ED-26 * ATYPICAL TERATOID/RHABDOID TUMOR OF THE CENTRAL NERVOUS SYSTEM IN A 27-YEARS OLD PATIENT

Abstract

INTRODUCTION: Atypical Teratoid/Rhabdoid Tumor (ATRT), according to the World Health Organization (WHO) Classification of Tumors, is a highly malignant neoplasm (grade IV) of the Central Nervous System (CNS) that preferentially manifests in children less than three years of age. These tumors are mainly composed of rhabdoid cells, with the addition or not of areas demonstrating characteristics of primitive neuroectodermal tumor. Less than 40 cases were published in the literature about this neoplasm. OBJECTIVE: In this context our aim is to present an unusual case of ATRT. MATERIAL AND METHOD: A 27-year old male, with intellectual disability. He referred headache, nausea and vomiting and was associated with progressive left femoral brachial paresis, so he consulted in the clinic in March 2013. Brain CT and magnetic resonance (MRI) spectroscopy revealed a solid cystic mass in the right temporal medial lobe of 65x57 mm, with an intense contrast (gadolinium) enhancement, the tumor compressed the third ventricles, displacing the midline and deforming the ventricles. RESULTS: The neoplasm was totally excised, histological and immunohistochemical analysis demonstrated atypical rhabdoid cells strongly and diffusely positive for EMA and Vimentin as well as focally immunoreactive for GFAP. The patient was treated with chemotherapy (vincristine) and radiotherapy. The patient finally died in Abril 2014. CONCLUSIONS: Few previous case of ATRT have been reported in adults, thus far. This unusual presentation underlines the necessity of considering this devastating neoplasm in the differential diagnosis of malignant brain tumor of young adults.