SMARCB1 缺陷型鼻腔鼻窦癌的预后：最大单中心横断面研究。

Outcomes of SMARCB1-deficient sinonasal carcinoma: Largest single-center cross-sectional study.

机构信息

Head and Neck Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York, USA.

Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA.

出版信息

Head Neck. 2024 Dec;46(12):3076-3084. doi: 10.1002/hed.27859. Epub 2024 Jul 23.

DOI:10.1002/hed.27859

PMID:39044555

Abstract

BACKGROUND

We evaluate outcomes of SMARCB1-deficient sinonasal carcinomas in the largest single-institution study.

METHODS

Retrospective cross-sectional study of patients with SMARCB1-deficient sinonasal carcinoma between 1998 and 2024. Disease-specific survival (DSS) and recurrence-free probability (RFP) at 1 and 5 years were measured by Kaplan-Meier method.

RESULTS

There were 47 patients with a median age of 53. Initial pathological diagnosis was altered in 33%. Twelve (34%) patients received neoadjuvant chemotherapy, with one partial response. Curative surgical approach was undertaken in 73%. Definitive chemoradiation was administered in 20%. DSS at 1 and 5 years was 93% and 45%, respectively. RFP at 1 and 5 years was 73% and 33%, respectively. On multivariate analysis, cranial nerve involvement (p = 0.01 for DSS) remained significantly worse for DSS and overall survival.

CONCLUSIONS

SMARCB1-deficient tumors had limited response to neoadjuvant chemotherapy. Cranial nerve involvement was associated with worse prognosis. Optimal treatment is unclear. Surgery should be offered to patients with resectable disease.

摘要

背景

我们评估了最大单机构研究中 SMARCB1 缺陷型鼻腔鼻窦癌的结局。

方法

回顾性分析 1998 年至 2024 年间 SMARCB1 缺陷型鼻腔鼻窦癌患者的资料。采用 Kaplan-Meier 法测量疾病特异性生存率（DSS）和 1 年及 5 年无复发生存率（RFP）。

结果

共纳入 47 例患者，中位年龄为 53 岁。初始病理诊断改变者占 33%。12 例（34%）患者接受了新辅助化疗，1 例部分缓解。73%的患者接受了根治性手术，20%的患者接受了根治性放化疗。1 年和 5 年 DSS 分别为 93%和 45%，1 年和 5 年 RFP 分别为 73%和 33%。多因素分析显示，颅神经受累（DSS 的 p 值=0.01）与 DSS 和总生存率显著相关。