link
Bookmarks
Lymphangioleiomyomatosis
A. Carlson Merrow, Jr., MD, FAAP; David B. Larson, MD, MBA
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
10
5
12

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Lymphangiomyomatosis
          • Definitions

            • Lymphangioleiomyomatosis (LAM): Disease characterized by abnormal proliferation of LAM cells in lungs & thoracic/retroperitoneal lymphatics
              • Occurs almost exclusively in women of childbearing age
            • 2 main forms
              • Tuberous sclerosis complex-associated LAM (TSC-LAM)
              • Sporadic LAM (S-LAM)
            • Radiologic/pathologic findings of TSC-LAM & S-LAM virtually identical

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        • Microscopic Features

                          • Extrapulmonary Findings

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
                                      2. Manoukian SB et al: Comprehensive imaging manifestations of tuberous sclerosis. AJR Am J Roentgenol. 204(5):933-43, 2015
                                      3. Gupta R et al: Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology. 47(3):106-17, 2014
                                      4. Tobino K et al: Computed tomographic features of lymphangioleiomyomatosis: Evaluation in 138 patients. Eur J Radiol. ePub, 2014
                                      5. Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
                                      6. Franz DN: Everolimus in the treatment of subependymal giant cell astrocytomas, angiomyolipomas, and pulmonary and skin lesions associated with tuberous sclerosis complex. Biologics. 7:211-21, 2013
                                      7. Mavroudi M et al: Lymphangioleiomyomatosis: current and future. J Thorac Dis. 5(1):74-9, 2013
                                      8. McCormack FX et al: Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. Am J Respir Crit Care Med. 186(12):1210-2, 2012
                                      9. Meraj R et al: Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Semin Respir Crit Care Med. 33(5):486-97, 2012
                                      10. Yao J et al: CT grading of lung disease in lymphangioleiomyomatosis. AJR Am J Roentgenol. 199(4):787-93, 2012
                                      11. Adriaensen ME et al: Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex. Clin Radiol. 66(7):625-8, 2011
                                      12. McCormack FX et al: Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 364(17):1595-606, 2011
                                      13. Avila NA et al: Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features. Radiology. 242(1):277-85, 2007
                                      14. Crino PB et al: The tuberous sclerosis complex. N Engl J Med. 355(13):1345-56, 2006
                                      15. Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005
                                      16. Pallisa E et al: Lymphangioleiomyomatosis: pulmonary and abdominal findings with pathologic correlation. Radiographics. 22 Spec No:S185-98, 2002
                                      Related Anatomy
                                      Loading...
                                      Related Differential Diagnoses
                                      Loading...
                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Synonyms

                                                • Lymphangiomyomatosis
                                              • Definitions

                                                • Lymphangioleiomyomatosis (LAM): Disease characterized by abnormal proliferation of LAM cells in lungs & thoracic/retroperitoneal lymphatics
                                                  • Occurs almost exclusively in women of childbearing age
                                                • 2 main forms
                                                  • Tuberous sclerosis complex-associated LAM (TSC-LAM)
                                                  • Sporadic LAM (S-LAM)
                                                • Radiologic/pathologic findings of TSC-LAM & S-LAM virtually identical

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            • Microscopic Features

                                                              • Extrapulmonary Findings

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
                                                                          2. Manoukian SB et al: Comprehensive imaging manifestations of tuberous sclerosis. AJR Am J Roentgenol. 204(5):933-43, 2015
                                                                          3. Gupta R et al: Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology. 47(3):106-17, 2014
                                                                          4. Tobino K et al: Computed tomographic features of lymphangioleiomyomatosis: Evaluation in 138 patients. Eur J Radiol. ePub, 2014
                                                                          5. Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
                                                                          6. Franz DN: Everolimus in the treatment of subependymal giant cell astrocytomas, angiomyolipomas, and pulmonary and skin lesions associated with tuberous sclerosis complex. Biologics. 7:211-21, 2013
                                                                          7. Mavroudi M et al: Lymphangioleiomyomatosis: current and future. J Thorac Dis. 5(1):74-9, 2013
                                                                          8. McCormack FX et al: Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. Am J Respir Crit Care Med. 186(12):1210-2, 2012
                                                                          9. Meraj R et al: Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Semin Respir Crit Care Med. 33(5):486-97, 2012
                                                                          10. Yao J et al: CT grading of lung disease in lymphangioleiomyomatosis. AJR Am J Roentgenol. 199(4):787-93, 2012
                                                                          11. Adriaensen ME et al: Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex. Clin Radiol. 66(7):625-8, 2011
                                                                          12. McCormack FX et al: Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 364(17):1595-606, 2011
                                                                          13. Avila NA et al: Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features. Radiology. 242(1):277-85, 2007
                                                                          14. Crino PB et al: The tuberous sclerosis complex. N Engl J Med. 355(13):1345-56, 2006
                                                                          15. Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005
                                                                          16. Pallisa E et al: Lymphangioleiomyomatosis: pulmonary and abdominal findings with pathologic correlation. Radiographics. 22 Spec No:S185-98, 2002