Regression of pulmonary lymphangioleiomyomatosis (PLAM)-associated retroperitoneal angiomyolipoma post-lung transplantation with rapamycin treatment

J Heart Lung Transplant. 2008 Apr;27(4):462-5. doi: 10.1016/j.healun.2008.01.005.

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Angiomyolipoma / diagnosis
  • Angiomyolipoma / drug therapy*
  • Antibiotics, Antineoplastic / administration & dosage
  • Antibiotics, Antineoplastic / therapeutic use*
  • Biopsy
  • Dose-Response Relationship, Drug
  • Female
  • Humans
  • Lung Neoplasms / surgery*
  • Lung Transplantation*
  • Lymphangioleiomyomatosis / surgery*
  • Neoplasms, Second Primary*
  • Postoperative Period
  • Retroperitoneal Neoplasms / diagnosis
  • Retroperitoneal Neoplasms / drug therapy*
  • Sirolimus / administration & dosage
  • Sirolimus / therapeutic use*
  • Surgery, Computer-Assisted
  • Tomography, X-Ray Computed

Substances

  • Antibiotics, Antineoplastic
  • Sirolimus