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颌骨原发性骨内黏液表皮样癌:对MD安德森癌症中心经验的重新评估

Primary intraosseous mucoepidermoid carcinoma of the jaw: Reappraisal of The MD Anderson Cancer Center experience.

作者信息

Bell Diana, Lewis Carol, El-Naggar Adel K, Weber Randal S

机构信息

Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas.

Department of Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas.

出版信息

Head Neck. 2016 Apr;38 Suppl 1:E1312-7. doi: 10.1002/hed.24219. Epub 2015 Aug 28.

Abstract

BACKGROUND

Mucoepidermoid carcinoma arises from major or minor salivary glands, making up 10% of salivary gland tumors. Intraosseous mucoepidermoid carcinomas are rare, and make up only 2% to 3% of all mucoepidermoid carcinomas. The t(11;19) and its CRTC1-MAML2 fusion gene transcript have been identified in mucoepidermoid carcinoma and are associated with a subset of mucoepidermoid carcinomas. The extent to which the transcript influences disease features and patient survival is unclear.

METHODS

We conducted a retrospective analysis of records for clinical features, surgical interventions, and prognoses. Reverse transcriptase-polymerase chain reaction (RT-PCR) and fluorescence in situ hybridization (FISH) used to assess the t(11;19) fusion gene in intraosseous mucoepidermoid carcinoma.

RESULTS

Twenty-five patients with intraosseous mucoepidermoid carcinoma treated between 1998 and 2013 were identified. The t(11;19) fusion gene transcript CRTC1-MAML2 manifested in 9 intraosseous mucoepidermoid carcinomas, whereas is was not detected in another 9 intraosseous carcinomas. Although the incidence of this fusion in mucoepidermoid carcinoma varies, it is generally accepted that more than 50% of this entity manifest the CRTC1-MAML2.

CONCLUSION

Intraosseous mucoepidermoid carcinoma diagnosis should be based on clinical and pathologic manifestations and complete resection is the first choice for patient treatment. The need for neck dissection and adjuvant treatment are debatable. Radiotherapy may improve prognosis and may be recommended in the postoperative period. Primary intraosseous mucoepidermoid carcinoma can manifest the fusion transcript in a subset of tumors. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1312-E1317, 2016.

摘要

背景

黏液表皮样癌起源于大唾液腺或小唾液腺,占唾液腺肿瘤的10%。骨内黏液表皮样癌罕见,仅占所有黏液表皮样癌的2%至3%。t(11;19)及其CRTC1 - MAML2融合基因转录本已在黏液表皮样癌中被鉴定出来,并与一部分黏液表皮样癌相关。该转录本对疾病特征和患者生存的影响程度尚不清楚。

方法

我们对临床特征、手术干预和预后记录进行了回顾性分析。采用逆转录聚合酶链反应(RT-PCR)和荧光原位杂交(FISH)评估骨内黏液表皮样癌中的t(11;19)融合基因。

结果

确定了1998年至2013年间接受治疗的25例骨内黏液表皮样癌患者。t(11;19)融合基因转录本CRTC1 - MAML2在9例骨内黏液表皮样癌中表现出来,而在另外9例骨内癌中未检测到。尽管这种融合在黏液表皮样癌中的发生率有所不同,但普遍认为该实体超过50%表现出CRTC1 - MAML2。

结论

骨内黏液表皮样癌的诊断应基于临床和病理表现,完整切除是患者治疗的首选。是否需要进行颈部清扫和辅助治疗存在争议。放疗可能改善预后,可在术后推荐使用。原发性骨内黏液表皮样癌可在一部分肿瘤中表现出融合转录本。©2015威利期刊公司。头颈外科38: E1312 - E1317, 2016。

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