Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review.

IF 2.1 4区医学 Q2 OTORHINOLARYNGOLOGY

Acta Otorhinolaryngologica Italica Pub Date : 2022-12-01 DOI:10.14639/0392-100X-N2087

Mario Turri-Zanoni, Gianluca Dalfino, Matt Lechner, Iacopo Dallan, Paolo Battaglia, Carla Facco, Francesca Franzi, Giacomo Gravante, Marco Ferrari, Dimitrios Terzakis, Amrita Jay, Martin D Forster, Andrea Luigi Ambrosoli, Maurizio Bignami, Christos Georgalas, Philippe Herman, Piero Nicolai, Valerie J Lund, Paolo Castelnuovo

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引用次数: 1

Abstract

Objective: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade cancer that was included from the 4th edition of WHO classification of head and neck tumours. The purpose of this study is to analyse clinical behaviour, pattern of recurrences and survival outcomes of this neoplasm.

Methods: Retrospective review of patients affected by BSNS who were treated via an endoscopic-assisted approach in 6 European tertiary-care referral hospitals. Cases of BSNS described in literature since 2012 to date were fully reviewed, according to PRISMA guidelines.

Results: A total of 15 patients were included. Seven patients were treated via an endoscopic endonasal approach, 4 with endoscopic transnasal craniectomy, and 4 via a cranio-endoscopic approach. Adjuvant treatment was delivered in 2 cases. After a mean follow-up of 27.3 months, systemic metastasis was observed in 1 case; the 5-year overall survival and disease-free survival rates were 100% and 80 ± 17.9%, respectively.

Conclusions: BSNS is a locally aggressive tumour with a low recurrence rate and encouraging survival outcomes if properly treated with surgical resection and free margins followed by adjuvant radiotherapy for selected cases. Endoscopic-assisted surgery is safe and effective as an upfront treatment within a multidisciplinary care protocol.

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双表型鼻窦肉瘤:欧洲多中心病例系列和系统文献综述。

目的:双表型鼻窦肉瘤(BSNS)是一种罕见的低级别肿瘤，已被列入世界卫生组织第4版头颈部肿瘤分类。本研究的目的是分析该肿瘤的临床行为、复发模式和生存结果。方法:回顾性分析6家欧洲三级医疗转诊医院经内窥镜辅助治疗的BSNS患者。根据PRISMA指南，对2012年至今文献中描述的BSNS病例进行了全面审查。结果:共纳入15例患者。7例患者经鼻内窥镜入路，4例经鼻内窥镜颅骨切除术，4例经颅内窥镜入路。辅助治疗2例。平均随访27.3个月，1例出现全身转移;5年总生存率为100%，无病生存率为80±17.9%。结论:BSNS是一种局部侵袭性肿瘤，复发率低，如果适当治疗，手术切除和游离切缘，然后对选定的病例进行辅助放疗，生存率高。内镜辅助手术作为多学科治疗方案的前期治疗是安全有效的。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Acta Otorhinolaryngologica Italica OTORHINOLARYNGOLOGY-

CiteScore

3.40

自引率

10.00%

发文量

审稿时长

6-12 weeks

期刊介绍： Acta Otorhinolaryngologica Italica first appeared as “Annali di Laringologia Otologia e Faringologia” and was founded in 1901 by Giulio Masini. It is the official publication of the Italian Hospital Otology Association (A.O.O.I.) and, since 1976, also of the Società Italiana di Otorinolaringoiatria e Chirurgia Cervico-Facciale (S.I.O.Ch.C.-F.). The journal publishes original articles (clinical trials, cohort studies, case-control studies, cross-sectional surveys, and diagnostic test assessments) of interest in the field of otorhinolaryngology as well as clinical techniques and technology (a short report of unique or original methods for surgical techniques, medical management or new devices or technology), editorials (including editorial guests – special contribution) and letters to the Editor-in-Chief. Articles concerning science investigations and well prepared systematic reviews (including meta-analyses) on themes related to basic science, clinical otorhinolaryngology and head and neck surgery have high priority.