Fujitaka Kazunori, Isobe Takeshi, Oguri Tetsuya, Yamasaki Masahiro, Miyazaki Mitsuru, Kohno Nobuoki, Takeshima Yukio, Inai Kouki
Second Department of Internal Medicine, Hiroshima University School of Medicine, Hiroshima, Japan.
Respiration. 2002;69(3):277-9. doi: 10.1159/000063635.
We present a case of a 39-year-old woman with sporadic tuberous sclerosis (TSC), whose chest radiograph demonstrated bilateral diffuse nodular shadowing. A transbronchial lung biopsy specimen revealed the possibility of multiple atypical adenomatous hyperplasia (AAH), which had not been reported in TSC. Thoracoscopic lung biopsy was, therefore, performed. The specimens revealed the characteristic histological and immunohistochemical features of micronodular pneumocyte hyperplasia, which has been reported as an extremely rare pulmonary manifestation of TSC. In addition, no evidence of AAH or any other pulmonary involvements of TSC including lymphangioleiomyomatosis were detected in biopsy specimens obtained at thoracoscopy.
我们报告一例39岁散发型结节性硬化症（TSC）女性患者，其胸部X线片显示双侧弥漫性结节状阴影。经支气管肺活检标本显示可能为多发性非典型腺瘤样增生（AAH），这在结节性硬化症中尚未见报道。因此，进行了胸腔镜肺活检。标本显示了微小结节性肺细胞增生的特征性组织学和免疫组化特征，这已被报道为结节性硬化症极为罕见的肺部表现。此外，在胸腔镜检查获取的活检标本中未检测到非典型腺瘤样增生或结节性硬化症的任何其他肺部受累证据，包括淋巴管平滑肌瘤病。
