A case of lymphangioleiomyomatosis diagnosed by EUS-FNA

Background : Lymphangioleiomyomatosis (LAM) is a systemic disease characterized by proliferation of smooth muscle-like tumor cells in the lungs and axial lymph nodes. It is a rather rare disease, and no effective treatment has been established yet. Herein, we report a case of LAM diagnosed by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA).Case : A woman in her 50 s was found to have high serum CA19-9 levels and multiple lymphadenopathy around the abdominal aorta. She had no significant past medical history or respiratory symptoms. EUS-FNA of the affected lymph nodes was performed under the suspicion of lymph node metastasis, and cytological examination revealed a strongly bound cluster of spindle-shaped cells arranged in fascicles. The cells had oval-to-spindle-shaped nuclei with fine granular chromatin and intranuclear cytoplasmic inclusion bodies. Histological examination of biopsy specimens revealed similar findings. Therefore, we report this case in which the diagnosis of LAM was confirmed by immunohistochemical analysis.Conclusion : To establish the diagnosis of LAM from cell findings other than LAM cell clusters in fluid samples from chylothorax, more case reports, confirmation of clinical findings and detailed cell observation, and comparison with the features of diseases included in the differential diagnosis are necessary. Immunocytochemical staining may be also important.