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Lymphangioleiomyomatosis
Melissa L. Rosado-de-Christenson, MD, FACR; Takeshi Johkoh, MD, PhD
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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

                • Proliferation of neoplastic smooth muscle-like cells
                  • Sporadic LAM (S-LAM)
                  • LAM associated with tuberous sclerosis (TSC-LAM)
                    • Neurocutaneous syndrome of multiorgan hamartomas, seizures, and cognitive disorders
                  • TSC-LAM is 5-10 times more common than S-LAM

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT 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. Johnson SR et al: Lymphangioleiomyomatosis. Clin Chest Med. 37(3):389-403, 2016
                                            2. Moir LM: Lymphangioleiomyomatosis: Current understanding and potential treatments. Pharmacol Ther. 158:114-24, 2016
                                            3. Ferreira Francisco FA et al: Multiple cystic lung disease. Eur Respir Rev. 24(138):552-64, 2015
                                            4. Gillott M et al: Imaging of cystic lung disease. Semin Roentgenol. 50(1):23-30, 2015
                                            5. Gupta N et al: Diffuse cystic lung disease. Part I. Am J Respir Crit Care Med. 191(12):1354-66, 2015
                                            6. Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
                                            7. Radzikowska E: Lymphangioleiomyomatosis: New treatment perspectives. Lung. 193(4):467-75, 2015
                                            8. Taveira-DaSilva AM et al: Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol. 7:249-57, 2015
                                            9. Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
                                            10. 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

                                                          • Proliferation of neoplastic smooth muscle-like cells
                                                            • Sporadic LAM (S-LAM)
                                                            • LAM associated with tuberous sclerosis (TSC-LAM)
                                                              • Neurocutaneous syndrome of multiorgan hamartomas, seizures, and cognitive disorders
                                                            • TSC-LAM is 5-10 times more common than S-LAM

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT 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. Johnson SR et al: Lymphangioleiomyomatosis. Clin Chest Med. 37(3):389-403, 2016
                                                                                      2. Moir LM: Lymphangioleiomyomatosis: Current understanding and potential treatments. Pharmacol Ther. 158:114-24, 2016
                                                                                      3. Ferreira Francisco FA et al: Multiple cystic lung disease. Eur Respir Rev. 24(138):552-64, 2015
                                                                                      4. Gillott M et al: Imaging of cystic lung disease. Semin Roentgenol. 50(1):23-30, 2015
                                                                                      5. Gupta N et al: Diffuse cystic lung disease. Part I. Am J Respir Crit Care Med. 191(12):1354-66, 2015
                                                                                      6. Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
                                                                                      7. Radzikowska E: Lymphangioleiomyomatosis: New treatment perspectives. Lung. 193(4):467-75, 2015
                                                                                      8. Taveira-DaSilva AM et al: Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol. 7:249-57, 2015
                                                                                      9. Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
                                                                                      10. Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005