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摘要: 目的 分析颞骨罕见恶性肿瘤的临床表现及治疗效果。方法 回顾性分析我院2014年3月-2020年12月收治的4例颞骨罕见恶性肿瘤资料：包括软骨肉瘤2例、纤维肉瘤1例、内淋巴囊乳头状腺癌1例；男3例，女1例，年龄28~56岁；常见症状包括听力下降、面瘫、耳鸣、头痛等；所有患者均经颞骨CT及头颅MRI评估；采用颞骨次全切除术或颞下窝入路切除肿瘤，术后辅助放疗。结果 1例软骨肉瘤患者术后无瘤生存75个月，另1例为术后复发再次手术，无瘤生存112个月；1例纤维肉瘤术后辅以放疗，无瘤生存28个月；1例内淋巴囊乳头状腺癌术后辅以放疗，无瘤生存63个月。结论 颞骨罕见恶性肿瘤发病率极低，病变位置隐蔽，症状不典型，临床上要提高警惕、争取早诊早治。治疗方式以手术切除为主，切缘阳性及无法完全切除者可辅助放疗。Abstract: Objective To analyze the diagnosis, treatment and prognosis of patients with rare malignant tumors of the temporal bone.Methods Four cases of rare temporal bone malignant tumors in our hospital between March 2014 and December 2020 were reviewed, including two cases of chondrosarcoma, one case of fibrosarcoma and one case of endolymphatic cystic papillary adenocarcinoma. There were three males and one female, ages between 28 and 56 years at the time of surgery. Common symptoms included hearing loss, facioplegia, tinnitus, and headache. All patients underwent imaging examinations to evaluate the extent of the lesions. Tumors were removed by subtotal temporal bone resection or infratemporal fossa approach, and postoperative adjuvant radiotherapy was applied if necessary.Results One of the two chondrosarcoma patients was cured by complete resection of the tumor for 75 months, the other one recurred after the first excision of the tumor and underwent infratemporal fossa approach resection of skull base mass again with no recurrence found yet for 112 months. One patient with fibrosarcoma survived for 28 months after surgery with a positive margin and post-operative radiotherapy. One patient with endolymphatic cystic papillary adenocarcinoma recurred 12 months after subtotal lithotomy, and underwent subtotal temporal bone resection again, combined with radiotherapy. No recurrence was found for 63 months.Conclusion The incidence of rare temporal bone malignant tumors is extremely low, the location is hidden, and the symptoms are atypical. Attention should be paid for early detection and early treatment. Surgical resection is the main treatment, and radiotherapy can be supplemented in the advanced stage or with a positive margin.
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Key words:
- temporal bone /
- malignancies /
- surgery /
- radiotherapy /
- prognosis
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表 1 颞骨罕见恶性肿瘤患者治疗及结果
例序 年龄/岁 性别 临床表现 病理 病变范围 术式 放疗 生存时间/月 预后 1 54 男 视力下降、头痛、头晕、听力下降 纤维肉瘤 乳突、乙状窦 颞骨次全切除术 放疗 28 无瘤生存 2 28 男 面瘫、耳垂下方肿物 高分化软骨肉瘤 中耳、乙状窦、腮腺 颞下窝A型入路颅底肿物切除术 无 75 无瘤生存 3 56 女 耳闷、听力下降、耳鸣、耳溢液、耳出血、头痛 内淋巴囊侵袭性乳头状腺癌 中耳、外耳道、岩尖 颞骨次全切除术 放疗 63 患者第一次于我院行岩骨次全切除术，术后1年发现左侧外耳道肉芽，再次行颞骨次全切除术(术中见肿瘤破坏外耳道前壁累及颞下颌关节囊，累及面神经水平段)，术后病理证实面神经未累及，现无瘤生存 4 39 男 面瘫、耳痛、耳鸣、听力下降 软骨肉瘤 中耳、颈静脉孔区 颞下窝A型入路颅底肿物切除术 无 112 患者第一次于外院行颞骨次全切除术，术后5年复发，于我院行颞下窝A型入路颅底肿物切除术，现无瘤生存 
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