Regression of Pulmonary Lymphangioleiomyomatosis (PLAM)-associated Retroperitoneal Angiomyolipoma Post–Lung Transplantation With Rapamycin Treatment
Received 16 September 2007; received in revised form 27 December 2007; accepted 2 January 2008.
Pulmonary lymphangioleiomyomatosis (PLAM) is an indication for lung transplantation (LTx). Angiomyolipomas occur in approximately 50% to 60% of patients with PLAM. We describe a patient presenting with hemoptysis post-LTx for PLAM. Computed tomography (CT) scan demonstrated no pulmonary abnormality, but identified a retroperitoneal mass confirmed as angiomyolipoma by CT-guided core biopsy. Based on experimental work that rapamycin may inhibit angiomyolipoma cells, we commenced the patient on low-dose rapamycin. She had no adverse reactions and follow-up CT scan after 7 months demonstrated almost complete resolution of the tumor. This suggests a role for rapamycin in routine post-LTx immunosuppression for PLAM.
aLung Transplant Service, Department of Allergy, Immunology and Respiratory Medicine, Monash University, The Alfred Hospital, Prahran, Victoria, Australia
bDepartment of Anatomical Pathology, Monash University, The Alfred Hospital, Prahran, Victoria, Australia
cDepartment of Radiology, Monash University, The Alfred Hospital, Prahran, Victoria, Australia
dDepartment of Medicine, Monash University, The Alfred Hospital, Prahran, Victoria, Australia.
Reprint requests: Judith M. Morton, FRACP, Department of Respiratory and Sleep Medicine, Monash Medical Centre, 246 Clayton Rd, Clayton, Victoria 3168, Australia.
ABSTRACT