title: Primary Intracranial Atypical Teratoid/Rhabdoid Tumors: A Clinicopathologic and Neuroradiologic Study.
abstract: Atypical teratoid/rhabdoid tumors are rare malignant pediatric brain tumors. This study was performed to characterize the clinicopathologic and neuroradiologic characteristics of atypical teratoid/rhabdoid tumors from 8 patients, including 5 male and 3 female infants (median age, 67 months). Neuroimaging revealed bulky masses of heterogeneous intensity with inhomogeneous enhancement. Three cases were infratentorial and 5 were supratentorial. Histopathologically, the tumors were predominantly composed of rhabdoid cells and undifferentiated small cells, mixed with some spindle or epithelial components. The tumors displayed striking polyphenotypic immunoreactivity, including varying degrees of positivity for vimentin, epithelial membrane antigen, smooth-muscle actin, cytokeratin, glial fibrillary acidic protein, neurofilament protein, synaptophysin, and CD99, and immunonegativity for desmin, placental alkaline phosphatase, and INI-1. The median survival duration was 9.5 months (range, 1-15 months) despite aggressive therapy. These results suggest that atypical teratoid/rhabdoid tumors display distinct clinicopathologic characteristics and indicate a poor prognosis. Immunohistochemistry facilitates the appropriate diagnosis of these tumors.
authors.affiliation: Department of Pathology, The People's Hospital of Zhengzhou University, Zhengzhou, Henan, People's Republic of China.
authors.forename: Rui-jiao
authors.initials: RJ
authors.lastname: Zhao
authors.affiliation: Department of Pathology, The People's Hospital of Zhengzhou University, Zhengzhou, Henan, People's Republic of China.
authors.forename: Kai-yan
authors.initials: KY
authors.lastname: Wu
authors.affiliation: Department of Neurosurgery, The People's Hospital of Zhengzhou University, Zhengzhou, Henan, People's Republic of China zhangjianguo2020@126.com.
authors.forename: Jian-guo
authors.initials: JG
authors.lastname: Zhang
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, People's Republic of China.
authors.forename: Yi-hui
authors.initials: YH
authors.lastname: Ma
authors.affiliation: Department of Pathology, The People's Hospital of Zhengzhou University, Zhengzhou, Henan, People's Republic of China.
authors.forename: Ling-fei
authors.initials: LF
authors.lastname: Kong
mesh_terms: D000071443:12E7 Antigen; D000293:Adolescent; D015703:Antigens, CD / Q000378:metabolism; D001932:Brain Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D015815:Cell Adhesion Molecules / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D002868:Chromosomal Proteins, Non-Histone / Q000378:metabolism; D004268:DNA-Binding Proteins / Q000378:metabolism; D005260:Female; D006801:Humans; D007223:Infant; D007633:Keratins / Q000378:metabolism; D008297:Male; D018396:Mucin-1 / Q000378:metabolism; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D000071796:SMARCB1 Protein; D013724:Teratoma / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D014057:Tomography, X-Ray Computed; D014157:Transcription Factors / Q000378:metabolism; D055815:Young Adult
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; D018396:Mucin-1; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; D014157:Transcription Factors; D007633:Keratins
keywords: Prognosis; atypical teratoid/rhabdoid tumor; brain neoplasm; immunohistochemistry