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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
- Rare neoplastic cystic lung disease; proliferation of atypical smooth muscle cells
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
- 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
- Crivelli P et al: Role of thoracic imaging in the management of lymphangioleiomyomatosis. Respir Med. 157:14-20, 2019
- 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
- Nijmeh J et al: Emerging biomarkers of lymphangioleiomyomatosis. Expert Rev Respir Med. 12(2):95-102, 2018
- Johnson SR et al: Lymphangioleiomyomatosis. Clin Chest Med. 37(3):389-403, 2016
- 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
- Moir LM: Lymphangioleiomyomatosis: current understanding and potential treatments. Pharmacol Ther. 158:114-24, 2016
- Ferreira Francisco FA et al: Multiple cystic lung disease. Eur Respir Rev. 24(138):552-64, 2015
- Gillott M et al: Imaging of cystic lung disease. Semin Roentgenol. 50(1):23-30, 2015
- Gupta N et al: Diffuse cystic lung disease. Part I. Am J Respir Crit Care Med. 191(12):1354-66, 2015
- Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
- Radzikowska E: Lymphangioleiomyomatosis: New treatment perspectives. Lung. 193(4):467-75, 2015
- Taveira-DaSilva AM et al: Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol. 7:249-57, 2015
- Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
- Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005
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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
- Rare neoplastic cystic lung disease; proliferation of atypical smooth muscle cells
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
- 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
- Crivelli P et al: Role of thoracic imaging in the management of lymphangioleiomyomatosis. Respir Med. 157:14-20, 2019
- 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
- Nijmeh J et al: Emerging biomarkers of lymphangioleiomyomatosis. Expert Rev Respir Med. 12(2):95-102, 2018
- Johnson SR et al: Lymphangioleiomyomatosis. Clin Chest Med. 37(3):389-403, 2016
- 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
- Moir LM: Lymphangioleiomyomatosis: current understanding and potential treatments. Pharmacol Ther. 158:114-24, 2016
- Ferreira Francisco FA et al: Multiple cystic lung disease. Eur Respir Rev. 24(138):552-64, 2015
- Gillott M et al: Imaging of cystic lung disease. Semin Roentgenol. 50(1):23-30, 2015
- Gupta N et al: Diffuse cystic lung disease. Part I. Am J Respir Crit Care Med. 191(12):1354-66, 2015
- Harari S et al: The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 46(5):1471-85, 2015
- Radzikowska E: Lymphangioleiomyomatosis: New treatment perspectives. Lung. 193(4):467-75, 2015
- Taveira-DaSilva AM et al: Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol. 7:249-57, 2015
- Trotman-Dickenson B: Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 83(1):39-46, 2014
- Abbott GF et al: From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3):803-28, 2005
