Cytologic findings of lymphangioleiomyomatosis in pleural effusion:a casereport
BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare disease occurring almostexclusively in females of reproductive age. Patients usually present with insidious and progressivedyspnea. Episodes of hemoptysis, pneumothorax, and chylothorax may occur, and the patientprogresses to eventual respiratory failure and death. The characteristic pathologic finding isproliferation of immature smooth muscle cells (LAM cells) in the lungs, lymphatics and lymph nodesof thorax and abdomen/ retroperitoneum. CASE: A 47-year-old woman with a 1-year history of LAMdiagnosed on iliac lymph node biopsy presented with progressive dyspnea and pleural effusion. Achest tube was placed. The collected pleural fluid, which represented chylothorax, yielded cohesiveclusters of cells consisting of 2 cell populations: an outer, discontinuous layer of flattened cellsand an inner portion of ovoid spindle cells. By immunohistochemistry the inner cells stained withsmooth muscle actin and were negative for keratin. CONCLUSION: The characteristic constellation ofclinical findings and distinctive cytology in conjunction with immunohistochemistry staining canrender the diagnosis of LAM in effusions.
Tynski,Z　Eisenberg,R
Department of Pathology, University Hospitals of Cleveland, Case WesternReserve University, Cleveland, Ohio 44106, USA.
Lymphangioleiomyomatosis　 findings　 Pleural Effusion　 Cytologic Thorax
