INTRAPARENCHYMAL MYXOPAPILLARY EPENDYMOMA: CASE REPORT Nikolaos Tzerakis, M.D. Department of Neurosurgery, G. Gennimatas General Hospital, Athens, Greece Nikolaos Georgakoulias, M.D., Ph.D. Department of Neurosurgery, G. Gennimatas General Hospital, Athens, Greece George Kontogeorgos, M.D., Ph.D. Department of Pathology, G. Gennimatas General Hospital, Athens, Greece Aristotelis Mitsos, M.D. Department of Neurosurgery, G. Gennimatas General Hospital, Athens, Greece, and Newcastle General Hospital, Newcastle upon Tyne, England Alistair Jenkins, F.R.C.S. Newcastle General Hospital, Newcastle upon Tyne, England George Orphanidis, M.D. Department of Neurosurgery, G. Gennimatas General Hospital, Athens, Greece Reprint requests: Nikolaos Tzerakis, M.D., Amfikleias 18-20, Poligono 11476, Athens, Greece. Email: ntzerakis@hotmail.com. Received, August 12, 2003. Accepted, March 4, 2004. OBJECTIVE AND IMPORTANCE: Myxopapillary ependymoma is a histological vari- ant of ependymoma found in the cauda equina region. The most characteristic histological feature of myxopapillary tumors is the abundance of intercellular and perivascular mucin and the arborizing vasculature, which tends to form papillae. Primary intracerebral myxopapillary ependymomas are extremely rare; only three cases have been reported in the previous literature. CLINICAL PRESENTATION: A 68-year-old man presented with disorientation and dizziness caused by a cystic left frontal intraparenchymal lesion. This proved to be a myxopapillary ependymoma. Similarities to previously reported cases are discussed, as are the findings on magnetic resonance imaging. There is also a literature review of the histological findings, natural history, and outcome of surgically treated myxopap- illary ependymoma. INTERVENTION: The lesion was totally removed. After surgery, the patient was neurologically intact and had an uneventful recovery. CONCLUSION: This is the fourth reported case of histologically proven primary myxopapillary intracranial ependymoma. KEY WORDS: Ependymoma, Intracranial, Myxopapillary Neurosurgery 55:E996-E1000, 2004 DOI: 10.1227/01.NEU.0000137278.84588.06 www.neurosurgery-online.com CASE REPORT Clinical History A 68-year-old man presented in the emer- gency room with a history of dizziness, memory disturbance, and confusion- disorientation in time and place for 10 days. He had never experienced similar episodes in the past, and there were no other symptoms. The patient was a nonsmoker, and there was a previous medical history of megaloblastic anemia treated with folic acid and upper gas- trointestinal bleeding 2 years previously after nimesulide intake. There was no history of allergies, drug uptake, or severe heart or pul- monary disease. There was no significant fam- ily history. The physical examination revealed no pathological signs. There was no cranial nerve palsy, hemiparesis, or other neurological signs. Tumor marker results were negative. Other laboratory findings were within normal limits. The computed tomographic scan revealed a large intra-axial cystic mass in the left frontal lobe producing mass effect on the ipsilateral lateral ventricle and midline. The cyst was well demarcated, with a solid component in its medial wall that strongly enhanced with intravenous contrast. Because the first diagno- sis was “glioma,” the patient was treated at first with anticonvulsants, mannitol, and dexamethasone. The patient improved with this treatment and underwent a magnetic resonance imaging (MRI) scan that showed intense contrast en- hancement of the solid part of the tumor and the cyst wall (Fig. 1). The left frontal horn was compressed, but there was no subependymal infiltration. Perifocal edema extended to the frontal and parietal lobes, and there was sub- falcine herniation. A few days after the MRI scan, the patient underwent a left frontal craniotomy and gross total removal of the tumor and the specimen was sent for pathological evaluation. After surgery, the patient was neurologically intact and had an uneventful recovery. He was dis- charged in the 10th postoperative day. Neuropathological Findings Histological examination showed a cellular tumor consisting of medium-sized neoplastic cells arranged around stromal vessels and E996 | VOLUME 55 | NUMBER 4 | OCTOBER 2004 www.neurosurgery-online.com