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Sporadic Lymphangioleiomyomatosis and Tuberous Sclerosis Complex with Lymphangioleiomyomatosis: Comparison of CT Features¹

¹ From the Diagnostic Radiology Department, Warren G. Magnuson Clinical Center (N.A.A., A.J.D.), and Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute (A.R., J.M.), National Institutes of Health, Bldg 10, Room 1C-660, 10 Center Dr, MSC 1182, Bethesda, MD 20892-1182. From the 2003 RSNA Annual Meeting. Received October 31, 2005; revision requested December 19; revision received January 27, 2006; accepted February 17; final version accepted April 19. Supported in part by the intramural research program of the National Institutes of Health, National Heart, Lung, and Blood Institute. Address correspondence to N.A.A. (e-mail: navila{at}nih.gov).

View larger version (123K): [in this window] [in a new window] [Download PPT slide]   Figure 1: Transverse prone nonenhanced high-spatial-resolution 1-mm-thick CT section in a 56-year-old woman with LAM shows severe involvement of the lungs. Note almost complete replacement of lung tissue by cysts.

View larger version (24K): [in this window] [in a new window] [Download PPT slide]   Figure 2: Graph shows that mean lung grades for patients with LAM are consistently greater than those for patients with TSC/LAM across all four age quartiles.

View larger version (111K): [in this window] [in a new window] [Download PPT slide]   Figure 3: Transverse 5-mm-thick contrast-enhanced CT section through upper lungs in a 55-year-old woman with TSC/LAM. Multiple lung nodules (arrowheads) consistent with multifocal micronodular pneumocyte hyperplasia and few lung cysts (arrow) indicate minimal lung involvement with LAM.

View larger version (13K): [in this window] [in a new window] [Download PPT slide]   Figure 4a: Schematics show overall and conditional probabilities of renal AML, hepatic AML, and LALM in patients with (a) LAM and (b) TSC/LAM. Numbers in boxes are overall probabilities of findings in each group. Number associated with each arrow is conditional probability of the finding to which the arrow points, given presence of the finding from which the arrow originates. For example, in patients with LAM, 32% had a renal abnormality, and of those 17% had LALM; 29% had LALM, and of those 19% had a renal abnormality. Renal and hepatic AMLs were more common in patients with TSC/LAM than in patients with LAM. LALM was more common in patients with LAM.

View larger version (13K): [in this window] [in a new window] [Download PPT slide]   Figure 4b: Schematics show overall and conditional probabilities of renal AML, hepatic AML, and LALM in patients with (a) LAM and (b) TSC/LAM. Numbers in boxes are overall probabilities of findings in each group. Number associated with each arrow is conditional probability of the finding to which the arrow points, given presence of the finding from which the arrow originates. For example, in patients with LAM, 32% had a renal abnormality, and of those 17% had LALM; 29% had LALM, and of those 19% had a renal abnormality. Renal and hepatic AMLs were more common in patients with TSC/LAM than in patients with LAM. LALM was more common in patients with LAM.

View larger version (131K): [in this window] [in a new window] [Download PPT slide]   Figure 5: Transverse 5-mm-thick contrast-enhanced CT section through midabdomen in a 46-year-old woman with TSC/LAM. Note fatty hepatic lesion (arrowhead) and too-numerous-to-count bilateral renal fatty lesions.

View larger version (121K): [in this window] [in a new window] [Download PPT slide]   Figure 6: Transverse 5-mm-thick contrast-enhanced CT section through lower abdomen in a 25-year-old woman with LAM and large multiseptated complex retroperitoneal mass consistent with LALM. Note anterior displacement of aorta (A) and IVC (V) by the mass (*).

View larger version (24K): [in this window] [in a new window] [Download PPT slide]   Figure 7: Graph shows that frequencies of renal AML and/or nephrectomy were consistently much higher in TSC/LAM group than in LAM group across all age quartiles.

View larger version (23K): [in this window] [in a new window] [Download PPT slide]   Figure 8: Graph shows that mean total renal score (total number of AMLs and nephrectomies in both kidneys) in patients with renal abnormalities was consistently much higher in TSC/LAM group than in LAM group across all age quartiles.

View larger version (14K): [in this window] [in a new window] [Download PPT slide]   Figure 9a: Scatterplots of age versus total renal score for patients with (a) LAM and (b) TSC/LAM. Comparison of distribution of data points demonstrates tendency of total renal scores of patients with LAM to be lower than those of patients with TSC/LAM across age range of patients examined.

View larger version (16K): [in this window] [in a new window] [Download PPT slide]   Figure 9b: Scatterplots of age versus total renal score for patients with (a) LAM and (b) TSC/LAM. Comparison of distribution of data points demonstrates tendency of total renal scores of patients with LAM to be lower than those of patients with TSC/LAM across age range of patients examined.

View larger version (20K): [in this window] [in a new window] [Download PPT slide]   Figure 10: Graph shows that for each grade (extent of lung disease), probability of renal disease is much higher in patients with TSC/LAM than in patients with LAM and is relatively constant across grades, without evidence of positive trend or correlation between extent of lung cysts and probability of renal disease.

View larger version (19K): [in this window] [in a new window] [Download PPT slide]   Figure 11: Graph shows that for each grade (extent of lung disease), mean total renal score in patients with renal abnormalities is much higher in patients with TSC/LAM than in patients with LAM, without evidence of positive trend or correlation between lung and renal disease severity.

View larger version (19K): [in this window] [in a new window] [Download PPT slide]   Figure 12: Cumulative probability distribution of patients' ages. Curves are shifted about 4 years relative to one another but are similar in shape, indicating that LAM patients were, on average, about 4 years older than TSC/LAM patients. Age distribution plots of patients in the two groups were of similar shape and/or spread.