Abstract | BACKGROUND: 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:
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Authors | Zhao W Yeoh, Vidya Navaratnam, Rupesh Bhatt, Ian McCafferty, Richard B Hubbard, Simon R Johnson |
Journal | Orphanet journal of rare diseases
(Orphanet J Rare Dis)
Vol. 9
Pg. 151
(Oct 03 2014)
ISSN: 1750-1172 [Electronic] England |
PMID | 25277108
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Topics |
- Adult
- Angiomyolipoma
(diagnosis, physiopathology)
- Cohort Studies
- Humans
- Lymphangioleiomyomatosis
(physiopathology)
- Middle Aged
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