• Jinjing Wang, Shuai Luo, Yao Li, and Hong Zheng.
• Department of Pathology, the First Affiliated Hospital of Zunyi Medical University, Guizhou, PR China.
• Medicine (Baltimore). 2021 Jan 22; 100 (3): e24377.

RationaleNasopharyngeal papillary adenocarcinoma is a region-specific tumor originating from the nasopharyngeal surface epithelium. Owing to its rarity, more attention has been paid to its clinicopathologic features, while little effort has been made to study the gene abnormalities that drive this tumor. We describe the first case of nasopharyngeal papillary adenocarcinoma harboring a fusion of ROS1 with GOPC.Patient ConcernsA 22-year-old female patient was diagnosed with nasopharyngeal papillary adenocarcinoma in our hospital, and she had right nasal obstruction for more than 6 months. Nasal endoscopy revealed a mass on the posterior roof of the nasopharynx.DiagnosesImmunohistochemical staining showed that the tumor cells were diffusely positive for transcription termination factor 1, vimentin, CK19, glypican-3, and CK7, and negative for melanocyte, CK5/6, CK20, P53, P63, S100, smooth muscle actin, p16, PAX8, and thyroglobulin. The Ki-67 index was approximately 5%; EBV-encoded small nuclear RNA was negative.InterventionsThe tumor was completely excised on endoscopy with a negative surgical margin.OutcomesNo sign of recurrence was observed during the 3-year follow-up period.LessonsOwing to its rarity, pathologists should be aware of this unusual neoplasm to avoid misdiagnosis. Further studies are needed to further characterize the relationship between ROS1-GOPC fusion and the pathogenesis of this carcinoma and its response to tyrosine kinase inhibitors in relapsed cases.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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