Wenaden Audrey Eleanor Therese, Copley Susan Jennifer
Department of Radiology, Hammersmith Hospital, London, UK.
J Thorac Imaging. 2005 Aug;20(3):226-8. doi: 10.1097/01.rti.0000155042.97403.3c.
Pulmonary involvement in tuberous sclerosis complex (TSC) resembles lymphangioleiomyomatosis (LAM) in its clinical, radiologic, and histopathological features. Typical high-resolution computed tomography (HRCT) findings are of bilateral thin-walled cysts symmetrically throughout both lungs, with associated increase in lung volumes. Atypical cases have been described; and we report a case of biopsy proven, focal unilateral disease. The presence of nodules has been reported in association with TSC that histologically are composed of proliferated type II pneumocytes. We discuss other atypical cases of TSC and sporadic LAM and review current concepts of the pathogenesis of the 2 diseases, with reference to our case.
结节性硬化症（TSC）的肺部受累在临床、放射学和组织病理学特征上类似于淋巴管平滑肌瘤病（LAM）。典型的高分辨率计算机断层扫描（HRCT）表现为双侧肺野弥漫性分布的薄壁囊肿，伴有肺容积增加。也有非典型病例的报道；我们报告一例经活检证实的局灶性单侧病变病例。有报道称，与TSC相关的结节在组织学上由增生的II型肺细胞组成。我们讨论了TSC和散发性LAM的其他非典型病例，并参考我们的病例回顾了这两种疾病发病机制的当前概念。
