Cytologic finding of chyloascites in lymphangioleiomyomatosis - A case report

BACKGROUND: Lymphangioleiomyomatosis is a rare disease, histologically char acterized by an abnormal proliferation of smooth muscle around the lymphati cs. Lung is the most common site of involvement, and patients usually prese nt with dyspnea, chest pain, and cough. Chylous pleural effusion and ascite s occasionally appear during the course of the disease. There are only a fe w reports on the cytologic findings in this disease. To our knowledge, the cytologic findings of chylous pleural effusion and chyloascites have not be en reported before. CASE: A 23-year-old female presented with chylothorax, chyloascites and a r etroperitoneal mass. Cytologic examination of chylous pleural effusion and chyloascites revealed numerous cohesive and thick clusters of cells with a high nuclear/cytoplasmic ratio, oval nuclei and slightly increased chromati n content. Mitosis and necrosis were not observed. Exploratory laparotomy a nd transbronchial lung biopsy were performed, and the histologic diagnosis was lymphangioleiomyomatosis involving the retroperitoneal lymph nodes, ute rine fundus and lungs. Immunohistochemistry showed that the characteristic clusters in chylous fluids were positive for alpha -mooth muscle actin. CONCLUSION: A diagnosis of lymphangioleiomyomatosis is possible from cytolo gic findings of effusions with the aid of clinical findings.