title: Primary atypical teratoid/rhabdoid tumor of central nervous system in children: a clinicopathological analysis and review of literature in China.
abstract: Atypical teratoid/rhabdoid tumor (AT/RT) is a very rare and highly malignant embryonal tumor in the central nervous system (CNS). Five patients (4 girls and 1 boy) with AT/RT were treated in our hospital. The clinical histories, symptoms, neuroimaging aspects, therapies, histological and immunohistochemical findings and follow-up information were reviewed. The patients ranged from 8 to 40 months with a mean age of 20.6 months. One tumor was located in the spinal cord, two in cerebellum and two in the pineal region. The imagings of the tumors resemble medulloblastomas. Pathological examinations showed that one patient had medulloblastoma differentiation, one had choroid plexus carcinoma differentiation, and one had mesenchymal components. Immunohistochemical staining showed that all of the tumors lost the nuclear expression of integrase interactor 1 (INI1), and were positive for Vimentin, S-100 protein and epithelial membrane antigen. One case with no recurrence after 24 months may have benefited from radical excision and postoperative radiotherapy. The other 4 patients died 8, 4, 1 and 1-month respectively after operation without radiotherapy. The diagnosis of AT/RT depends on full sampling, careful observation the morphological characteristics and INI1 examination, even when the tumor are presented in uncommon sites, such as the spinal cord and the pineal region.
authors: Department of Pathology, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Min
authors: M
authors: Yang
authors: Department of Experimental Testing, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Xi
authors: X
authors: Chen
authors: Department of Neurosurgery, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Ning
authors: N
authors: Wang
authors: Department of Pathology, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Kun
authors: K
authors: Zhu
authors: Department of Radiology, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Ying-Zi
authors: YZ
authors: Hu
authors: Department of Pathology, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Yun
authors: Y
authors: Zhao
authors: Department of Pathology, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Yan
authors: Y
authors: Shu
authors: Department of Pathology, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Man-Li
authors: ML
authors: Zhao
authors: Department of Pathology, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Wei-Zhong
authors: WZ
authors: Gu
authors: Department of Pathology, Children's Hospital, Zhejiang University School of Medicine Hangzhou 310003, China.
authors: Hong-Feng
authors: HF
authors: Tang
mesh_terms: D000367:Age Factors; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D002454:Cell Differentiation; D016543:Central Nervous System Neoplasms / Q000737:chemistry / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D002675:Child, Preschool; D002681:China; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011237:Predictive Value of Tests; D018335:Rhabdoid Tumor / Q000737:chemistry / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D013724:Teratoma / Q000737:chemistry / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: Atypical teratoid/rhabdoid tumor; cerebellum; integrase interactor 1; pineal gland; spinal cord