Regression of Pulmonary Lymphangioleiomyomatosis (PLAM)-associated Retroperitoneal Angiomyolipoma Post–Lung Transplantation With Rapamycin Treatment

Morton, Judith M.; McLean, Catriona; Booth, Stephen S.; Snell, Gregory I.; Whitford, Helen M.

doi:10.1016/j.healun.2008.01.005

« Previous Next »The Journal of Heart and Lung Transplantation
Volume 27, Issue 4 , Pages 462-465, April 2008

Regression of Pulmonary Lymphangioleiomyomatosis (PLAM)-associated Retroperitoneal Angiomyolipoma Post–Lung Transplantation With Rapamycin Treatment

Judith M. Morton, MBBS, FRACP
- Lung Transplant Service, Department of Allergy, Immunology and Respiratory Medicine, Monash University, The Alfred Hospital, Prahran, Victoria, Australia
- Department 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.
Catriona McLean, MBBS, FRCPA
- Department of Anatomical Pathology, Monash University, The Alfred Hospital, Prahran, Victoria, Australia
Stephen S. Booth, MB, ChB
- Department of Radiology, Monash University, The Alfred Hospital, Prahran, Victoria, Australia
Gregory I. Snell, MBBS, FRACP
- Lung Transplant Service, Department of Allergy, Immunology and Respiratory Medicine, Monash University, The Alfred Hospital, Prahran, Victoria, Australia
- Department of Medicine, Monash University, The Alfred Hospital, Prahran, Victoria, Australia.
Helen M. Whitford, MBBS, FRACP
- Lung Transplant Service, Department of Allergy, Immunology and Respiratory Medicine, Monash University, The Alfred Hospital, Prahran, Victoria, Australia
- Department of Medicine, Monash University, The Alfred Hospital, Prahran, Victoria, Australia.

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.