DOI: 10.14260/jemds/2014/2944 CASE REPORT J of Evolution of Med and Dent Sci/ eISSN- 2278-4802, pISSN- 2278-4748/ Vol. 3/ Issue 27/July 07, 2014 Page 7609 PRIMARY CNS MELANOMA IN AN ALBINO: A RARE CASE REPORT Kishore Sanjeev 1 , Bhardwaj Aparna 2 , Gupta Mohit 3 , Seema Acharya 4 , Kudesia Sandip 5 HOW TO CITE THIS ARTICLE: Kishore Sanjeev, Bhardwaj Aparna, Gupta Mohit , Seema Acharya, Kudesia Sandip. “Primary CNS Melanoma in an Albino: A Rare Case Report”. Journal of Evolution of Medical and Dental Sciences 2014; Vol. 3, Issue 27, July 07; Page: 7609-7613, DOI: 10.14260/jemds/2014/2944 ABSTRACT: Primary intracranial melanoma is a rare and uncommon lesion. Association of primary CNS melanoma in an albino has not been reported in literature searched till now. We are presenting a rare case of primary CNS melanoma in a 52years old male with occulocutaneous albinism. The patient presented with repeated episodes of generalized headache, vomiting and ataxia for duration of 5months. MRI examination showed a tumor in the posterior fossa that was diagnosed as Ependymoma radiologically. Surgical treatment with total removal of tumor was done. Intra- operative squash cytology and frozen section followed by histopathology confirmed the diagnosis of Melanoma. A thorough investigation of the patient was performed including chest radiography, ocular examination, ultrasonography of abdomen and barium enema to rule out any other site of primary melanoma in the body. Thus a final diagnosis of primary CNS melanoma was given. KEYWORDS: CNS, Melanoma, Occulocutaneous albinism, Ependymoma. INTRODUCTION: Primary melanocytic tumours of CNS are rare and comprise 1% of all the tumours. [1] An estimated incidence of 0.9 per 10 million of these tumours has been reported from the literature. [2] Their spectrum varies from benign to malignant and includes leptomeningeal melanocytosis, melanocytoma to its overtly malignant counterpart melanoma. [1] The diagnosis of intracranial melanomas is often complicated and a differential diagnosis of other pigmented lesions like pigmented Meningioma, Schwannoma, Medulloblastoma, Choroid plexus papilloma, Astrocytoma and pituitary tumours should be considered. A diagnosis of primary CNS melanoma should be made after extensive exclusion of cutaneous or mucosal / retinal melanomas by clinical and radiological examination. [3] Herein, we present a rare case of Primary CNS melanoma in an albino patient who presented to Neurosurgery OPD with generalized headache. CASE REPORT: A 52years old albino male patient presented to Neurosurgery department with repeated episodes of generalized headaches, vomiting and ataxia for duration of 3-4 months. MRI of the patient was performed and a mass lesion with T2 hypointense hemosiderin rim involving the cerebellum off midline that involved mainly left cerebellar hemisphere and vermis was detected. The mass measured 5.0x4.4x4.5cms. approximately on radiological examination. Based on these findings a diagnosis of Ependymoma was given on radiology. (Fig. 1a) Intraoperative squash cytology and frozen section were performed. The intraoperative smears were highly cellular, composed of discohesive sheets of spindle to medium sized epithelioid like cells. These cells had prominent macronucleoli, intranuclear inclusions of cytoplasm and dense abundant eosinophillic cytoplasm. (Fig. 1b) Intracytoplasmic melanin could be identified easily. On Frozen section, sheets of malignant cells with eccentrically placed nuclei and punched out prominent nucleoli were seen. These cells displayed abundant eosinophillic cytoplasm and presence of fine to coarsely abundant brownish black pigment which bleached with potassium permanganate. (Fig. 1c &