Ishii Makoto, Asano Koichiro, Kamiishi Nobufumi, Hayashi Yuichiro, Arai Daisuke, Haraguchi Mizuha, Sugiura Hiroaki, Naoki Katsuhiko, Tasaka Sadatomo, Soejima Kenzo, Sayama Koichi, Betsuyaku Tomoko
Division of Pulmonary Medicine, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
J Med Case Rep. 2012 Oct 16;6:352. doi: 10.1186/1752-1947-6-352.
The majority of multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis complex is diagnosed with the classical clinical triad of seizures, mental retardation, and skin lesions. We report a rare case of tuberous sclerosis complex with no classical clinical findings, which was diagnosed through incidental computed tomography findings of multiple nodular lesions of multifocal micronodular pneumocyte hyperplasia.
A chest computed tomography scan of a 51-year-old Japanese woman showed multiple nodular ground-glass opacities that were not seen on chest X-ray. Video-assisted thoracoscopic surgery was performed. A histological examination demonstrated type II pneumocyte hyperplasia with thickened fibrotic alveolar septa, which was consistent with multifocal micronodular pneumocyte hyperplasia. Brain magnetic resonance imaging displayed multiple cortical tubers, and abdominal computed tomography showed bilateral renal angiomyolipoma. Our patient was finally diagnosed as having tuberous sclerosis complex with multifocal micronodular pneumocyte hyperplasia, although she had no episodes of epilepsy, no skin lesions, and no family history.
Multifocal micronodular pneumocyte hyperplasia with latent tuberous sclerosis complex should be considered in the differential diagnosis of multiple ground-glass opacities.
大多数与结节性硬化症相关的多灶性微小结节性肺细胞增生是通过癫痫、智力迟钝和皮肤病变这一经典临床三联征来诊断的。我们报告一例罕见的结节性硬化症病例，该病例无典型临床症状，通过计算机断层扫描偶然发现多灶性微小结节性肺细胞增生的多个结节性病变而得以诊断。
一名51岁日本女性的胸部计算机断层扫描显示多个结节状磨玻璃影，胸部X线片未见异常。遂行电视辅助胸腔镜手术。组织学检查显示II型肺细胞增生伴纤维化肺泡间隔增厚，符合多灶性微小结节性肺细胞增生。脑部磁共振成像显示多个皮质结节，腹部计算机断层扫描显示双侧肾血管平滑肌脂肪瘤。尽管该患者无癫痫发作、无皮肤病变且无家族史，但最终被诊断为结节性硬化症合并多灶性微小结节性肺细胞增生。
在鉴别诊断多个磨玻璃影时，应考虑合并潜在结节性硬化症的多灶性微小结节性肺细胞增生。
