Successful treatment of a patient with chyluria due to lymphangioleiomyomatosis using sirolimus

Abstract

Lymphangioleiomyomatosis (LAM) is a rare and progressive neoplastic disease of young woman, characterized by the proliferation of abnormal smooth muscle-like cells (LAM cells) in the lungs and axial lymphatics. A 44-year-old woman was referred to our hospital because pleural effusion was detected during a health checkup. She had chylothorax, chylous ascites, and chyluria, and her computed tomography scan showed a solid tumor in the pelvis. Surgical biopsy was performed; she was diagnosed as having LAM. We could not control the fluid collection and chyluria using standard medical treatments. Therefore, we chose to administer sirolimus, and her symptoms dramatically improved. The mechanism of chyluria presumably involved LAM cell infiltrates in the ureter via the lymphatic vessel flow, which causes LAM to develop because of ureter wall exposure.

Central Lymphatic Imaging in Adults with Spontaneous Chyluria

“…Therefore, previous treatment methods, including silver nitrate instillation into the kidneys and soft tissue surgery ligation around the renal hilum, have been based on empirical experience rather than addressing the underlying nature of the damage. 5,6 With recent advancements in imaging diagnostics of the lymphatic system, a more in-depth study of the lymphatic circulation in chyluria patients has become possible. This allows for accurate visualization of the lymphatic branches entering the renal hilum, providing valuable insights for surgeons to focus on suturing or interventional radiologist for embolization of uro-lymphatic fistulas.…”