Sellar Region Atypical Teratoid/Rhabdoid Tumors in Adults: Clinicopathological Characterization of Five Cases and Review of the Literature

Michael A. Paolini, Benjamin R. Kipp, William R. Sukov, Sarah M. Jenkins, Emily G. Barr Fritcher, Derick Aranda, Karen S. SantaCruz, Sadeq Al-Dandan, Pete Fisher, William C. McDonald, Charles P. Bondurant, Grant Van Dyke Darkow, Caterina Giannini, Joseph E Parisi, Mark E. Jentoft, Aditya Raghunathan

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Abstract

Atypical teratoid/rhabdoid tumors (AT/RTs) are highly malignant CNS neoplasms that typically occur in children <2 years of age. These are characterized by high-grade histologic features and mutations of the INI1/SMARCB1 gene readily detected by loss of expression by immunohistochemistry. Among adults, the majority of AT/RTs occurs in the cerebral hemispheres. A small number of adult AT/RTs involving the sellar and suprasellar region reported in the literature suggest a distinct clinical course for this group. Here, we describe detailed clinical and genetic characterization of 5 adult patients with AT/RTs involving the sellar and suprasellar region, and provide a review of the available clinical and genetic features of 22 previously reported cases in order to help increase our understanding of this unusual entity.

Original languageEnglish (US)
Pages (from-to)1115-1121
Number of pages7
JournalJournal of Neuropathology and Experimental Neurology
Volume77
Issue number12
DOIs
StatePublished - Dec 1 2018

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ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Cite this

Paolini, M. A., Kipp, B. R., Sukov, W. R., Jenkins, S. M., Barr Fritcher, E. G., Aranda, D., SantaCruz, K. S., Al-Dandan, S., Fisher, P., McDonald, W. C., Bondurant, C. P., Van Dyke Darkow, G., Giannini, C., Parisi, J. E., Jentoft, M. E., & Raghunathan, A. (2018). Sellar Region Atypical Teratoid/Rhabdoid Tumors in Adults: Clinicopathological Characterization of Five Cases and Review of the Literature. Journal of Neuropathology and Experimental Neurology, 77(12), 1115-1121. https://doi.org/10.1093/jnen/nly091