Natural history of angiomyolipoma in lymphangioleiomyomatosis: implications for screening and surveillance
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RESEARCH
Open Access
Natural history of angiomyolipoma in lymphangioleiomyomatosis: implications for screening and surveillance Zhao W Yeoh1, Vidya Navaratnam2, Rupesh Bhatt3, Ian McCafferty3, Richard B Hubbard2 and Simon R Johnson1,4*
Abstract Background: 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. Aims: To document the natural history of angiomyolipoma in a national cohort of patients with sporadic LAM to inform tumour screening and surveillance protocols. Methods: 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. Results: 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. Conclusions: 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. Keywords: Lymphangioleiomyomatosis, Tuberous sclerosis, Kidney disease, Natural history
Introduction Lymphangioleiomyomatosis (LAM) is a rare systemic disease, almost exclusively affecting women with a prevalence of 5–10/million women [1]. LAM causes lung cysts and lymphatic abnormalities leading to recurrent pneumothorax, respiratory impairment and chylous collections [2]. LAM occurs both sporadically and in patients with tuberous sclerosis complex (TSC). Angiomyolipoma, a * Correspondence: [email protected] 1 Divisions of Respiratory Medicine and Respiratory Research Unit, School of Medicine, University of Nottingham, D Floor, South Block. Queens Medical Centre, Nottingham NG7 2UH, UK 4 National Centre for Lymphangioleiomyomatosis, Nottingham University Hospitals NHS Trust, Nottingham, UK Full list of author information is available at the end of the article
benign tumour, is present in up to half of patients with sporadic LAM [3,4] and nearly all patients with TSCLAM [5,6]. The majority of angiomyolipomas do not cause sympt
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