Shehzad Mustafa, Sabir Beenish, Shehzad Dawood, Malik Haris Mumtaz, Jha Anurag, Saddique Muhammad Nabeel, Iqbal Javed
Hackensack University Medical Center, New Jersey, United States.
Rawalpindi Medical University, Rawalpindi, Pakistan.
Radiol Case Rep. 2025 May 2;20(7):3510-3514. doi: 10.1016/j.radcr.2025.02.103. eCollection 2025 Jul.
Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease characterized by abnormal smooth muscle proliferation, leading to cystic destruction of the lung parenchyma. We present the case of a female in her early 40s who presented with intermittent chest pain for 1 month. Imaging revealed left hydropneumothorax with multiple bilateral pulmonary parenchymal cysts, and a subsequent lung biopsy confirmed the diagnosis of pulmonary LAM. The patient underwent video-assisted thoracoscopic surgery (VATS) with mechanical and chemical pleurodesis. Given her stable condition, no immediate sirolimus therapy was initiated, and close follow-up with serial imaging was planned. This case highlights the diagnostic challenges of LAM, especially with low VEGF-D levels, and underscores the role of pleurodesis as a management option in select patients. Early recognition and tailored management are essential to optimize patient outcomes.
淋巴管平滑肌瘤病（LAM）是一种罕见的进行性肺部疾病，其特征为异常的平滑肌增生，导致肺实质的囊性破坏。我们报告一例40岁出头的女性病例，该患者出现间歇性胸痛1个月。影像学检查显示左侧液气胸伴双侧多个肺实质囊肿，随后的肺活检确诊为肺LAM。该患者接受了电视辅助胸腔镜手术（VATS）及机械和化学胸膜固定术。鉴于其病情稳定，未立即开始西罗莫司治疗，并计划进行连续影像学密切随访。该病例突出了LAM的诊断挑战，尤其是在血管内皮生长因子-D（VEGF-D）水平较低的情况下，并强调了胸膜固定术在特定患者中的治疗作用。早期识别和个性化管理对于优化患者预后至关重要。
