Chuah K L, Tan P H
Department of Pathology, Singapore General Hospital, Singapore.
Pathology. 1998 Aug;30(3):242-6. doi: 10.1080/00313029800169386.
Tuberous sclerosis is a rare genetic disease with protean clinical manifestations. The lesion most commonly described in the lung is lymphangiomyomatosis. There have been recent reports of multifocal micronodular pneumocyte hyperplasia, as well as a single case documentation of a clear cell tumor of the lung, in patients affected by the disease. We detail a case of a female Chinese patient with tuberous sclerosis who was incidentally discovered to have bilateral pneumothoraces. The open lung biopsy revealed combined histological features of multifocal micronodular pneumocyte hyperplasia, lymphangiomyomatosis and clear cell micronodules.
结节性硬化症是一种临床表现多样的罕见遗传病。肺部最常描述的病变是淋巴管平滑肌瘤病。最近有报道称，在患有该疾病的患者中出现了多灶性微小结节性肺细胞增生，以及一例肺透明细胞瘤的病例记录。我们详细介绍了一名患有结节性硬化症的中国女性患者，她偶然被发现患有双侧气胸。开胸肺活检显示有多灶性微小结节性肺细胞增生、淋巴管平滑肌瘤病和透明细胞微小结节的联合组织学特征。
