A precontrast computed tomography (CT) scan showed a hyperdense hemorrhagic lesion located in the left occipital lobe with large cerebral edema; after administration of contrast medium, the lesion showed a omogeneous contrast enhancement c. The MRI FLAIR-weighted images showed intra-axial pseudo nodular lesion in left occipital TE D lobe with edema surrounded by a hypointense rim of hemosiderin d. T1-weighted post-gadolinium coronal image demonstrated omegeneous contrast ehnacement EP e. The signal void of hypertrophied vessel was shown in the super aspect of tumor. MRI AC C sagittal susceptibility weighted imaging (SWI) sequence confirmed a drainage hypertrophied vein Fig. 2 Intraoperative images and post-operative CT scan a. After a left occipital craniotomy and dura opening, a well-circumscribed lesion with abnormal vascular structures both inside and around was found; an arterialized draining vein was localized on the superior pole of the lesion (black arrow) b. Disconnection of vascular feeders of hemangioblastoma (black arrow) c. A gross total resection was achieved at the end of surgery ACCEPTED MANUSCRIPT d. After disconnection of fistulous feeder of hemangioblatoma, the cortical draining vein was no longer arterialized (black arrow) AC C EP TE D M AN U SC RI PT e. A post-operative CT scan confirmed the total resection of the mass AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT ACCEPTED MANUSCRIPT Highlights 1. Hemangioblastomas (HBL) are very rare lesions 2. Supratentorial location is uncommon and almost always associated with Von Hippel Lindeau Syndrome (VHL) RI PT 3. In supratentorial HBL, status epilepticus and hemorrhagic presentation were not yet reported. 4. We report a common hemorrhagic lesion with edema that hide an infrequent case of a AC C EP TE D M AN U SC supratentorial HBL ACCEPTED MANUSCRIPT Abbreviations list: HBL. Hemangioblastoma CT. Computed tomographic scan MRI. Magnetic resonance imaging AC C EP TE D M AN U SC DMARDS. New disease modifying antirheumatic drugs RI PT VHL. Von Hippel Linedeau Syndrome