Sinonasal NUT carcinoma: A retrospective case series from a single institution

Lei Wang; Zhenzhen Zhu; Weiqing Wang; Yang Zha; Xiaowei Wang; Aodeng Surita; Yuzhuo Liu; Wei Lv

doi:10.3389/fsurg.2023.1098704

Sinonasal NUT carcinoma: A retrospective case series from a single institution

Front Surg. 2023 Mar 1:10:1098704. doi: 10.3389/fsurg.2023.1098704. eCollection 2023.

Authors

Lei Wang¹, Zhenzhen Zhu¹, Weiqing Wang¹, Yang Zha¹, Xiaowei Wang¹, Aodeng Surita¹, Yuzhuo Liu¹, Wei Lv¹

Affiliation

¹ Department of Otolaryngology-Head and Neck Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.

Abstract

Purpose: Nuclear protein in testis (NUT) carcinoma is a rare, aggressive tumor defined by the presence of NUT gene rearrangement. The aim of this study was to describe the clinical, radiologic, and biological features of sinonasal NUT carcinoma.

Methods: We retrospectively investigated NUT expression with clinicopathologic features in 145 cases with sinonasal malignancies diagnosed from January 2017 to December 2021 and reviewed the reported cases.

Results: Three (3/145, 2.07%) cases showed strong nuclear expression for NUT immunohistochemical, including one male and two females with ages from 37 to 57 years (mean, 45.33 years). All three cases involved the nasal cavity and sinuses; one of them involved the orbit and intracranial area. Histologically, all subjects showed poorly differentiated, small round cell morphology with distinct nuclei. All patients received surgery and chemoradiotherapy. One patient died of the disease 13 months after diagnosis, and two survived 12 and 15 months, respectively, without evidence of tumor recurrence. 51 cases of sinonasal NUT carcinoma (mean age 40.96 years) have been described to date. Among them, 28 are male, and 23 are female. Most cases expressed p63, AE1/AE3, as well as p40.

Conclusion: NUT carcinoma is a rare and aggressive disease with a poor prognosis. It is crucial to perform NUT rearrangement-related tests for differential diagnosis of poorly differentiated/undifferentiated tumors in the nasal cavity and sinuses.

Keywords: NUTM1 protein human; molecular targeted therapy; nasal cavity; paranasal sinuses; prognosis.

Grants and funding

National Natural Science Foundation of China (grant number 82071027 and 82101200); Natural Science Foundation of Beijing (grant number 7202162); National High Level Hospital Clinical Research Funding (grant number 2022-PUMCH-B-096 and 2022-PUMCH-A-030).