PULMONARY LYMPHANGIOMYOMATOSIS DIAGNOSED BY EFFUSION CYTOLOGY - A CASE-REPORT

BACKGROUND: Pulmonary lymphangiomyomatosis (P-LAM) is a rare disease, characterized by an abnormal proliferation of smooth muscle throughout the lung and occurring in females of reproductive age. The typical cl inical picture in P-LAM is progressive dyspnea, often punctuated by ep isodes of chylothorax or pneumothorax, with eventual death from respir atory failure. The definitive diagnosis is usually performed on open l ung biopsies. CASE: The cytologic findings in a chylous pleural effusi on from a patient with P-LAM ave presented. The effusion contained an abundance of globular cell clusters composed of two distinct cell type s, inner cove spindle cells and surface flat cells. Immunocytochemical examination revealed that the core cells were derived from immature s mooth muscle cells and the surface cells from endothelium. Histologic examination of the excised specimen showed the typical findings of P-L AM, and the dilated peripheral lymphatics contained cell clusters simi lar to those in the pleural effusion. The cell clusters in the lymphat ics in the P-LAM lung tissue appeared to have ruptured into the thorac ic cavity. CONCLUSION: Cell clusters seem to be pathognomonic of P-LAM , and cytologic examination can obviate the need to perform open lung biopsy to confirm the diagnosis.