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Lymphangioleiomyomatosis
Melissa L. Rosado de Christenson, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Lymphangioleiomyomatosis (LAM)
                • Tuberous sclerosis complex (TSC)
              • Definitions

                • Rare neoplastic cystic lung disease; proliferation of atypical smooth muscle cells
                  • Sporadic LAM (S-LAM)
                  • LAM associated with tuberous sclerosis (TSC-LAM)
                    • TSC: Neurocutaneous syndrome of multiorgan hamartomas, seizures, and cognitive disorders

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • Other Modality Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Kim C et al: Effects of sirolimus in lymphangioleiomyomatosis patients on lung cysts and pulmonary function: long-term follow-up observational study. Eur Radiol. 30(2):735-43, 2020
                                              2. Crivelli P et al: Role of thoracic imaging in the management of lymphangioleiomyomatosis. Respir Med. 157:14-20, 2019
                                              3. Park HJ et al: Differentiation between lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome: analysis of pulmonary cysts on CT images. AJR Am J Roentgenol. 212(4):766-72, 2019
                                              4. Nijmeh J et al: Emerging biomarkers of lymphangioleiomyomatosis. Expert Rev Respir Med. 12(2):95-102, 2018
                                              5. Johnson SR et al: Lymphangioleiomyomatosis. Clin Chest Med. 37(3):389-403, 2016
                                              6. McCormack FX et al: Official American Thoracic Society/Japanese Respiratory Society clinical practice guidelines: lymphangioleiomyomatosis diagnosis and management. Am J Respir Crit Care Med. 194(6):748-61, 2016
                                              7. Moir LM: Lymphangioleiomyomatosis: current understanding and potential treatments. Pharmacol Ther. 158:114-24, 2016
                                              8. Ferreira Francisco FA et al: Multiple cystic lung disease. Eur Respir Rev. 24(138):552-64, 2015
                                              9. Gillott M et al: Imaging of cystic lung disease. Semin Roentgenol. 50(1):23-30, 2015
                                              10. Gupta N et al: Diffuse cystic lung disease. Part I. Am J Respir Crit Care Med. 191(12):1354-66, 2015
                                              11. Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
                                              12. Radzikowska E: Lymphangioleiomyomatosis: New treatment perspectives. Lung. 193(4):467-75, 2015
                                              13. Taveira-DaSilva AM et al: Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol. 7:249-57, 2015
                                              14. Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
                                              15. Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Lymphangioleiomyomatosis (LAM)
                                                            • Tuberous sclerosis complex (TSC)
                                                          • Definitions

                                                            • Rare neoplastic cystic lung disease; proliferation of atypical smooth muscle cells
                                                              • Sporadic LAM (S-LAM)
                                                              • LAM associated with tuberous sclerosis (TSC-LAM)
                                                                • TSC: Neurocutaneous syndrome of multiorgan hamartomas, seizures, and cognitive disorders

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • Other Modality Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Kim C et al: Effects of sirolimus in lymphangioleiomyomatosis patients on lung cysts and pulmonary function: long-term follow-up observational study. Eur Radiol. 30(2):735-43, 2020
                                                                                          2. Crivelli P et al: Role of thoracic imaging in the management of lymphangioleiomyomatosis. Respir Med. 157:14-20, 2019
                                                                                          3. Park HJ et al: Differentiation between lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome: analysis of pulmonary cysts on CT images. AJR Am J Roentgenol. 212(4):766-72, 2019
                                                                                          4. Nijmeh J et al: Emerging biomarkers of lymphangioleiomyomatosis. Expert Rev Respir Med. 12(2):95-102, 2018
                                                                                          5. Johnson SR et al: Lymphangioleiomyomatosis. Clin Chest Med. 37(3):389-403, 2016
                                                                                          6. McCormack FX et al: Official American Thoracic Society/Japanese Respiratory Society clinical practice guidelines: lymphangioleiomyomatosis diagnosis and management. Am J Respir Crit Care Med. 194(6):748-61, 2016
                                                                                          7. Moir LM: Lymphangioleiomyomatosis: current understanding and potential treatments. Pharmacol Ther. 158:114-24, 2016
                                                                                          8. Ferreira Francisco FA et al: Multiple cystic lung disease. Eur Respir Rev. 24(138):552-64, 2015
                                                                                          9. Gillott M et al: Imaging of cystic lung disease. Semin Roentgenol. 50(1):23-30, 2015
                                                                                          10. Gupta N et al: Diffuse cystic lung disease. Part I. Am J Respir Crit Care Med. 191(12):1354-66, 2015
                                                                                          11. Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
                                                                                          12. Radzikowska E: Lymphangioleiomyomatosis: New treatment perspectives. Lung. 193(4):467-75, 2015
                                                                                          13. Taveira-DaSilva AM et al: Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol. 7:249-57, 2015
                                                                                          14. Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
                                                                                          15. Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005