-
摘要: 目的 探讨喉部横纹肌肉瘤(rhabdomyosarcoma，RMS)的临床表现、病理类型、治疗及预后，提高对该病临床特点的认识，提高诊断率，降低误诊率，探讨有效的诊治方法。方法 回顾性分析2015年5月—2021年5月郑州大学第一附属医院收治的5例喉RMS的临床及随访资料。结果 5例喉部RMS早期均被误诊，多发生在声带，肉眼观为光滑的肿块，多表现为声嘶。3例为胚胎型，1例多形型，1例梭形型。除早期行手术切除并术后辅以放化疗的患者随访至今无复发，3例因肿瘤复发死亡，1例肿瘤全身多处转移。结论 喉RMS发病率低，恶性程度高、预后差，易被误诊为良性肿块，确诊后尽早行扩大范围的手术切除并辅以放化疗。Abstract: Objective To explore the clinical manifestations, the type of pathology, treatment and prognosis of laryngeal rhabdomyosarcoma, and to enhance the understanding of the clinical characteristics of the disease, while improving the diagnosis rateand reducing the misdiagnosis rate, in order to explore effective diagnosis and treatment methods.Methods A retrospective analysis was conducted on the clinical data of 5 cases of laryngeal rhabdomyosarcoma treated in the First Affiliated Hospital of Zhengzhou University from May 2015 to May 2021.Results All 5 cases of laryngeal rhabdomyosarcoma were misdiagnosed in the early stage. with tumors mostly occurring in the vocal cords and appearing as smooth mass. The clinical symptoms were mostly hoarseness. According to pathological classification, three cases were embryonic type, one case was polymorphic type, and one case was spindle type.Three patients died due to tumor recurrence, one patient had multiple systemic metastases, and another patient who underwent surgical resection in the early stage and supplemented with postoperative radiotherapy and chemotherapyhas been followed up to date without recurrence.Conclusion Laryngeal rhabdomyosarcoma has low incidence rate, high malignancy degree and poor prognosis. It is easy to be misdiagnosed as a benign mass. Extensive surgical resection combined with radiotherapy and chemotherapy should be performed as soon as possible after diagnosis.
-
Key words:
- laryngeal neoplasms /
- rhabdomyosarcoma /
- treatment /
- prognosis
-
-
表 1 5例喉部RMS的临床资料
例序 性别 年龄
/岁临床表现 肿瘤位置 分期 治疗方式 病理类型 预后 1 女 75 吞咽困难伴憋气 杓状软骨表面 T1N0M0
Ⅰ期切除活检后放化疗 胚胎性，免疫组织化学Desmin(+)，My0D1(+)，Myogenin(弱+) 7个月后肿瘤复发死亡 2 男 49 声嘶2年 左室带侵犯声门下 T2N0M0
Ⅱ期喉裂开+放疗 梭形，免疫组织化学Desmin(+)，My0D1(弱+)，Myogenin(弱+) 1年后肿瘤复发死亡 3 男 61 声嘶半年 后联合、杓会厌壁 T2N0M0
Ⅱ期支撑喉镜下低温切除+放疗 胚胎性免疫组织化学Desmin(+)My0D1(-)，Myogenin(-)， 术后32个月肿瘤复发死亡 4 男 38 声嘶伴闷
气3个月左声门旁间隙，左侧环状软骨、杓状软骨、甲状软骨受累，左侧甲状腺侵犯，左侧面静脉内可见栓子 T4N0M0
Ⅳa期化疗后+全喉切+颈清扫+左甲状腺全切，右甲状腺部分切除+放疗 多形型，免疫组织化学Desmin(+)，My0D1(局灶+)，Myogenin(-)；颈部淋巴结未见肿瘤转移胚胎型，免疫组织化学Desmin(+)My0D1(+)，Myogenin(+)；颈部淋巴结未见肿瘤转移 随访术后5个月上腔静脉及无名静脉出现癌栓，肝转移、颈胸椎转移，现带瘤生存 5 男 42 声嘶2年 右声带前1/3一红色、光滑肿块 T1N0M0
Ⅰ期支撑喉镜下活检+化疗后18个月复发+垂直半喉切+右侧颈部淋巴结清扫 术后随访18个月无复发 
下载: 导出CSV
-
[1] Liu C, Li D, Hu J, et al. Chromosomal and genetic imbalances in Chinese patients with rhabdomyosarcoma detected by high-resolution array comparative genomic hybridization[J]. Int J Clin Exp Pathol, 2014, 7(2): 690-698.
[2] Jo VY, Fletcher CD. WHO classification of soft tissue tumours: an update based on the 2013(4th)edition[J]. Pathology, 2014, 46(2): 95-104. doi: 10.1097/PAT.0000000000000050
[3] Pittore B, Fancello G, Cossu Rocca P, et al. Rhabdomyosarcoma: a rare laryngeal neoplastic entity[J]. Acta Otorhinolaryngol Ital, 2010, 30(1): 52-57.
[4] 陈雪燕, 许春伟, 林丽燕, 等. 横纹肌肉瘤临床病理分析[J]. 临床与病理杂志, 2018, 38(7): 1446-1450.
[5] Hu J, Lu D, Ren J, et al. Adult laryngeal Embryonal Rhabdomyosarcoma: a case report and literature review[J]. BMC Surg, 2020, 20(1): 173. doi: 10.1186/s12893-020-00831-7
[6] Cobanoglu B, Simsek M, Senol S. Rhabdomyosarcoma of the upper lip in an adult patient[J]. Case Rep Med, 2015, 2015: 508051.
[7] Li Y, Fu Z, Chen W, et al. Embryonal Rhabdomyosarcoma of the Adult's Vocal Cord: A Case Report[J]. Iran Red Crescent Med J, 2015, 17(8): e28876.
[8] Little DJ, Ballo MT, Zagars GK, et al. Adult rhabdomyosarcoma: outcome following multimodality treatment[J]. Cancer, 2002, 95(2): 377-388. doi: 10.1002/cncr.10669
[9] Schrock A, Jakob M, Zhou H, et al. Laryngeal pleomorphic rhabdomyosarcoma[J]. Auris Nasus Larynx, 2007, 34(4): 553-556. doi: 10.1016/j.anl.2007.02.009
[10] 刘宇飞, 杨春蓉, 谈中华, 等. 成人鼻部胚胎性横纹肌肉瘤2例报道及文献复习[J]. 重庆医科大学学报, 2011, 36(8): 1014-1017.
[11] 杨征, 房居高, 钟琦, 等. 成人喉部横纹肌肉瘤临床分析[J]. 中国耳鼻咽喉头颈外科, 2017, 24(6): 275-278. https://www.cnki.com.cn/Article/CJFDTOTAL-EBYT201706001.htm
[12] Doyle LA, Nascimento AF. Soft Tissue Tumors[J]. Essentials of Anatomic Pathology, 2011: 995-1045.
[13] Ferrari A, Dileo P, Casanova M, et al. Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution[J]. Cancer, 2003, 98(3): 571-580.
-
图(2)
表(1)
计量
- 文章访问数: 736
- PDF下载数: 174
- 施引文献: 0