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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).

Purpose: To retrospectively compare the frequencies of computed tomographic (CT) findings in patients with lymphangioleiomyomatosis (LAM) and patients with tuberous sclerosis complex (TSC) and LAM.

Materials and Methods: Institutional review board approval and informed consent were obtained for the HIPAA-compliant study. In 256 patients with LAM (mean age, 44 years) and 67 patients with TSC/LAM (mean age, 40 years), CT scans of the chest, abdomen, and pelvis were reviewed by a single radiologist. The fraction of lung involvement with cysts was estimated from high-spatial-resolution CT scans. Other findings assessed included noncalcified pulmonary nodules, pleural effusion, thoracic duct dilatation, hepatic and renal angiomyolipomas (AMLs), lymphangioleiomyoma (LALM), ascites, nephrectomy, and renal embolization. Confidence intervals and hypothesis tests of differences in frequencies, comparison of age quartiles, RIDIT analysis, analysis of variance, and correlation coefficients were used in the statistical analysis.

Results: Patients with LAM had more extensive lung involvement (RIDIT score, 0.36) and higher frequency of LALM (29% vs 9%, P < .001), thoracic duct dilatation (4% vs 0, P = .3), pleural effusion (12% vs 6%, P = .2), or ascites (10% vs 6%, P = .3). Patients with TSC/LAM had higher frequency of noncalcified pulmonary nodules (12% vs 1%, P < .01), hepatic (33% vs 2%, P < .001) and renal (93% vs 32%, P < .001) AMLs, nephrectomy (25% vs 7%, P < .001), or renal artery embolization (9% vs 2%, P < .05).

Conclusion: The extent of lung disease is greater in LAM than TSC/LAM. Hepatic and renal AMLs and noncalcified lung nodules are more common in TSC/LAM, while lymphatic involvement—thoracic duct dilatation, chylous pleural effusion, ascites, and LALM—is more common in LAM.

© RSNA, 2006