淋巴管平滑肌瘤病(LAM)是一种罕见的囊性疾病,由于平滑肌样细胞的异常增殖而发生。它主要影响肺,但也可以有肺外表现,如淋巴管平滑肌瘤和血管平滑肌脂肪瘤。在育龄妇女中更为常见,女性性激素导致了疾病进程。LAM可以通过偶发性突变或结节性硬化症(TSC)基因的遗传遗传而发展。TSC,LAM,子宫内膜癌与mTOR通路激活有关,这可以解释为什么这些疾病可以共存,尽管LAM和子宫内膜癌同时存在于同一患者中是非常罕见的。由于LAM的囊性,气胸最常在疾病过程中至少发生一次,大多数时候,这是在LAM中观察到的第一个表现。这些患者发生复发性气胸的风险也很高,当这种情况发生时,指示胸膜固定术。不幸的是,胸膜固定术仍不能排除气胸的发生。我们介绍了一名患有LAM和子宫内膜癌的女性患者,该患者在胸膜固定术后被发现患有自发性水气胸。LAM患者应密切监测其他mTOR相关疾病的可能发展。此外,在对极高危患者的复发性气胸进行胸膜固定术时,应采用复发率最低的手术.
Lymphangioleiomyomatosis (LAM) is a rare cystic disease that occurs due to the abnormal proliferation of smooth muscle-like cells. It primarily affects the lungs but can also have extrapulmonary manifestations such as lymphangioleiomyoma and angiomyolipomas. It is more common in young women of childbearing age, with female sex hormones contributing to the disease course. LAM can develop either through sporadic mutations or through genetic inheritance of the tuberous sclerosis complex (TSC) genes. TSC, LAM, and endometrial cancer are associated with mTOR pathway activation, which can explain why these diseases can co-exist, although the co-existence of LAM and endometrial cancer in the same patient is very rare. Due to the cystic nature of LAM, pneumothorax most often occurs at least once during the course of the disease, and most times, it is the first manifestation observed in LAM. These patients are also at high risk for recurrent pneumothorax, and when that occurs, pleurodesis is indicated. Unfortunately, pleurodesis still does not preclude a pneumothorax from occurring. We present the case of a female patient with LAM and endometrial cancer who was found to have an incidental spontaneous
hydropneumothorax after pleurodesis. Patients with LAM should be closely monitored for the possible development of other mTOR-associated diseases. Moreover, when performing pleurodesis for recurrent pneumothorax in very high-risk patients, the procedure with the lowest recurrence rate should be utilized.