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.

Sellar region atypical teratoid/rhabdoid tumors in adults: Clinicopathological characterization of five cases and review of the literature

Giannini C.;
2018

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.
2018
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.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/726693
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