Cyst burden on high-resolution computed tomography (HRCT) correlates with VEGF-D in lymphangioleiomyomatosis

Abstract

<b>Introduction:</b> Lymphangioleiomyomatosis (LAM) is a rare metastasizing neoplastic disease that leads to parenchymal destruction and cystic lung disease in women. Diagnostic criteria include high resolution CT chest characteristic of LAM plus one of; serum VEGF-D levels ≥ 800pg/ml, presence of Tuberous Sclerosis Complex, renal angiomyolipomas, chylous effusions or lymphangioleiomyomas.&nbsp;Serum VEGF-D can predict progression but is normal in approximately 1/3 of cases. Method Data on 42 patients with LAM was interrogated to evaluate age, menopausal status, presence of tuberous sclerosis complex, VEGF-D levels, pneumothorax and chylous effusion history,renal angiomyolipomas (AMLs) and PFTs for patients. Low attenuation volumes (LAV) were obtained from DICOM images using SyngoVia post processing software. LAV percentages were calculated at -950 HU and correlated with DLCO, FEV1 and VEGF-D. <b>Results:</b> Forty two patients had a mean age 51.63 years. Fourteen (33%) had previous pneumothorax, 6 (14%) had chylothorax and 13 (31%) had AML. Mean VEGF-D level was 577.82pg/mL (SD+/− 551.15). Nine (21%) had a history of mTOR inhibitor use. FEV1 (r = -0.3708, p = 0.2477) and DLCO (r =-0.2788, p = 0.2477) did not correlate with LAV percentage but there was a positive correlation between VEGF-D levels and LAV percentage (r = 0.6305, p = 0.0313) <b>Conclusion:</b> With 42 patients identified with LAM to date in ireland, this equates to 17/million females, much higher than the reported worldwide prevalence. We aim to further explore the role of developing biomarkers to evaluate progression and prognosis in this group.