Cancer Control. 2006 Oct;13(4):276-85. doi: 10.1177/107327480601300405.


Background: Lymphangioleiomyomatosis (LAM) is a rare disease that is characterized by proliferation of abnormal smooth muscle-like cells (LAM cells), which leads to the formation of lung cysts, fluid-filled cystic structures in the axial lymphatics, and abdominal tumors. It primarily affects women.

Methods: The authors present a review of large series, registries, and protocols to highlight the prevalence, pathology, clinical features, diagnosis, and treatment options for patients with LAM.

Results: LAM commonly presents with progressive breathlessness or with recurrent pneumothorax, chylothorax, or sudden abdominal hemorrhage. Computed tomography (CT) scans show numerous thin-walled cysts throughout the lungs, abdominal angiomyolipomas, and lymphangioleiomyomas. Pulmonary function tests show decreased forced expiratory volume in 1 second (FEV1) and diffusion capacity for carbon monoxide (DLCO). Exercise testing shows gas-exchange abnormalities, ventilatory limitation, and hypoxemia that may occur with near-normal lung function.

Conclusions: No effective treatment currently exists for this progressive disorder. However, recent progress in cancer and smooth muscle cell biology and a better understanding of the factors regulating angiogenesis and lymphangiogenesis may provide a foundation for the development of new therapeutic strategies.

Publication types

  • Review

MeSH terms

  • Angiomyolipoma / diagnosis
  • Angiomyolipoma / epidemiology
  • Angiomyolipoma / therapy
  • Disease Progression
  • Exercise Tolerance
  • Humans
  • Lung Neoplasms* / diagnosis
  • Lung Neoplasms* / epidemiology
  • Lung Neoplasms* / pathology
  • Lung Neoplasms* / physiopathology
  • Lung Neoplasms* / therapy
  • Lymphangioleiomyomatosis* / diagnosis
  • Lymphangioleiomyomatosis* / epidemiology
  • Lymphangioleiomyomatosis* / pathology
  • Lymphangioleiomyomatosis* / physiopathology
  • Lymphangioleiomyomatosis* / therapy
  • Neoplasm Staging
  • Prevalence
  • Respiratory Function Tests
  • Sex Factors
  • Tomography, X-Ray Computed