title: Regression of pulmonary lymphangioleiomyomatosis (PLAM)-associated retroperitoneal angiomyolipoma post-lung transplantation with rapamycin treatment.
abstract: 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.
authors: Lung Transplant Service, Department of Allergy, Immunology and Respiratory Medicine, Monash University, The Alfred Hospital, Prahran, Victoria, Australia. judy.morton@bigpond.com
authors: Judith M
authors: JM
authors: Morton
authors: Catriona
authors: C
authors: McLean
authors: Stephen S
authors: SS
authors: Booth
authors: Gregory I
authors: GI
authors: Snell
authors: Helen M
authors: HM
authors: Whitford
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000175:diagnosis / Q000188:drug therapy*; D000903:Antibiotics, Antineoplastic / Q000008:administration & dosage / Q000627:therapeutic use*; D001706:Biopsy; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000601:surgery*; D016040:Lung Transplantation; D018192:Lymphangioleiomyomatosis / Q000601:surgery*; D016609:Neoplasms, Second Primary; D011184:Postoperative Period; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000188:drug therapy*; D020123:Sirolimus / Q000008:administration & dosage / Q000627:therapeutic use*; D025321:Surgery, Computer-Assisted; D014057:Tomography, X-Ray Computed
chemical_list: D000903:Antibiotics, Antineoplastic; D020123:Sirolimus