腺泡状横纹肌肉瘤伴胎盘转移的临床病理特点  

Alveolar rhabdomyosarcoma with placental metastasis during pregnancy

作　　者：程学斌[1] 田侠[1] 王昀[1] 刘爱军[2] 王利群[1] CHENG Xue-bin;TIAN Xia;WANG Yun;LIU Ai-jun;WANG Li-qun(Department of Pathology,the First Medical Center of PLA General Hospital,Beijing 100853,China;Department of Pathology,the 7th Medical Center of PLA General Hospital,Beijing 100010,China)

机构地区：[1]解放军总医院第一医学中心病理科,北京100039 [2]解放军总医院第七医学中心病理科,北京100010

出　　处：《诊断病理学杂志》2024年第1期16-19,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的观察孕妇腺泡状横纹肌肉瘤合并胎盘转移的临床病理特征及其对孕妇和胎儿的影响。方法回顾分析1例原发部位不明的妊娠中晚期胸腹部、肝脏及胎盘转移的腺泡状横纹肌肉瘤患者的临床病理资料,并复习相关文献。结果28岁女性患者入院时停经26+5周,孕2产1,腹胀超过半个月,胸闷3天。超声显示双侧胸腔积液及腹腔积液。大网膜增厚,厚约3.4 cm。MRI显示肝门周围转移,脾大。超声引导下经右侧胸腔穿刺引流,病理检查肝右前叶活检。因胎儿宫内死亡,至引产,胎盘病理。显微镜下胸腔积液、肝穿刺及胎盘组织显示小细胞恶性肿瘤,未成熟分化。免疫组化肌源性标记Desmin、Myo D1和Myogenin主要呈阳性表达。引产后,由于产妇的一般情况不佳,只给予对症治疗,无法给予相应的化疗。家属放弃治疗,患者在出院几天后死亡。结论孕妇腺泡状横纹肌肉瘤合并胎盘转移的情况很少见,预后不佳。在病理诊断中,应注意小细胞恶性肿瘤的鉴别诊断。考虑到治疗对孕妇和胎儿的影响,应慎重选择手术和化疗的时机。Objective To study the clinicopathological features of alveolar rhabdomyosarcoma with placental metastasis in pregnant women and its impact on pregnant women and fetuses. Methods The clinicopathological data of a case of alveolar rhabdomyosarcoma with sternoabdominal, liver and placental metastasis in the middle and late trimester of pregnancy with unknown primary site were retrospectively analyzed, and the relevant literatures were reviewed. Results The 28-year-old female patient was admitted to the hospital with menopause of 26+5 weeks, 2 parturients and 1 pregnancy, abdominal distension for more than half a month and chest tightness for 3 days. Ultrasonography showed bilateral pleural effusion and abdominal effusion. The omentum was thickened and the thicker part was about 3.4 cm. MRI showed perihilar metastasis, greater omentum thickening and splenomegaly. Ultrasound-guided puncture and drainage were performed on the right pleural cavity, followed by pathological examination and biopsy of the right anterior lobe of the liver. Because of fetal intrauterine death, the induced labor was performed with analyses of placental pathology. Microscopic pleural effusion, liver puncture and placental tissue showed small round blue cell tumor with immature differentiation. Immunohistochemistry mainly showed positive expression of myogenic markers Desmin, MyO-D1 and Myogenin. After induced labor, due to the poor general condition of the puerpera, only symptomatic treatment was given, and corresponding chemotherapy could not be given. The family members gave up treatment, and the patient died several days after discharge. Conclusion Fetal alveolar rhabdomyosarcoma with placental metastasis is rare and has a poor prognosis. In pathological diagnosis, attention should be paid to the differentiation of small cell malignant tumor. Considering the effect of treatment on pregnant women and fetus, the timing of surgery and chemotherapy should be carefully selected.

关 键 词：腺泡状横纹肌肉瘤 妊娠 胎盘转移 

分 类 号：R714.259[医药卫生—妇产科学]