Development of an Atypical Teratoid Rhabdoid Tumor in a Meningioma. (September 2017)

• Record Type: Journal Article
• Authors: Lach, Boleslaw; Kameda-Smith, Michelle; Singh, Sheila; Ajani, Olufemi
• Is Part Of: International journal of surgical pathology. Volume 25:Number 6(2017)
• Page Start: 567
• Page End: 572
• Publication Date: 2017-09
• Subjects: rhabdoid meningioma -- atypical teratoid rhabdoid tumor -- INI1 -- pediatric CNS tumors -- childhood meningioma; Pathology, Surgical -- Periodicals; 617.07
• DOI: 10.1177/1066896917707039
• Languages: English
• ISSNs: 1066-8969

Abstract

We describe an atypical teratoid rhabdoid tumor (AT/RT) with a component of low-grade and anaplastic rhabdoid meningioma in a 7-year-old child. The AT/RT was uniformly negative for INI1 and displayed immunoreactivity for vimentin, P53, CD99, cytokeratins with AE1/AE3 antibodies, epithelial membrane antigen, β-catenin, smooth muscle actin, E-cadherin, and S-100 protein. AT/RT was continuous, with small foci of recognizable low-grade and anaplastic meningioma. The low-grade meningioma was INI1 positive with scattered INI1-negative nuclei, whereas the remaining tumor components were INI1 negative. A recurrent tumor 6 months after partial resection contained only INI1-negative AT/RT. This case supports the hypothesis that rare examples of AT/RT may emerge from a preexisting "parent" neoplasm as a result of a second hit mutation.