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目的分析散发型淋巴管肌瘤病(S-LAM)的临床及病理特点,提高对该病的认识。方法对11例经病理证实的S-LAM进行回顾性分析,采用SMA、vimentin、HMB45、Ki-67、CD34、D2-40、ER和PR等抗体进行免疫组化染色,并复习文献。结果 11例均为女性,表现为乳糜性胸或腹水,有胸闷、呼吸困难、气促等症状;病程中均有多个部位累及,包括肺、腹膜后、淋巴结。胸部CT示两肺散在或弥漫分布囊腔影,直径0.2~2 cm,囊壁薄而清晰;腹膜后受累者CT显示囊性低密度影。11例均有月经不调,其中3例合并子宫肌瘤,1例合并卵巢畸胎瘤、肾血管平滑肌脂肪瘤及肝血管瘤,1例合并小肠胃肠间质瘤,2例合并肝囊肿。免疫组化显示11例HMB45均(+),5例ER灶性(+),8例PR局灶(+)。随访5~60个月,患者均存活,且症状缓解。结论 LAM的临床表现无特异性,CT表现有一定的特异性,最终确诊依赖于组织病理,熟悉这些特点有助于早期诊断,但预后差。
Objective To analyze the clinical and pathological features of sporadic lymphangiomyosarcoma (S-LAM) and to improve the understanding of the disease. Methods 11 cases of pathologically confirmed S-LAM were retrospectively analyzed. Immunohistochemical staining was performed using antibodies such as SMA, vimentin, HMB45, Ki-67, CD34, D2-40, ER and PR, and reviewed the literature. Results All the 11 cases were female, showing chylothorax or ascites with chest tightness, dyspnea, shortness of breath and other symptoms. There were multiple sites involved in the course of the disease, including lung, retroperitoneum and lymph nodes. Chest CT showed two lungs scattered or diffuse cyst cavity shadow, diameter 0.2 ~ 2 cm, thin and clear wall; CT showed retroperitoneal cystic low density. All 11 cases had irregular menstruation, including 3 cases with uterine fibroids, 1 case with ovarian teratoma, renal angiomyolipoma and hepatic hemangioma, 1 case with small intestinal gastrointestinal stromal tumors and 2 cases with hepatic cysts. Immunohistochemistry showed 11 cases of HMB45 (+), 5 cases of ER focal (+), 8 cases of PR focal (+). All patients were followed up for 5 ~ 60 months and their symptoms were relieved. Conclusions The clinical manifestations of LAM are nonspecific. The CT findings have certain specificity. The final diagnosis depends on the histopathology. Familiarity with these characteristics is helpful for early diagnosis, but the prognosis is poor.