Atypical teratoid/rhabdoid tumor presenting with subarachnoid and intraventricular hemorrhage

Abstract

Background: Sellar masses comprise 14–18% of all intracranial tumors. Pituitary adenomas account for 85% of these lesions, while 15% of sellar masses stem from other etiologies. Intratumoral hemorrhage (apoplexy), while not exceptionally common, can be discovered at presentation. While the hemorrhage pattern is typically contained within the tumor, an extension of bleeding beyond the sella has been reported. Case Description: A 55-year-old female presented with an anterior interhemispheric subarachnoid hemorrhage and extensive intraventricular hemorrhage (IVH). Initially, the IVH was thought to be due to a ruptured aneurysm. After further workup, a hemorrhagic sellar mass was diagnosed. The patient underwent transsphenoidal resection of a pituitary adenoma. The patient returned to the emergency department6 weeks later with an atypical rapidly expanding sellar mass. After tumor debulking, the pathology revealed an atypical teratoid/rhabdoid tumor (ATRT). Here, we reviewed this and other such cases of sellar ATRT. Conclusion: The early diagnosis of sellar ATRT with lack of integrase interactor 1 expression and elevated Ki67 proliferation indices can prompt more aggressive attempted gross total resection, chemotherapy, and radiation treatments.

Purely suprasellar (Hypothalamic) atypical teratoid rhabdoid tumor presenting with diabetes insipidus and panhypopituitarism in an adult male: A case report and review of literature

“…All cases reported in sellar/suprasellar region presented mainly with headache and diplopia. [2,[4][5][6][7][8][9][10][11][12][13][14][15][16] Two of the reported cases presented with subarachnoid hemorrhage associated with intraventricular extension. [2,11] As our case is purely suprasellar (hypothalamic) with compression of pituitary stalk, so presentation was a little bit different as diabetes insipidus and panhypopituitarism.…”

“…[2] Prognosis is poor, and majority of sellar/suprasellar cases die within 2 years of diagnosis. [2] There is no previous case as purely suprasellar (hypothalamic). Thus, we have no data about the prognosis of purely suprasellar lesions.…”

“…To our knowledge, all reported cases in sellar/suprasellar regions occurred in females and all of them have sellar component with suprasellar extension. [2,[4][5][6][7][8][9][10][11][12][13][14][15][16] Our case is the first reported case to occur in middle-aged males with purely suprasellar location without any sellar components. Intraoperative lesion appeared to be arising Clinical presentation varies based on patient age, location, and tumor size.…”

“…The cases reported in sellar/ suprasellar region have a sellar component with suprasellar extension. [2] According to the World Health Organization classification of central nervous system tumors 2016, ATRT is Grade 4. Diagnosis is mainly pathological through immunohistochemistry either by alterations or loss of integrase interactor 1 (INI1) protein or BRG1 protein.…”

Unusually long survival of an adult patient with atypical teratoid/rhabdoid tumor of the sellar region: A follow-up report

“…Its occurrence in the sellar region is particularly rare, with only 31 cases reported in the literature to date [Table 1]. [5,[7][8][9][10] Sellar AT/RTs have a female predominance, although male predominance with a reported ratio of 3:2-2:1 is notable for AT/RT outside the sellar region. [11] In most adult AT/RT cases, there are no consistent treatment protocols, and any decisions on treatment are extrapolated from the pediatric literature.…”