Thoracic Computed Tomographic Manifestations of Tuberous Sclerosis in Adults

A 47-year-old woman with tuberous sclerosis. (A) Transaxial chest computed tomography (CT) image at the level of the carina viewed with lung windows shows diffuse replacement of the lung parenchyma by variably-sized thin-walled cysts, consistent with lymphangioleiomyomatosis (LAM). (B) Coronal reformation CT image viewed with lung windows shows a uniform craniocaudal distribution of LAM. (C) Transaxial intravenous contrast-enhanced CT image at the level of the kidneys shows bilateral, large, fat-containing renal angiomyolipomas. Note the complicating bilateral retroperitoneal hemorrhage (arrows).

A 39-year-old woman with tuberous sclerosis. (A) and (B) Transaxial chest computed tomographic (CT) images viewed with lung windows at the level of the carina (A) and lower lobes (B) show bilateral scattered small lymphangioleiomyomatosis cysts (arrows). Note the few bilateral scattered nodules of multifocal micronodular pneumocyte hyperplasia (arrowheads). (C) Transaxial chest CT image at the level of the lung base viewed with soft-tissue windows shows a small left chylothorax (arrow). (D) Transaxial chest CT image viewed with bone windows at the level of the mid thoracic spine shows a flame-shaped sclerotic bone focus in the posterior part of a right-sided rib (arrow). (E) Transaxial intravenous contrast-enhanced CT image at the level of the kidneys shows bilateral large renal angiomyolipomas. Note the simple cortical right renal cyst (arrow).

A 53-year-old male with tuberous sclerosis. (A) and (B) Transaxial chest computed tomographic (CT) images viewed with lung windows at the level of the upper lobe (A) and carina (B) show a few bilateral scattered small well-defined nodules of multifocal micronodular pneumocyte hyperplasia. (C) and (D) Two transaxial chest CT image viewed with lung windows at the level of the lower lobes show basal lymphangioleiomyomatosis cysts (arrows). (E) Intravenous contrast-enhanced transaxial chest CT image viewed with soft-tissue windows at the level of the heart shows a subtle intramyocardial fat focus in the left ventricular wall (arrow). (F) and (G) Two transaxial chest CT images viewed with bone windows at the level of the upper thoracic spine show variably-shaped sclerotic bone foci (arrows) in the posterior elements (E) and vertebral body (F).

A 43-year-old woman with tuberous sclerosis. (A) and (B) Two transaxial chest computed tomographic (CT) images viewed with lung windows at the level of the upper lobes show bilateral scattered small ill- and well-defined nodules of multifocal micronodular pneumocyte hyperplasia (MMPH). (C) Coronal reformation CT image viewed with lung windows show upper-lobar predominance of the MMPH nodules (arrows). (D) and (E) Two nonenhanced transaxial chest CT images viewed with soft-tissue windows at the level of the heart show intramyocardial fat foci (arrows) in the left ventricular free wall (D) and interventricular septum (E).

A 31-year-old woman with tuberous sclerosis. (A) Transaxial chest computed tomographic (CT) image viewed with bone windows at the level of the sternal manubrium shows variably-shaped sclerotic bone foci in the sternum (arrows). (B) Nonenhanced transaxial chest CT images viewed with soft-tissue windows at the level of the heart shows intramyocardial fat foci in the interatrial (arrow) and interventricular septa (arrowhead).

A 37-year-old woman with tuberous sclerosis. (A) Intravenous contrast-enhanced transaxial chest computed tomographic (CT) image viewed with soft-tissue windows at the level of the heart shows a subtle intramyocardial fat focus near the left ventricular apex (arrow). (B) Nonenhanced transaxial CT image at the level of the kidneys shows bilateral large and heterogeneous renal angiomyolipomas (AML). Note the tiny hepatic AML as well (arrow).

A 51-year-old woman with tuberous sclerosis. (A) Transaxial chest computed tomographic (CT) images viewed with bone windows at the level of the lower thoracic spine shows a large and irregular sclerotic bone focus in a vertebral body (arrow). (B) and (C) Two nonenhanced transaxial CT images at different upper abdominal levels show 2 hepatic angiomyolipomas (AML) (arrows in B and C) and bilateral large and heterogeneous renal AMLs.

A 60-year-old woman with tuberous sclerosis. (A) Intravenous (IV) contrast-enhanced trans-axial computed tomographic (CT) image at the level of the kidneys shows a large and heterogeneous right renal angiomyolipomas (AML). (B) and (C) Two IV contrast-enhanced transaxial CT images at the level of the kidneys obtained 2 years after (A) show interval enlargement of the right renal AML. Note another small left renal AML (arrow in B).