Background: Lymphangioleiomyomatosis is a rare condition in women of childbearing age that affects the lymphatic system, giving rise to blockage of the thoracic duct with chylothorax and chyloascites. The diagnosis is often delayed because the characteristic muscular proliferation may be missed by the pathologist.
Case: A 29-year-old female presented with lymphangioleiomyomatosis and bilaterally dermoid ovarian cysts. She had enlarged paraaortal and supraclavicular "lymph nodes" and pleural chylothorax on the left side. Fine needle aspiration cytology from the supraclavicular "lymph node" yielded clear, watery fluid containing groups of relatively large monomorphic spindle cells. The final diagnosis was possible after histopathologic examination of an excised paratubar lymph node.
Conclusion: Lymphangioleiomyomatosis can be suggested by means of ultrasonography and fine needle aspiration cytology.