Sellar Region Atypical Teratoid/Rhabdoid Tumors in Adults: Clinicopathological Characterization of Five Cases and Review of the Literature. Academic Article uri icon

abstract

  • Atypical teratoid/rhabdoid tumors (AT/RTs) are highly malignant CNS neoplasms that typically occur in children <2years 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.

published proceedings

  • J Neuropathol Exp Neurol

altmetric score

  • 1

author list (cited authors)

  • Paolini, M. A., Kipp, B. R., Sukov, W. R., Jenkins, S. M., Barr Fritcher, E. G., Aranda, D., ... Raghunathan, A.

citation count

  • 21

complete list of authors

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

publication date

  • December 2018