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

doi:10.1093/jnen/nly091

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

Laboratory Medicine and Pathology

Research output: Contribution to journal › Article › peer-review

10 Scopus citations

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 language	English (US)
Pages (from-to)	1115-1121
Number of pages	7
Journal	Journal of Neuropathology and Experimental Neurology
Volume	77
Issue number	12
DOIs	https://doi.org/10.1093/jnen/nly091
State	Published - Dec 1 2018

Keywords

ATRT
INI1
Pituitary
SMARCB1
Sellar

ASJC Scopus subject areas

General Medicine

Access to Document

10.1093/jnen/nly091

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

Sellar region atypical teratoid/rhabdoid tumors in adults: Clinicopathological characterization of five cases and review of the literature. / Paolini, Michael A.; Kipp, Benjamin R.; Sukov, William R. et al.
In: Journal of Neuropathology and Experimental Neurology, Vol. 77, No. 12, 01.12.2018, p. 1115-1121.

Research output: Contribution to journal › Article › peer-review

Paolini, MA, Kipp, BR, Sukov, WR, Jenkins, SM, Barr Fritcher, EG, Aranda, D, SantaCruz, KS, Al-Dandan, S, Fisher, P, McDonald, WC, Bondurant, CP, Van Dyke Darkow, G, Giannini, C, Parisi, JE, Jentoft, ME & 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, vol. 77, no. 12, pp. 1115-1121. https://doi.org/10.1093/jnen/nly091

@article{b5311188b56040f2b1fa7756f73793cc,

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

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.",

keywords = "ATRT, INI1, Pituitary, SMARCB1, Sellar",

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

note = "Publisher Copyright: {\textcopyright} 2018 American Association of Neuropathologists, Inc.",

year = "2018",

month = dec,

day = "1",

doi = "10.1093/jnen/nly091",

language = "English (US)",

volume = "77",

pages = "1115--1121",

journal = "Journal of Neuropathology and Experimental Neurology",

issn = "0022-3069",

publisher = "Oxford University Press",

number = "12",

}

TY - JOUR

T1 - Sellar region atypical teratoid/rhabdoid tumors in adults

T2 - Clinicopathological characterization of five cases and review of the literature

AU - Paolini, Michael A.

AU - Kipp, Benjamin R.

AU - Sukov, William R.

AU - Jenkins, Sarah M.

AU - Barr Fritcher, Emily G.

AU - Aranda, Derick

AU - SantaCruz, Karen S.

AU - Al-Dandan, Sadeq

AU - Fisher, Pete

AU - McDonald, William C.

AU - Bondurant, Charles P.

AU - Van Dyke Darkow, Grant

AU - Giannini, Caterina

AU - Parisi, Joseph E.

AU - Jentoft, Mark E.

AU - Raghunathan, Aditya

PY - 2018/12/1

Y1 - 2018/12/1

N2 - 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.

AB - 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.

KW - ATRT

KW - INI1

KW - Pituitary

KW - SMARCB1

KW - Sellar

UR - http://www.scopus.com/inward/record.url?scp=85056667139&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85056667139&partnerID=8YFLogxK

U2 - 10.1093/jnen/nly091

DO - 10.1093/jnen/nly091

M3 - Article

C2 - 30295777

AN - SCOPUS:85056667139

SN - 0022-3069

VL - 77

SP - 1115

EP - 1121

JO - Journal of Neuropathology and Experimental Neurology

JF - Journal of Neuropathology and Experimental Neurology

IS - 12

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this