Pelvic Presentation Prior to Onset of Pulmonary Symptom in a Patient with Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is an extremely rare disease, unfamiliar to many clinicians, possibly resulting in a delayed or missed diagnosis. LAM most frequently involves the lung and rarely involves extrapulmonary sites. The most common presentations are progressive exertional dyspnea, spontaneous pneumothorax, and less frequently, chylothorax. We report a case of LAM in a 26-year-old, nonsmoking, married woman who presented with pelvic pain due to a pelvic mass (16×11 cm). The huge mass was surgically excised and shown histologically a LAM. Chest X-ray findings were normal at the time of presentation. Two years later, a chest X-ray and chest computed tomography (CT) scan showed characteristic features of LAM. Three years later, patient underwent right single lung transplantation because of end-stage LAM. After one year, the patient died of bronchiolitis obliterans syndrome. The case illustrates that LAM can involve extrapulmonary sites and demonstrates that high resolution CT (HRCT) evaluation of the lungs should be routinely performed for early detection of asymptomatic pulmonary LAM with extrapulmonary manifestations.

並列關鍵字

lymphangioleiomyomatosis ； angiomyolipoma ； progressive exertional dyspnea ； proliferation of smooth muscle cells

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