摘要
目的:平滑肌肉瘤(leiomyosarcoma,LMS)约占所有女性生殖系统恶性肿瘤的1%,由于该疾病相对少见,目前对其认识有限。LMS早期诊断困难,病理学检查是诊断的“金标准”。本研究探讨女性生殖系统LMS的临床病理学特征、诊断及鉴别诊断。方法:收集首都医科大学附属北京妇产医院2019至2023年确诊的13例LMS,回顾性分析其临床病理特征。结果:患者年龄40~77岁,发生于子宫的10例,输卵管系膜1例,盆腔l例,阴道1例。大体观:肿瘤最大径为4~18 cm,1例为破碎肿瘤组织,其余肿物均呈巨大结节状,切面灰白或灰黄,实性,细腻,质软-中,部分病例呈典型的鱼肉样外观。镜下观:3例为上皮样LMS,10例为经典型(梭形细胞)LMS,大多数病例肿瘤细胞密集,核分裂象活跃。免疫表型:平滑肌肌动蛋白(smooth muscle actin,SMA)、结蛋白(Desmin)、高分子量钙调素结合蛋白(h-caldesmon)均呈弥漫阳性,CD34、CD117、间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)均为阴性,细胞增殖指数Ki-67为15%~90%。术后随访1~32个月,5例局部复发。结论:LMS是女性生殖系统最常见的肉瘤类型,其临床表现缺乏特异性,影像学检查对术前诊断很重要,病理诊断需结合形态学特征和免疫表型。治疗以手术完整切除为主。该肿瘤侵袭性强,复发及转移率高,预后差。
Objective:Leiomyosarcoma(LMS)accounts for approximately 1%of all malignancies in the female reproductive system.Due to the relative rarity of this disease,it is currently not well understood.LMS is difficult to diagnose early,and pathological examination is the“gold standard”for diagnosis.This study aims to investigate the clinicopathological characteristics,diagnosis,and differential diagnosis of LMS in the female reproductive system.Methods:A total of 13 cases of LMS diagnosed between 2019 and 2023 at the Beijing Obstetrics and Gynecology Hospital were collected.The clinicopathological characteristics of these cases were retrospectively analyzed.Results:Patients ranged in age from 40 to 77 years.LMS occurred in the uterus in 10 cases,the mesosalpinx in 1 case,the pelvis in 1 case,and the vagina in 1 case.Gross examination showed tumor sizes ranging from 4 to 18 cm in diameter,with 1 case having fragmented tumor tissues.The remaining tumors appeared as large nodular masses with grayish-white or grayish-yellow,solid,smooth,and soft to medium textures,and some exhibited a typical fish-flesh appearance.Microscopically,3 cases were epithelioid LMS and 10 cases were classic LMS(spindle cell).Most tumors had densely packed cells with active mitotic figures.Immunophenotype analysis showed diffuse positivity for smooth muscle actin(SMA),Desmin,and h-caldesmon,while CD34,CD117,and anaplastic lymphoma kinase(ALK)were negative.The cell multiplication index Ki-67 ranged from 15%to 90%.Postoperative follow-up lasted from 1 to 32 months,with 5 cases experiencing local recurrence.Conclusion:LMS is the most common type of sarcoma in the female reproductive system,with nonspecific clinical manifestations.Imaging examination is important for preoperative diagnosis,and pathological diagnosis requires combining morphological features with immunophenotype.The primary treatment is complete surgical resection.LMS is highly aggressive,with high recurrence and metastasis rates,and has a poor prognosis.
作者
罗冬云
郑兴征
钟萍萍
支文雪
金玉兰
LUO Dongyun;ZHENG Xingzheng;ZHONG Pingping;ZHI Wenxue;JIN Yulan(Department of Pathology,Beijing Obstetrics and Gynecology Hospital,Capital Medical University/Beijing Maternal and Child Health Care Hospital,Beijing 100006,China)
出处
《临床与病理杂志》
CAS
2024年第4期532-539,共8页
Journal of Clinical and Pathological Research
基金
北京市自然科学基金(7202048)。
关键词
平滑肌肉瘤
子宫
女性生殖系统
免疫组织化学
诊断
leiomyosarcoma
uterus
female reproductive system
immunohistochemistry
diagnosis
