INI1/SMARCB1-Deficient Carcinoma (Rhabdoid Tumor) of the Lacrimal Gland

Integrase interactor 1 (INI1) is a tumor suppressor gene that is ubiquitously expressed in all nucleated cells. The loss of INI1 protein activity was first demonstrated in aggressive pediatric tumors, including atypical teratoid/rhabdoid (AT/RT) tumor of the central nervous system and malignant rhabdoid tumor of the kidney. Subsequently, INI1 deficiency was discovered in other pediatric and some adult neoplasms. The spectrum of INI1-negative tumors includes a wide variety of neoplasms that occur over a wide age range, are variably aggressive, and have a variable rhabdoid component on histopathologic evaluation. In this report, the authors describe a 27-year-old gravid woman with INI1-deficient carcinoma of the lacrimal gland, previously not described in this location. Accepted for publication December 10, 2018. The authors have no financial or conflict of interest for disclosure. Address correspondence and reprint requests to Jurij R. Bilyk, M.D., Suite 930, Wills Eye Hospital, 840 Walnut Street, Philadelphia, PA 19107. E-mail: jrbilyk@aol.com © 2019 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.

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