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目的总结肺淋巴管肌瘤病早期诊断的经验,进一步提高对本病的认识。方法回顾分析9例经胸腔镜、纤维支气管镜(纤支镜)活检病理确诊的肺淋巴管肌瘤病患者的临床影像学表现、腔镜检查结果。结果9例皆为女性,平均年龄35岁(20～49岁)。胸部CT检查可见两肺弥漫多发小囊状透光区,其中3例行腹部CT检查见腹膜后多发淋巴结肿大。9例均为肺组织病理活检确诊,其中胸腔镜手术3例,纤支镜检查6例。术后病理诊断与术前诊断符合率为44.4%(4/9)。结论肺淋巴管肌瘤病临床罕见,CT检查可提示诊断,运用胸腔镜及纤支镜活检是早期确诊的重要手段。
Objective To summarize the experience of early diagnosis of pulmonary lymphangiomyosarcoma and further improve the understanding of this disease. Methods The clinical imaging findings and endoscopic findings of 9 patients with pulmonary lymphangiomyoma confirmed by thoracoscopy and bronchofiberscopy were analyzed retrospectively. Results All 9 cases were female, with an average age of 35 years (20-49 years). Chest CT showed multiple diffuse many saccular light-transmitting areas, including three cases of abdominal CT examination showed multiple retroperitoneal lymph nodes. Nine cases were diagnosed by pathological biopsy of the lung, including thoracoscopic surgery in 3 cases, bronchoscopy in 6 cases. The coincidence rate of postoperative pathological diagnosis and preoperative diagnosis was 44.4% (4/9). Conclusions Lung lymphangiomyosarcoma is rare in clinic. The CT examination can be used to diagnose it. The use of thoracoscope and fiberoptic biopsy is an important means of early diagnosis.