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Unilateral lymphangioleiomyomatosis
Audrey Eleanor Therese Wenaden1, Susan Jennifer Copley
1Department of Radiology, Hammersmith Hospital, London, UK.
Journal of Thoracic Imaging
|August 4, 2005
Summary
Pulmonary involvement in Tuberous Sclerosis Complex (TSC) mimics Lymphangioleiomyomatosis (LAM). This case highlights atypical, focal unilateral lung disease in TSC, expanding understanding of these related conditions.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- Tuberous Sclerosis Complex (TSC) and Lymphangioleiomyomatosis (LAM) share pulmonary manifestations.
- High-resolution computed tomography (HRCT) typically shows bilateral cysts in TSC lung disease.
Observation:
- This report details an unusual case of biopsy-proven, focal, unilateral lung disease in TSC.
- Atypical presentations of TSC and sporadic LAM are discussed.
Findings:
- The pulmonary findings in TSC can resemble LAM, including cystic changes and increased lung volumes.
- Histological examination of nodules in TSC may reveal proliferated type II pneumocytes.
Implications:
- This case broadens the spectrum of known pulmonary involvement in TSC.
- Understanding atypical presentations is crucial for accurate diagnosis and management of TSC and LAM.