Suzuki Kazuhiko, Tanaka Hiroshi, Shiratori Masanori, Yamada Gen, Itoh Takayuki, Tanaka Shintaro, Nagata Manabu, Saizen Hitoki, Satoh Masaaki, Abe Shosaku
Third Department of Internal Medicine, Sapporo Medical University School of Medicine.
Intern Med. 2002 Oct;41(10):879-82. doi: 10.2169/internalmedicine.41.879.
A 44-year-old woman was admitted to our hospital in August 1999 for multiple large nodules detected on chest roentgenogram in an annual health check. Chest CT scans showed bilateral large nodules (>10 mm in diameter) with irregular margins and multiple thin walled cystic lesions. From these radiologic examinations, we suspected pulmonary Langerhans cell histiocytosis. Histological examination of the biopsy specimen by video-assisted thoracoscopy revealed a marked proliferation of the spindle cells, which were immunologically positive for alpha-smooth muscle actin and HMB-45, in the cyst walls and lung parenchyma. The large nodules consisted of proliferation of the smooth muscle cells surrounded by a dense layer of hemosiderinladen macrophages. During the two years subsequent to these 1999 examinations, the opacities have gradually diminished and the patient was found to have pulmonary lymphangioleiomyomatosis. This case exhibited rare radiologic manifestations of multiple large nodules mimicking Langerhans cell histiocytosis.
一名44岁女性于1999年8月因年度健康检查时胸部X线片发现多个大结节而入院。胸部CT扫描显示双侧大结节（直径>10mm），边缘不规则，并有多个薄壁囊性病变。根据这些影像学检查，我们怀疑为肺朗格汉斯细胞组织细胞增多症。经电视辅助胸腔镜活检标本的组织学检查显示，囊肿壁和肺实质中的梭形细胞显著增殖，这些细胞对α-平滑肌肌动蛋白和HMB-45免疫呈阳性。大结节由平滑肌细胞增殖组成，周围有一层密集的含铁血黄素巨噬细胞。在1999年这些检查后的两年里，肺部混浊逐渐减轻，该患者被诊断为肺淋巴管平滑肌瘤病。此病例表现出罕见的影像学表现，即多个大结节类似朗格汉斯细胞组织细胞增多症。
