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头颈部黏液表皮样癌:CRTC1/3-MAML2 易位及其预后因素。

Mucoepidermoid carcinoma of the head and neck: CRTC1/3 MAML 2 translocation and its prognosticators.

机构信息

Department of Otolaryngology- Head and Neck Surgery, Amsterdam University Medical Centers, Vrije Universiteit Amsterdam, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands.

Department of Epidemiology and Biostatistics, Amsterdam University Medical Centers, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.

出版信息

Eur Arch Otorhinolaryngol. 2022 May;279(5):2573-2581. doi: 10.1007/s00405-021-07039-2. Epub 2021 Aug 17.

DOI:10.1007/s00405-021-07039-2
PMID:34405264
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8986707/
Abstract

PURPOSE

Mucoepidermoid carcinoma (MEC) of the head and neck is a prevalent malignant salivary gland tumour with a reported good outcome. The aim of this study was to report the outcome in our centre.

METHODS

A retrospective chart analysis with survival analyses was performed combined with fluorescence in situ hybridization (FISH) analysis to assess CRTC1/3 MAML 2 fusion gene presence.

RESULTS

Sixty-four cases of MEC were identified. Median age at presentation was 51.4 years with a predominance for parotid gland involvement. Five, 10- and 20- year disease-free survival was 98%, 90% and 68%, respectively. Overall survival was 94%, 90% and 64%, respectively. Local recurrence was seen up to 14 years after primary diagnosis; distant metastases were diagnosed up to 17 years later. The overall recurrence rate was less than 20 per cent. CRTC1/3 MAML 2 fusion gene presence showed no survival benefit.

CONCLUSION

MEC of the head and neck has a favorable outcome with the exception of high-grade MEC. PNI and nodal involvement are not rare. CRTC1/3 MAML 2 fusion gene presence showed no survival benefit. The tendency for late onset of loco-regional and distant recurrence should not be underestimated.

摘要

目的

头颈部黏液表皮样癌(MEC)是一种常见的恶性涎腺肿瘤,报告的预后良好。本研究旨在报告我们中心的结果。

方法

进行了回顾性图表分析,并结合荧光原位杂交(FISH)分析评估 CRTC1/3 MAML 2 融合基因的存在,进行生存分析。

结果

共发现 64 例 MEC。发病时的中位年龄为 51.4 岁,以腮腺受累为主。5、10 和 20 年无病生存率分别为 98%、90%和 68%。总生存率分别为 94%、90%和 64%。局部复发发生在原发性诊断后长达 14 年;远处转移发生在 17 年后。总体复发率低于 20%。CRTC1/3 MAML 2 融合基因的存在并未显示出生存获益。

结论

头颈部 MEC 的预后良好,但高级别 MEC 除外。PNI 和淋巴结受累并不少见。CRTC1/3 MAML 2 融合基因的存在并未显示出生存获益。不应低估局部和远处复发的迟发性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/8ca27e96f4ed/405_2021_7039_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/9c59a7591600/405_2021_7039_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/ab84d3398a02/405_2021_7039_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/6681085d01ea/405_2021_7039_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/9c0789b08e9a/405_2021_7039_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/8ca27e96f4ed/405_2021_7039_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/9c59a7591600/405_2021_7039_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/ab84d3398a02/405_2021_7039_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/6681085d01ea/405_2021_7039_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/9c0789b08e9a/405_2021_7039_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/457c/8986707/8ca27e96f4ed/405_2021_7039_Fig5_HTML.jpg

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