title: Case of atypical teratoid/rhabdoid tumor in an adult, with long survival.
abstract: Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant tumor that mostly occurs in early childhood and has poor prognosis despite aggressive therapy. Adult cases are rare and, as far as we are aware, only 30 cases have been reported to date. Here we present the case of a 27-year-old female with left parietal AT/RT with the chief complaint of numbness of the right superior limb. First, the tumor was surgically removed and the diagnosis was grade II glioma. With additional radiotherapy, the clinical course after surgery was favorable. After 6 years, she had an operation for recurrence and the diagnosis was grade III glioma. Temozolomide was prescribed, and a disease-free period of 2 years followed. Surgery was performed for a third time for second recurrence with histology of diffuse growth of rhabdoid cells. Immunohistochemistry was partially positive for vimentin and epithelial membrane antigen. Ki-67 labeling index was extremely high and tumor cells showed no staining of INI1 suggestive of diagnosis of AT/RT. We re-evaluated past specimens and none had immunoreactivity of INI1. Ki-67 labeling index and O-6 methylguanine DNA methyltransferase (MGMT) staining were also re-examined and both increased gradually. She is still alive without recurrence for more than 1 year. As far as we are aware, this is the second longest survival of an adult with AT/RT.
authors.affiliation: Laboratory of Cancer Research, Department of Pathology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-Ku, Sapporo, Hokkaido, Japan.
authors.forename: Kenta
authors.initials: K
authors.lastname: Takahashi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nishihara
authors.forename: Masahito
authors.initials: M
authors.lastname: Katoh
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Yoshinaga
authors.forename: Roshan
authors.initials: R
authors.lastname: Mahabir
authors.forename: Hiromi
authors.initials: H
authors.lastname: Kanno
authors.forename: Taichi
authors.initials: T
authors.lastname: Kimura
authors.forename: Mishie
authors.initials: M
authors.lastname: Tanino
authors.forename: Jun
authors.initials: J
authors.lastname: Ikeda
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Sawamura
authors.forename: Kazuo
authors.initials: K
authors.lastname: Nagashima
authors.forename: Shinya
authors.initials: S
authors.lastname: Tanaka
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis*; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D002868:Chromosomal Proteins, Non-Histone / Q000032:analysis; D003131:Combined Modality Therapy; D015254:DNA Modification Methylases / Q000032:analysis; D045643:DNA Repair Enzymes / Q000032:analysis; D004268:DNA-Binding Proteins / Q000032:analysis; D003606:Dacarbazine / Q000031:analogs & derivatives / Q000627:therapeutic use; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D009364:Neoplasm Recurrence, Local; D019635:Neurosurgical Procedures; D010296:Parietal Lobe; D018335:Rhabdoid Tumor / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D000071796:SMARCB1 Protein; D000077204:Temozolomide; D013724:Teratoma / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D013997:Time Factors; D014157:Transcription Factors / Q000032:analysis; D025521:Tumor Suppressor Proteins / Q000032:analysis
chemical_list: D014408:Biomarkers, Tumor; D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; D014157:Transcription Factors; D025521:Tumor Suppressor Proteins; D003606:Dacarbazine; D015254:DNA Modification Methylases; C515491:MGMT protein, human; D045643:DNA Repair Enzymes; D000077204:Temozolomide