Computed tomographic features of lymphangioleiomyomatosis: evaluation in 138 patients.

ABSTRACT

PURPOSE: The aim was to characterize the computed tomographic (CT) findings from Japanese patients with lymphangioleiomyomatosis (LAM). MATERIALS AND METHODS: CT scans of the chest, abdomen, and pelvis from 124 patients with sporadic LAM (S-LAM, mean age, 37.4 years) and 14 patients with tuberous sclerosis complex (TSC)-LAM (mean age, 35.6 years) were analyzed. RESULTS: Pulmonary nodules (18.8%) and hepatic angiomyolipoma (AML, 24.3%) were more common in our patients than those in previous reports. Compared with TSC-LAM, S-LAM group had a higher frequency of pulmonary nodules (28.6% vs 32.3%, P<0.01) and lower frequencies of air-space consolidation (21.4% vs 2.4%, P<0.01), pneumothorax (28.6% vs 8.1%, P=0.02), pulmonary hilar lymphadenopathy (14.3% vs 0.8%, P<0.01), renal AML (85.7% vs 17.4%, P<0.01), hepatic AML (71.4% vs 17.4%, P<0.01), and retrocrural lymphadenopathy (14.3% vs 1.4%, P=0.04). Axial lymphatic abnormalities (i.e., thoracic duct dilatation, lymphadenopathy, and lymphangioleiomyoma) were most common in the pelvis and tended to decrease in incidence with increased distance from the pelvis. CONCLUSION: The incidence of some CT findings in Japanese patients differed from those in previous reports. Axial lymphatic abnormalities noted here suggest that the origin of LAM cells may be the pelvis.