Atypical Teratoid/Rhabdoid Tumor of the Sellar Region in an
Introduction:
Atypical teratoid/rhabdoid tumor (AT/RT) is a very aggressive central nervous system (CNS) neoplasm that is most often seen in infants and young children [1, 2]. About half of AT/RTs are found in the posterior fossa but can also occur anywhere in the brain or spinal cord. It was originally described a histological variant of Wilms’ tumor in 1978 [3]. Primary intracranial diseases were initially reported in 1987 and subsequently, defined as a distinct CNS neoplasm in 1996 and added to the World Health Organization (WHO) Brain Tumor Classification in 2000 (grade IV) [1, 4, 5].
Prognosis for AT/RT is poor, with a median survival time of less than 1 year [1-3]. The tumor is characterized by loss of function mutations in INI-1/hSNF5 gene located on chromosome 22q11.2 [6]. The clinical presentation varies with tumor location. Typically, patient with AT/RT is treated with surgery and craniospinal radiation therapy which is often followed by systemic chemotherapy. Here we report a case of AT/RT that originated in sellar and suprasellar region in a 36-year-old female patient with brief review of the survival rate in this rare aggressive tumor in adult population.

Case Report:
A 36-year-old female not known to have any medical illnesses, mother of five children presented to the emergency department on October 2013 with a history of severe headache associated with double vision and vomiting.
The headache was progressive over 3 months, then became severe and associated with vomiting and double vision 1 month prior to presentation. She denied galactorrhea, but her menstruation had ceased 2 months earlier. There was no weakness or convulsion. She was on no medication, and had no significant past medical history and family history was unremarkable. Her neurological examination showed no neurological deficits apart from bilateral sixth nerve palsies with pale optic disc. The rest of examination was unremarkable....

http://www.jocmr.org/index.php/JOCMR/article/view/2922/1729
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