Guinee D G, Feuerstein I, Koss M N, Travis W D
Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD.
Arch Pathol Lab Med. 1994 Aug;118(8):846-9.
The diagnosis of lymphangioleiomyomatosis was established in a 35-year-old woman with hemoptysis, mild cough, and dyspnea based on histologic review of results of a transbronchial biopsy correlated with high-resolution computed tomographic scan findings. A chest x-ray film revealed diffuse interstitial lung disease, and a high-resolution computed tomographic scan showed diffuse cystic changes throughout both lungs. The transbronchial biopsy specimen revealed cystic changes and a patchy, sometimes nodular proliferation of smooth muscle that focally expanded the interstitium suggestive of lymphangioleiomyomatosis. The smooth-muscle nature of the cells was confirmed by positive immunohistochemical stains for actin and desmin; positive staining for HMB-45 was also observed. Although the diagnosis of lymphangioleiomyomatosis usually requires an open lung biopsy, this case shows that rarely, in the appropriate clinical setting, the diagnosis may be rendered based on results of a transbronchial biopsy in conjunction with findings from ancillary immunohistochemical studies and high-resolution computed tomography.
一名35岁咯血、轻度咳嗽和呼吸困难的女性，经组织学检查支气管活检结果并与高分辨率计算机断层扫描结果相关联，确诊为淋巴管平滑肌瘤病。胸部X光片显示弥漫性间质性肺病，高分辨率计算机断层扫描显示双肺弥漫性囊性改变。支气管活检标本显示囊性改变以及平滑肌呈斑片状、有时呈结节状增生，局部扩张间质，提示淋巴管平滑肌瘤病。肌动蛋白和结蛋白免疫组化染色阳性证实了细胞的平滑肌性质；同时也观察到HMB - 45染色阳性。虽然淋巴管平滑肌瘤病的诊断通常需要开胸肺活检，但该病例表明，在适当的临床情况下，结合辅助免疫组化研究结果和高分辨率计算机断层扫描，很少能通过支气管活检做出诊断。
