Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Pulmonary lymphangioleiomyomatosis (LAM) with extrapulmonary lymphangioleiomyoma
- Radiologic Findings
- Chest CT shows diffuse thin-walled cysts in both lungs, sparing the upper posterior lung fields. Low density lesions with weak enhancement are revealed in the retrocrural and retroperitoneal regions. On FDG-PET, there is no appreciable FDG uptake by masses. Retrocrural lymphangioleiomyoma is identified after surgical resection, and pulmonary lesions by VATS biopsy.
- Brief Review
- Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease that affects women exclusively, typically during their reproductive years. LAM is characterized by the abnormal proliferation of smooth muscle cells (LAM cells) in the lungs, and thoracic and retroperitoneal lymphatics. The primary target of LAM is the chest. However, extrapulmonary LAM rarely occurs with or without subsequent involvement of the lung.
21% of pulmonary LAM patients had extrapulmonary lymphangioleiomyomas. Avila et al. observed a positive trend between the presence of lymphangioleiomyomas and the severity of lung disease, with the probability of lymphangioleiomyomas increasing severity of the lung disease (p=0.12).
Diurnal variation of lymphangioleiomyomas with normal morning pressure and increasing size of mass in the afternoon resulting from increased chyle during the day after meal and exercise provides useful radiographic features in distinguishing lymphangioleiomyomas (when it has thick and irregular wall, or solid), from lymphoma, solid lymphatic mass, and sarcoma.
- References
- 1.Avil NA, Bechtle J, Dwyer AJ, Ferrans VJ, Moss J. Lymphangioleiomyomatosis: CT of Diurnal variation of lymphangioleiomyomas. Radiology 2001;221:415-421
2.Matsui K, Tatsuguchi A, Valencia J, Yu Zx, Bechtle J, Beasley MB, Avila N, Travis WD, Moss J, Ferrans VJ. Extrapulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22 cases. Hum Pathol. 2000;31(10):1242-1248
- Keywords
- Lung, Mediastinum, Interstitial lung disease, LAM, ILD,