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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
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 are virtually identical
- Lymphangioleiomyomatosis (LAM): Disease characterized by abnormal proliferation of LAM cells in lungs & thoracic/retroperitoneal lymphatics
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
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
- Bhattacharya I et al: Oxygen-enhanced functional lung imaging using a contemporary 0.55 T MRI system. NMR Biomed. 34(8):e4562, 2021
- Daccord C et al: Effect of everolimus on multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis complex. Respir Med Case Rep. 31:101310, 2020
- Walkup LL et al: Cyst ventilation heterogeneity and alveolar airspace dilation as early disease markers in lymphangioleiomyomatosis. Ann Am Thorac Soc. 16(8):1008-16, 2019
- Boronat S et al: Less common manifestations in TSC. Am J Med Genet C Semin Med Genet. 178(3):348-54, 2018
- Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
- Manoukian SB et al: Comprehensive imaging manifestations of tuberous sclerosis. AJR Am J Roentgenol. 204(5):933-43, 2015
- Tobino K et al: Computed tomographic features of lymphangioleiomyomatosis: evaluation in 138 patients. Eur J Radiol. 84(3):534-41, 2015
- Gupta R et al: Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology. 47(3):106-17, 2014
- Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
- 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
- Mavroudi M et al: Lymphangioleiomyomatosis: current and future. J Thorac Dis. 5(1):74-9, 2013
- 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
- Meraj R et al: Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Semin Respir Crit Care Med. 33(5):486-97, 2012
- Yao J et al: CT grading of lung disease in lymphangioleiomyomatosis. AJR Am J Roentgenol. 199(4):787-93, 2012
- Adriaensen ME et al: Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex. Clin Radiol. 66(7):625-8, 2011
- McCormack FX et al: Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 364(17):1595-606, 2011
- Avila NA et al: Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features. Radiology. 242(1):277-85, 2007
- Crino PB et al: The tuberous sclerosis complex. N Engl J Med. 355(13):1345-56, 2006
- Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005
- Pallisa E et al: Lymphangioleiomyomatosis: pulmonary and abdominal findings with pathologic correlation. Radiographics. 22 Spec No:S185-98, 2002
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
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 are virtually identical
- Lymphangioleiomyomatosis (LAM): Disease characterized by abnormal proliferation of LAM cells in lungs & thoracic/retroperitoneal lymphatics
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
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
- Bhattacharya I et al: Oxygen-enhanced functional lung imaging using a contemporary 0.55 T MRI system. NMR Biomed. 34(8):e4562, 2021
- Daccord C et al: Effect of everolimus on multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis complex. Respir Med Case Rep. 31:101310, 2020
- Walkup LL et al: Cyst ventilation heterogeneity and alveolar airspace dilation as early disease markers in lymphangioleiomyomatosis. Ann Am Thorac Soc. 16(8):1008-16, 2019
- Boronat S et al: Less common manifestations in TSC. Am J Med Genet C Semin Med Genet. 178(3):348-54, 2018
- Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
- Manoukian SB et al: Comprehensive imaging manifestations of tuberous sclerosis. AJR Am J Roentgenol. 204(5):933-43, 2015
- Tobino K et al: Computed tomographic features of lymphangioleiomyomatosis: evaluation in 138 patients. Eur J Radiol. 84(3):534-41, 2015
- Gupta R et al: Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology. 47(3):106-17, 2014
- Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
- 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
- Mavroudi M et al: Lymphangioleiomyomatosis: current and future. J Thorac Dis. 5(1):74-9, 2013
- 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
- Meraj R et al: Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Semin Respir Crit Care Med. 33(5):486-97, 2012
- Yao J et al: CT grading of lung disease in lymphangioleiomyomatosis. AJR Am J Roentgenol. 199(4):787-93, 2012
- Adriaensen ME et al: Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex. Clin Radiol. 66(7):625-8, 2011
- McCormack FX et al: Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 364(17):1595-606, 2011
- Avila NA et al: Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features. Radiology. 242(1):277-85, 2007
- Crino PB et al: The tuberous sclerosis complex. N Engl J Med. 355(13):1345-56, 2006
- Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005
- Pallisa E et al: Lymphangioleiomyomatosis: pulmonary and abdominal findings with pathologic correlation. Radiographics. 22 Spec No:S185-98, 2002
