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

Abstract:

: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.

authors

Srinivasan A,Tuluc M,Jordan AJ Jr,Curry JM,Bilyk JR

doi

10.1097/IOP.0000000000001311

subject

Has Abstract

pub_date

2019-03-01 00:00:00

pages

e41-e43

issue

2

eissn

0740-9303

issn

1537-2677

pii

00002341-201903000-00032

journal_volume

35

pub_type

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