A rare aggressive tumor of the sellar region: atypical teratoid/rhabdoid tumor
B. Erturk*a (Dr), I. Isikaya (Dr), E. Gumelera (Dr), SN. Sendura (Dr), F. Soylemezoglua (Dr), T. Erbasa (Dr)
a Hacettepe University, Ankara, TURKEY
Introduction: Atypical teratoid rhabdoid tumor (AT/RT) is a rare, highly aggressive tumor of the sellar region. AT/RT is generally seen in children however, it has been reported in increasing numbers in adults. In this article, we report a metastatic AT/RT case in an adult who displayed an aggressive clinical course and complicated with pituitary apoplexy.
Observations: A fifty-five-year-old woman was admitted to the hospital for headache, polyuria and polydipsia that started about one month ago. The patient also had a complaint of visual impairment in the left eye. Brain MRI identified a 12x20 mm pituitary mass. Within weeks, the patient complained of worsening headache and decreased vision. On physical examination, visual acuity was decreased in both eyes with limited temporal vision. Follow-up pituitary MRI demonstrated a multilobular sellar tumor with 35x28 mm diameter that fills the sellar region and extends to the suprasellar location. The tumor displayed indentation to the floor of the third ventricle without infiltration of the cavernous sinuses. On hormonal evaluation the patient was demonstrated to have panhypopituitarism and hypernatremia secondary to diabetes insipidus. The patient was commenced on prednisone 7.5 mg/day and desmopressin 60 µg/day. On day three after hospital admission, the patient’s headache intensified and a control pituitary MRI revealed haemorrhage in tumor. Transsphenoidal surgery was performed to excise sellar tumor. After complete resection of the tumor, bleeding occurred in the pons and mesencephalon. Pathologic examinations showed an atypical teratoid rhabdoid tumor. Spinal and cranial MRI examinations were performed to detect putative metastasis and there were multiple metastatic lesions on the thoracic vertebras. Radiotherapy was applied to the patient. The patient is still being followed in the intensive care unit.
Discussion: AT/RT is a tumor of the central nervous system which is rare in adults and in sellar region. AT/RTs are highly aggressive tumors and require early intervention. Imaging methods may not be distinctive in the diagnosis of the disease, therefore, these tumors should be suspected in the rapid clinical course and should be treated multimodally.
The author has declared no conflict of interest.