CAJ | 학술논문

目的分析卵巢、子宫恶性中胚叶混合瘤(malignant mullerian mixed tumor,MMMT)的临床病理学特征,探讨其诊断要点及其预后.方法回顾性分析解放军总医院病理科2008 - 2013年12例经组织学诊断为卵巢、子宫恶性中胚叶混合瘤的病例,观察其病理组织学特点,分析其免疫表型,结合文献总结MMMT的临床病理学特征.结果 12例MMMT患者年龄43 ~ 74岁,平均年龄59.6岁.肿瘤发生部位:子宫3例,单侧卵巢8例,双侧卵巢及宫颈管1例.临床表现缺乏特异性,主要表现为阴道不规则流血、腹痛及盆腔包块.组织学上包括癌和肉瘤,其中浆液性乳头状癌6例,鳞状细胞癌1例,子宫内膜样腺癌5例;软骨肉瘤2例,平滑肌肉瘤4例,横纹肌肉瘤2例,子宫内膜间质肉瘤3例,子宫内膜间质肉瘤混合软骨肉瘤1例.免疫组化染色:所有癌成分CK阳性,肉瘤成分Vimentin阳性,其中平滑肌肉瘤h-caldesmon阳性,横纹肌肉瘤Desmin阳性,子宫内膜间质肉瘤CD10阳性,软骨肉瘤S-100阳性.治疗以手术+术后化疗为主.10例患者随访至2014年10月(2例失访),1例于术后1个月内死亡,7例于术后6 ~ 58个月复发,其余2例术后1 ~ 2年无肿瘤复发和转移.结论恶性中胚叶混合瘤属于恶性上皮-间叶性肿瘤,发病率低,确诊依据组织形态学特征及免疫组化标记综合判断,临床预后差.
목적 분석란소、자궁악성중배협혼합류(malignant mullerian mixed tumor,MMMT)적림상병이학특정,탐토기진단요점급기예후.방법 회고성분석해방군총의원병이과2008 - 2013년12례경조직학진단위란소、자궁악성중배협혼합류적병례,관찰기병리조직학특점,분석기면역표형,결합문헌총결MMMT적림상병이학특정.결과 12례MMMT환자년령43 ~ 74세,평균년령59.6세.종류발생부위:자궁3례,단측란소8례,쌍측란소급궁경관1례.림상표현결핍특이성,주요표현위음도불규칙류혈、복통급분강포괴.조직학상포괄암화육류,기중장액성유두상암6례,린상세포암1례,자궁내막양선암5례;연골육류2례,평활기육류4례,횡문기육류2례,자궁내막간질육류3례,자궁내막간질육류혼합연골육류1례.면역조화염색:소유암성분CK양성,육류성분Vimentin양성,기중평활기육류h-caldesmon양성,횡문기육류Desmin양성,자궁내막간질육류CD10양성,연골육류S-100양성.치료이수술+술후화료위주.10례환자수방지2014년10월(2례실방),1례우술후1개월내사망,7례우술후6 ~ 58개월복발,기여2례술후1 ~ 2년무종류복발화전이.결론 악성중배협혼합류속우악성상피-간협성종류,발병솔저,학진의거조직형태학특정급면역조화표기종합판단,림상예후차.
Objective To study the clinicopathological features of ovary and uterus malignant mullerian mixed tumor (MMMT), and investigate its diagnosis and prognosis.Methods Twelve cases with ovary and uterus malignant mullerian mixed tumor in department of pathology, Chinese PLA General Hospital from 2008 to 2013 were enrolled in this study, their macroscopy, microscopy and immunohistochemistry were investigated, and clinical data and literatures were reviewed.Results The average age of MMMT cases was 59.6 years (range from 43 to 74 years). Of the 12 cases, 3 cases were uterus MMMT, 8 cases were unilateral ovary and 1 case was both ovaries and cervix MMMT. The patients usually presented with abnormal vaginal bleeding, abdominal pain and pelvic mass with no specificity in clinic. The morphological changes included epithelial and mesenchymal components. Of the 12 cases, 6 cases were serous papillary carcinoma, 1 case was squamous cell carcinoma, 5 cases were endometrioid carcinoma and 2 cases had chondrosarcoma, 4 cases had leiomyosarcoma, 2 cases had rhabdomyosarcoma, 3 cases had endometrial stromal sarcoma, and 1 case had endometrial stromal sarcoma mixed chondrosarcoma. Immunohistochemically, CK was positive in all epithelial components, Vimentin was positive in all mesenchymal components, H-caldesmon was positive in leiomyosarcoma, Desmin was positive in rhabdomyosarcoma, CD10 was positive in endometrial stromal sarcoma, and S-100 was positive in chondrosarcoma. The main treatment was surgery plus chemotherapy. Ten cases were followed up (2 cases lost follow-up), 1 case died within one month after surgery, 7 cases relapsed during six tofifty-eight months and 2 cases had no tumor progression and metastasis during one and two years after surgery.Conclusion MMMT is a rare subtype of malignant epithelial mesenchymal mixed tumors. Because its clinical features are unspecific,final diagnosis need pathological and immunohistochemical staining and its prognosis is extremely poor.