Brain and Spine (Jan 2024)

A rare case of atypical teratoid rhabdoid tumor at the sellar region in an adult: Case report and review of literature

  • Georgios Georgountzos,
  • Ioannis Gkalonakis,
  • Georgios Kyriakopoulos,
  • Cleanthi Doukaki,
  • Dimitra Argyro Vassiliadi,
  • Konstantinos Barkas

Journal volume & issue
Vol. 4
p. 104138

Abstract

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Introduction: Atypical teratoid/rhabdoid tumor (AT/RT) of Central Nervous System (CNS) is a rare malignancy, usually confronted in childhood. Although few cases were reported in adults, it seems that there is a preference in supratentorial areas and specifically at the sellar region in middle-aged females with this subgroup presenting distinct features. Research question: We share a case report of a rare intracranial ATRT in the sellar area with lung metastasis in an adult female. Material/methods: Patient's medical records (laboratory tests, radiology examinations, histopathology report) were reviewed after retrieving an informed consent. A literature search within Pubmed and further “snowballing” was performed with the use of keywords “sellar”, “adult”, “ATRT” to address the current literature. Results: We present the case of a 51-year-old woman with headaches and left ptosis, diagnosed with a sellar mass, infiltrating the cavernous sinus. She underwent endoscopic transsphenoidal debulking of the lesion. The pathology report showed an aggressive AT/RT and the patient received radio- and chemo-therapy. On follow up imaging studies, lung metastases were shown and the patient died 7 months after the initial diagnosis. Discussion & conclusion: Only a few cases of sellar/suprasellar ATRT with lung metastases have been described so far. ATRT should be in the differential diagnosis of fibrous sellar masses in adult women.

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