Natural history of angiomyolipoma in lymphangioleiomyomatosis: implications for screening and surveillance.

[lymphangioleiomyomatosis]

LAM is a rare disease of women categorised by lung cysts and lymphatic abnormalities . The disease occurs sporadically or associated with Tuberous Sclerosis Complex (TSC-LAM ). Angiomyolipoma , a benign tumour , prone to haemorrhage , occurs mostly in the kidneys in many of these patients . Treatment guidelines exist for angiomyolipoma in patients with TSC but the natural history of angiomyolipoma in sporadic LAM has not been studied .To document the natural history of angiomyolipoma in a national cohort of patients with sporadic LAM to inform tumour screening and surveillance protocols .Demographic data , clinical features , lung function and tumour size were obtained from clinical records of patients attending the National Centre for LAM in Nottingham , UK .122 patients with definite or probable LAM by European Respiratory Society criteria were identified . One hundred and seven had sporadic LAM , of which 53 (50 %) had at least one angiomyolipoma . In patients with sporadic LAM presentation of angiomyolipoma preceded or followed onset of lung symptoms by up to 11 and 38 years respectively . Mean tumour size was 28 mm (range 5 -140 mm ) at presentation and growth was 1 .8 mm /yr (95 % C .I . 0 .42 -3 .82 ) thereafter . Eleven patients with sporadic LAM had had a nephrectomy due to angiomyolipoma bleeding . The need for intervention did not differ between those with TSC-LAM and sporadic LAM .Patients with LAM have a high prevalence of symptomatic angiomyolipoma which can present at any time . Angiomyolipoma in sporadic-LAM have a similar risk of bleeding to those with TSC . All patients should be screened for angiomyolipoma at diagnosis of lung disease by MRI scanning and the tumours require continuous monitoring .