{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T12:42:14Z","timestamp":1756384934575},"reference-count":39,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2010,8]]},"abstract":"<jats:p>An atypical teratoid\/rhabdoid tumor (AT\/RT) is a highly malignant embryonal tumor most often occurring in the posterior fossa in children younger than 3 years of age. Adult cases of AT\/RT are very rare, and 27 cases with a diagnosis of either AT\/RT or (malignant) rhabdoid tumor have been reported to date. The authors report an adult case of an AT\/RT occurring in the pineal region with molecular cytogenetic and immunohistochemical confirmation. A 33-year-old woman presented with a 2-month history of headache and blurred vision progressing to diplopia, and was admitted emergently due to deteriorating mental status. An MR image showed a heterogeneously enhancing mass involving the posterior third ventricle and pineal region with mild hydrocephalus. She underwent a subtotal resection of the tumor and was then treated with chemoradiation. Thirteen months after surgery, she was still alive with radiological evidence of recurrence\/residual lesions. Histological sections showed epithelioid cellular sheets of rhabdoid tumor cells with scattered mitotic figures. Immunohistochemically, the tumor cells were diffusely and strongly positive for epithelial membrane antigen and vimentin, and showed focal expression of glial fibrillary acidic protein, pancytokeratin, and neurofilament protein. Loss of nuclear immunoreactivity for INI1 protein was observed. Fluorescence in situ hybridization analysis showed monosomy 22. Histologically, this tumor consisted exclusively of epithelioid tumor cells with rhabdoid features. The differential diagnoses include rhabdoid glioblastoma, metastatic carcinoma, and rhabdoid meningioma. Molecular testing to identify monosomy 22 or deletions of the chromosome 22q11 containing the <jats:italic>INI1\/hSNF5<\/jats:italic> gene and\/or immunohistochemical staining with INI1 antibody is of great importance for the diagnosis of this tumor.<\/jats:p>","DOI":"10.3171\/2009.10.jns09964","type":"journal-article","created":{"date-parts":[[2009,11,13]],"date-time":"2009-11-13T15:03:44Z","timestamp":1258124624000},"page":"374-379","source":"Crossref","is-referenced-by-count":33,"title":["Atypical teratoid\/rhabdoid tumor of the pineal region in an adult"],"prefix":"10.3171","volume":"113","author":[{"given":"Hidehiro","family":"Takei","sequence":"first","affiliation":[{"name":"Departments of Pathology, The Methodist Hospital,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adekunle M.","family":"Adesina","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer Center and Baylor College of Medicine; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vidya","family":"Mehta","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer Center and Baylor College of Medicine; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suzanne Z.","family":"Powell","sequence":"additional","affiliation":[{"name":"Departments of Pathology, The Methodist Hospital,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lauren A.","family":"Langford","sequence":"additional","affiliation":[{"name":"The University of Texas, MD Anderson Cancer Center, Houston, Texas"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-jns09964","series-title":"Neuroradiology","first-page":"363","article-title":"Primary malignant rhabdoid tumour of the brain in an adult","volume":"42","author":"Arrazola","year":"2000"},{"key":"b2-jns09964","series-title":"Med Pediatr Oncol","first-page":"223","article-title":"Implantation metastasis of primary malignant rhabdoid tumor of the brain in an adult (one case report)","volume":"28","author":"Ashraf","year":"1997"},{"key":"b3-jns09964","series-title":"Cancer","first-page":"1937","article-title":"Histopathology and prognosis of Wilms tumors: results from the First National Wilms' Tumor Study","volume":"41","author":"Beckwith","year":"1978"},{"key":"b4-jns09964","series-title":"Hum Pathol","first-page":"156","article-title":"A role for fluorescence in situ hybridization detection of chromosome 22q dosage in distinguishing atypical teratoid\/rhabdoid tumors from medulloblastoma\/central primitive neuroectodermal tumors","volume":"32","author":"Bruch","year":"2001"},{"key":"b5-jns09964","series-title":"Int J Radiat Oncol Biol Phys","first-page":"247","article-title":"Regarding Weiss et al., IJROBP 41:103\u2013109; 1998","volume":"45","author":"Byram","year":"1999"},{"key":"b6-jns09964","series-title":"J Neurooncol","first-page":"217","article-title":"Atypical teratoid\/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma","volume":"84","author":"Chacko","year":"2007"},{"key":"b7-jns09964","series-title":"Int J Radiat Oncol Biol Phys","first-page":"1038","article-title":"Impact of radiotherapy for pediatric CNS atypical teratoid\/rhabdoid tumor (single institute experience)","volume":"64","author":"Chen","year":"2006"},{"key":"b8-jns09964","series-title":"Virchows Arch A Pathol Anat Histopathol","first-page":"81","article-title":"Rhabdoid tumours of the central nervous system. 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