6791 Pituitary Apoplexy as Presentation of Atypical Teratoid/Rhabdoid Tumor

Abstract

Abstract Disclosure: S.I. Babli: None. H. Alkubedan: None. N.M. Ali: None. R. Alqawahmed: None. M.M. Elhassan: None. Introduction: Pituitary apoplexy occurs in a pituitary adenoma; it migh occur in other less common types of tumors. The atypical teratoid/rhabdoid tumor (ATRT) is a very aggressive malignant tumor of the central nervous system and has a poor prognosis. It is rare to occur in adult and in the sellar region. Clinical Case: 32 years old male presented to ER with 4 days history of fever, headache and vomiting. He was conscious but confused. Vital signs Temperature 39 C, BP 120/80, HR 110 beats/min and Saturation of 95 %, Possibility of meningoencephalitis was raised, patient Brain CT showed Sellar and suprasellar mass with compression over the optic chiasma and invasion to the left cavernous sinus. Patient suddenly developed Generalize tonic-clonic convulsion and hypotension. He received IV midazolam, intubated and was started on noradrenaline and hydrocortisone, he was admitted to ICU as a case of meningoencephalitis and started on Ceftriaxone, Vancomycin and Acyclovir. Cerebrospinal fluid analysis showed RBC 175/cumm, WBC 20/cumm with 20% polymorph and 80% mononuclear cells, Protein 1.38 g/l (0.24-0.49) glucose 7.9 mmol/l with plasma glucose of 10 mmol/l. Repeated Brain CT showed Sellar and suprasellar mass with compression over the optic chiasma and invasion to the left cavernous sinus and with minimal heterogenicity and increased density suggestive of hemorrhagic components. Endocrine history revealed weight gain but no history to suggest acromegaly, Cushing’s syndrome or hypogonadism . Examinations: Patient was intubated and sedated BP 90/60 on Maximum inotropic support. Left oculomotor nerve palsy, Bilateral papilledema, he was having acanthosis nigricans with no signs of acromegaly, CS or hypogonadism. Laboratory finding: WBC17 with neutrophilia, Hgb 14.7 g/dl plt 360 Na 140 mmol/l, creatnin 180 mmol/, FT4 6.2 pmol/l, TSH 0.015, PROLACTINE 22, IGF1 29 ng/ml (41-246) Free testosterone index 2.13 (33-106), LH 0.5, FSH 0.3 .Patient had TTS and histopathology showed: SMARCB1 deficient malignant neoplasm, consistent with sellar atypical teratoid rhabdoid tumor. Post OP, patient had Ventilator associated pneumonia and recurrent attacks of seizures despite multiple antiepileptic medications. Patient was evaluated by medical and radiation oncologists but due to his general condition the treatment was postpone. One month after TSS, he developed intractable seizure new Brain CT showed interventricular haemorrhage with hydrocephalus and marked enlargement of hyperdense pituitary lesion which suggests haemorrhage. External ventricular drain was inserted but patient arrested and died. Conclusions: ATRT of the CNS usually affects young children. In the literature review performed in adults, only nine cases have occurred in the sellar or suprasellar region. Survival is variable in the different cases reported in the literature, with a mean of 26 months. Presentation: 6/2/2024