Sellar Region Atypical Teratoid/Rhabdoid Tumors in Adults: Clinicopathological Characterization of Five Cases and Review of the Literature. Issue 12 (14th September 2018)

• Record Type: Journal Article
• 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
• Is Part Of: Journal of neuropathology and experimental neurology. Volume 77:Issue 12(2018)
• Page Start: 1115
• Page End: 1121
• Publication Date: 2018-09-14
• Subjects: ATRT -- INI1 -- Pituitary -- Sellar -- SMARCB1; Neurology -- Diseases -- Periodicals; Neurology -- Diseases -- Physiopathology -- Periodicals; 616.8047
• DOI: 10.1093/jnen/nly091
• Languages: English
• ISSNs: 0022-3069

Abstract

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.