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. 2017 Apr 1;18(4):949-954.
doi: 10.22034/APJCP.2017.18.4.949.

Atypical Teratoid/ Rhabdoid Tumor of Brain: a Clinicopathologic Study of Eleven Patients and Review of Literature

Nasir Ud Din  1 , Abrar Barakzai, Aisha Memon, Sheema Hasan, Zubair Ahmad
Affiliations

Atypical Teratoid/ Rhabdoid Tumor of Brain: a Clinicopathologic Study of Eleven Patients and Review of Literature

Nasir Ud Din et al. Asian Pac J Cancer Prev. .

Abstract

Background: 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 the cerebrum. 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-1 was performed in all 11 cases. Follow up was obtained. Results: A total of 11 cases were identified. Seven patients were males and 4 were females. The ages ranged from 1 month to 48 months (mean 26.6 months). Six tumors were located in the cerebrum and 3 in the posterior fossa. Exact Location was not known in 2 cases. Histologically, rhabdoid cells were present in sheets in variable proportions in five cases, Medulloblastoma and PNET like areas were seen in 2 cases each. 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 in which it was performed. All 11 tumors lacked immunoreactivity for INI-1 protein. Four patients died of disease with a 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.

Keywords: Atypical teratoid/rhabdoid tumor; posterior fossa; cerebrum; INI 1.

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Figures

Figure 1
Figure 1
A) Mixture of Spindle and Epithelioid Tumor Cells and B) Sheets of Epithelioid Cells (H and E, 200x Magnification)
Figure 2
Figure 2
Immunohistochemical Expression of AT/RT. the Tumor Cells Showed Positivity for Cytokeratin AE1/AE3 (A), EMA (B) and Vimentin (C). There is Loss of Expression of INI 1 (D)
See this image and copyright information in PMC

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