Pleomorphic xanthoastrocytoma of the pineal region B.H. Srinivas a , Megha S. Uppin a , Manas K. Panigrahi b , M. Vijaya Saradhi a , Y. Jyotsna Rani c , Sundaram Challa a, * a Department of Pathology, Nizam’s Institute of Medical Sciences, Punjagutta, Hyderabad 500082, Andhra Pradesh, India b Department of Neurosurgery, Nizam’s Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India c Department of Radiology, Nizam’s Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India article info Article history: Received 7 January 2010 Accepted 4 February 2010 Keywords: Pleomorphic xanthoastrocytoma Pineal region Immunohistochemistry abstract Pleomorphic xanthoastrocytomas are indolent, astrocytic tumors usually located in the superficial cere- bral cortex. They have not been described as occurring in the pineal region. We describe a patient with an astrocytic tumor arising in the pineal region that fulfilled all of the morphologic and immunohistochem- ical criteria of a pleomorphic xanthoastrocytoma. To our knowledge, this is the first description of a pleo- morphic xanthoastrocytoma located in the pineal region. Ó 2010 Elsevier Ltd. All rights reserved. 1. Introduction Tumors of the pineal region account for 0.4% to 1.0% of intracra- nial tumors in adults and 3.0% to 8.0% of brain tumors in children. They have a varied histology and are divided into germ cell and non–germ cell derivatives. 1 Most tumors are the result of displaced embryonic tissue, malignant transformation of the pineal paren- chymal cells or transformation of the surrounding astroglia. 2 The occasional gliomas seen in the pineal region are usually pilocytic astrocytomas or glioblastoma multiformes, which are thought to arise from resident pineal astroglial cells. 3,4 To the best of our knowledge, primary pleomorphic xanthoastrocytoma of the pineal region has not been described in the literature. We describe one such instance that showed the characteristic histological and immunohistochemical features of pleomorphic xanthoastrocytoma. 2. Case report A 30-year-old man presented to our facility with gradual onset, progressive bifrontal headache for a period of 1 month. He was not hypertensive or diabetic. There was no history of vomiting, seizure or weakness. On examination, the higher motor functions and cranial nerves were normal. The gross motor and sensory system examination was also normal. Fundus examination revealed gross papilledema. A CT scan of the brain showed an isodense, well- circumscribed tumor in the posterior third ventricle, resulting in hydrocephalus (Fig. 1A). The tumor enhanced with contrast administration and showed a speck of calcification. There was associated hydrocephalus. MRI revealed isointensity on T1- weighted and hyperintensity on T2-weighted MRI, with contrast enhancement suggestive of pineocytoma (Fig. 1B). Initially, a ventriculoperitoneal shunt was inserted, followed by a transtentorial occipital craniotomy for total excision of the tumor. Operatively, the tumor was found to be grayish white in color, moderately firm, well defined and not able to be removed by suction. The tumor was entirely excised. Post-operatively, the pa- tient developed an upward gaze palsy without other focal deficits. 2.1. Neuropathological findings The tumor sections showed a cellular lesion comprised of mono- and multinucleated giant astrocytes with a high degree of pleomorphism. There were many pleomorphic giant cells arranged on a fibrillary background. The nuclei of these cells were bizarre, with prominent intranuclear inclusions. The cytoplasm was abun- dant and focally granular. Atypical mitotic figures or areas of necrosis were absent. The background showed numerous eosino- philic granular bodies (EGB; Fig. 2A,B). The intratumoral vessels showed perivascular lymphocytes and increased reticular deposi- tion around the vessels. Interspersed between these pleomorphic cells, there were large xanthomatous cells with a multivacuolated appearance of the cytoplasm, pushing the nucleus to the periphery (Fig. 2C). Immunohistochemistry with glial fibrillary acidic protein (GFAP) and S-100 showed intense cytoplasmic positivity in the pleomorphic cells (Supplementary Fig. 1), whereas CD34 and synaptophysin immunohistochemistry was negative. The Ki-67% index of the tumor was less than 1%. Ultrastructural findings were not available. 2.2. Follow up The patient was alive and well 1 year after the primary operation. 3. Discussion First described by Kepes et al., pleomorphic xanthoastrocytoma is an uncommon brain tumor usually observed in the cerebral hemi- sphere of young adults; it is often associated with cyst formation. 5,6 As per the World Health Organization (WHO) guidelines, the mor- phological and immunohistochemical features of our patient’s tumor were characteristic of a pleomorphic xanthoastrocytoma (WHO grade II). 1 The main differential diagnostic consideration in this instance is of glioblastoma multiforme (GBM), owing to the high cellular pleomorphism. Moreover, GBM can occur in the pineal re- gion, albeit rarely. However, the absence of mitoses and microvascu- lar proliferation, the presence of EGB and the low proliferation index do not support the diagnosis of GBM. 7 Another important consideration is that of pineal parenchymal tumors with astrocytic differentiation. These are known to occur in * Corresponding author. Tel.: +91 40 2348 9051; fax: +91 40 2331 0076. E-mail address: challa_sundaram@yahoo.com (S. Challa). Case Reports / Journal of Clinical Neuroscience 17 (2010) 1439–1441 1439