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EnglishAtypical 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.
| Titolo: | Sellar region atypical teratoid/rhabdoid tumors in adults: Clinicopathological characterization of five cases and review of the literature |
| Autore/i: | 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. |
| Autore/i Unibo: | |
| Anno: | 2018 |
| Rivista: | |
| Digital Object Identifier (DOI): | http://dx.doi.org/10.1093/jnen/nly091 |
| 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. |
| Data stato definitivo: | 2020-02-16T00:51:57Z |
| Appare nelle tipologie: | 1.01 Articolo in rivista |
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