一例双侧颌下腺Warthin样黏液表皮样癌的 诊断与治疗
Diagnosis and Treatment of Bilateral Warthin-Like Mucoepidermoid Carcinoma of the Submandibular Glands: A Case Report
摘要: 目的:报告1例双侧颌下腺Warthin样黏液表皮样癌(WL-MEC)病例,探讨其临床特征及诊疗策略。方法:回顾分析1例41岁女性双侧颌下腺WL-MEC患者,结合影像学、病理学、免疫组化及FISH检测MAML2基因重排明确诊断。患者接受“右侧颌下腺摘除术 + 双侧颌下肿物切除术”并行术后调强放疗(200 cGy/30次,共6000 cGy),随访评估预后。结果:患者双侧颌下腺肿物,影像学提示囊实性病变,病理示双侧病灶囊性变并缺乏典型的Warthin瘤双层嗜酸性上皮结构,免疫组化显示Ki-67增殖指数约10%。FISH检测MAML2基因易位阳性。放疗过程顺利,术后28个月随访未见复发或转移。结论:双侧颌下腺WL-MEC为罕见病例,MAML2基因易位是诊断及鉴别诊断的关键。对于高危病例,术后辅助放疗能有效巩固局部控制。
Abstract: Objective: To report a case of bilateral Warthin-like mucoepidermoid carcinoma (WL-MEC) of the submandibular glands and to investigate its clinical characteristics, diagnostic approaches, and therapeutic strategies. Methods: A retrospective analysis was conducted on a 41-year-old female patient with bilateral WL-MEC of the submandibular glands. The diagnosis was confirmed through a combination of imaging, histopathology, immunohistochemistry, and fluorescence in situ hybridization (FISH) detection of MAML2 gene rearrangement. The patient underwent “right submandibular gland resection combined with bilateral submandibular mass excision”, followed by postoperative intensity-modulated radiotherapy (200 cGy per fraction, 30 fractions, total dose 6000 cGy). Prognosis was evaluated through follow-up. Results: The patient presented with bilateral submandibular gland masses. Imaging suggested cystic and solid lesions. Histopathology revealed cystic changes in bilateral lesions and the absence of the typical bilayered oncocytic epithelium characteristic of Warthin tumor. Immunohistochemistry showed a Ki-67 proliferation index of approximately 10%. FISH detection was positive for MAML2 gene translocation. The radiotherapy course was uneventful. At the 28-month postoperative follow-up, no recurrence or metastasis was observed. Conclusion: Bilateral WL-MEC of the submandibular glands is a rare entity. MAML2 gene translocation is pivotal for its diagnosis and differential diagnosis. For high-risk cases, postoperative adjuvant radiotherapy can effectively consolidate local control.
文章引用:夏顺贤, 谭叶, 陆海军. 一例双侧颌下腺Warthin样黏液表皮样癌的 诊断与治疗[J]. 临床医学进展, 2026, 16(4): 4579-4585. https://doi.org/10.12677/acm.2026.1641729

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