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Patrick Yang1, Christopher B Toomey1, Jonathan Lin2
1Division of Oculofacial Plastic and Reconstructive Surgery, Viterbi Family, Department of Ophthalmology, University of California, San Diego, California, USA.
Survey of Ophthalmology
|April 13, 2019
Summary
Integrase interactor 1-deficient sinonasal carcinoma (SMARCB1-deficient) is a rare cancer. A recent case highlights permanent vision loss following hemorrhage and orbital compartment syndrome.
Area of Science:
- Oncology
- Pathology
Background:
- Switch/sucrose non-fermentable-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1)-deficient sinonasal carcinoma is a rare malignancy.
- First described in 2014, only 39 cases have been reported, often with basaloid or plasmacytoid/rhabdoid morphology.
Observation:
- This report details a patient diagnosed with SMARCB1-deficient sinonasal carcinoma.
- The patient experienced permanent vision loss.
Findings:
- The vision loss occurred subsequent to acute hemorrhage.
- The hemorrhage was induced by Valsalva maneuver, leading to orbital compartment syndrome.
Implications:
- This case underscores the potential for severe complications in SMARCB1-deficient sinonasal carcinoma.
- Highlights the critical need for prompt recognition and management of hemorrhage and associated orbital complications.
