Sellar Atypical Teratoid Rhabdoid Tumor (Atrt) In An Adult: A Case Report and Review of The Literature

Daniel Barsky*¹, Uri P Hadelsberg², Lior Gonen² and Nevo Margalit²

Received: December 03, 2018;   Published: December 17, 2018

DOI: 10.26717/BJSTR.2018.12.002227

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Abstract

Background: Atypical teratoid rhabdoid tumor (ATRT) is a rare type of central nervous system (CNS) tumor, which is seen mostly in the pediatric population. However, there are a few dozen of cases reported in adults. This entity was first differentiated from a Wilm’s tumor in 1978. Since then much progress has been made in understanding the pathophysiology of this disease, but only slight progression has been done in terms of treatment and prognosis, thus ATRT remains a high mortality-disease with grim prognosis and outcome.

Case Description: 54 year-old female, presented with symptoms concurrent of a pituitary macroadenoma (e.g. temporal field vision loss, hyperprolactinemia) which was found out to be a tumor of ATRT characteristics on pathology.

Conclusion: ATRT is a rare entity, but should be kept in mind since it has a poor prognosis. Moreover, considering the grim prognosis, and the rarity of the disease, once ATRT diagnosis was made, it should be treated by a multidisciplinary team composed with surgeons, oncologists and radiation oncologists.

Keywords :ATRT; Macroadenoma; Trans-sphenoidal surgery

Abbreviations : ATRT: Atypical Teratoid Rhabdoid Tumor; CNS: Central Nervous System; CT: Computed Tomography; MRI: Magnetic Resonance Imaging; AT/RT: Atypical Teratoid/Rhabdoid Tumor: ICU: Intensive care Unit; DI: Diabetes Insipidus; LMD: Leptomeningeal Dissemination; EMA: Epithelial Membrane Antigen; GFAP: Glial Fibrillary Acidic Protein; TSG: Tumor Suppressor Gene