Document Type

Article

Department

Pathology and Laboratory Medicine

Abstract

Creative Commons Attribution LicenseBackground: Atypical teratoid/ rhabdoid tumor (AT/RT) is a rare aggressive embryonal central nervous system(CNS) tumor of infancy and early childhood. Majority of the cases arise in the posterior fossa, and remaining in thecerebrum. Aims: To analyze the clinicopathologic features of AT/RT on a cohort of cases. Materials and methods:All reported cases of AT/RT at the Department of Pathology and Laboratory Medicine, Aga Khan University Hospital(AKUH) from 2007 to 2016 were reviewed for clinical and pathological features. Immunohistochemical stain for INI-1was performed in all 11 cases. Follow up was obtained. Results: A total of 11 cases were identified. Seven patients weremales and 4 were females. The ages ranged from 1 month to 48 months (mean 26.6 months). Six tumors were locatedin the cerebrum and 3 in the posterior fossa. Exact Location was not known in 2 cases. Histologically, rhabdoid cellswere present in sheets in variable proportions in five cases, Medulloblastoma and PNET like areas were seen in 2 caseseach. Immunohistochemical stains EMA (10/10), vimentin (7/7), CKAE1/AE3 (8/9), and CD99 (3/4), GFAP (6/10),ASMA (3/4) and synaptophysin (3/4) were positive in varying proportions while desmin was negative in all 6 cases inwhich it was performed. All 11 tumors lacked immunoreactivity for INI-1 protein. Four patients died of disease witha follow up ranging from 5 to 24 months. Conclusions: AT/RT is a rare highly aggressive embryonal tumor of CNS.A male predominance was noted in our series. We report the first and largest series from Pakistan.

Publication (Name of Journal)

Asian Pacific Journal of Cancer Prevention

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