Linfangioleiomiomatosis esporádica. Presentación de un caso con enfermedad pulmonar y linfangioleiomiomas retroperitoneales
Sporadic lymphangioleiomyomatosis. Presentation of a case of pulmonary disease with retroperitoneal lymphangioleiomyomas
Ninguna publicación, nacional o extranjera, podrá reproducir ni traducir sus artículos ni sus resúmenes sin previa autorización escrita del editor; sin embargo los usuarios pueden descargar la información contenida en ella, pero deben darle atribución o reconocimiento de propiedad intelectual, deben usarlo tal como está, sin derivación alguna.
Mostrar biografía de los autores
La linfangioleiomiomatosis (LAM) es una enfermedad multisistémica asociada a mutaciones del gen TSC 2, que se presenta en forma esporádica (LMA-E) o asociada al complejo de esclerosis tuberosa (LAM-CET). La LAM cursa con enfermedad pulmonar relacionada con quistes en el parénquima. Sin embargo, los pacientes con LAM-E pueden evidenciar alteraciones mediastinales, retroperitoneales y renales, con adenomegalias, linfangioleiomiomas y angiomiolipomas.
Presentamos el caso de una paciente con LAM-E y enfermedad pulmonar quística asociada a linfangioleiomiomas retroperitoneales y pélvicos.
Visitas del artículo 794 | Visitas PDF 1766
Descargas
- Mavroudi M, Zarogoulidis P, Katsikogiannis N, Tsakiridis K, Huang H, Sakkas A, et al. Lymphangioleiomyomatosis: current and future. J Thorac Dis. 2013;5(1):74-79.
- Cudzilo CJ, Szczesniak RD, Brody AS, et al. Lymphangioleiomyomatosis screening in women with tuberous sclerosis. Chest. 2013;144(2): 578–585.
- Taveira-DaSilva AM, Moss J. Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clinical Epidemiology 2015;7 249-57.
- Grzegorek I, Drozdz K, Podhorska-Okolow M, Szuba A, Dziegiel P. LAM cells biology and lymphangioleiomyomatosis. Folia histochemica et cytobiologica. 2013;51:1–10.
- Henske EP, McCormack FX. Lymphangioleiomyomatosis a wolf in sheep’s clothing. J Clin Invest. 2012;122(11):3807–16.
- Jawad H, Walker CM, Wu CC, Chung JH. Cystic interstitial lung disease: recognizing the common and uncommon entities. Curr Probl Diagn Radiol. 2014:115-27.
- Lee K-H, Lee JS, Lynch DA, Song KS, Lim TH. The radiologic differential diagnosis of diffuse lung disease characterizes by multiple cysts or cavities. J Comput Assist Tomogr. 2002;26(1):5-12.
- Trotman-Dickenson B. Cystic lung disease: achieving a radiologic diagnosis. Eur J Radiol. 2014;83:39-46.
- Avila NA, Dwyer AJ, Rabel A, Moss J. Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features. Radiology. 2007;242(1):277-85.
- Seaman DM, Meyer CA, Gilman MD, McCormack FX.Diffuse cystic lung disease at high resolution CT. AJR 2011;196:1305–11.
- Johnson SR, Cordier JF, Lazor R, Costbel U, Harari S, Reynoud-
- Gaubert M et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010;35(1):14–26.
- Young LR, Vandyke R, Gulleman PM. Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest. 2010;138:674–81.
- Young L, Lee HS, Inoue Y, Moss J, Singer LG, Strange C, et al. Serum VEGF-D a concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response:a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. Lancet Respir Med. 2013;1(6):445–52.
- Pallisa E, Sanz P, Roman A, Majó J, Andreu J, Caceres J. Lymphangioleiomyomatosis: pulmonary and abdominal findings with pathologic correlation. RadioGraphics. 2002;22:S185–98.
- Xu KF, Lo BH. Lymphangioleiomyomatosis: differential diagnosis and optimal management. Ther Clin Risk Management. 2014;10:691-700.
- Barrera EA, Mancheño N, Vera-Sempere F, Padilla J. Lymphangioleiomyomatosis. Arch Bronconeumol. 2011;47(2):85-93.
- Adriaensen MEPM, Schaefer-Prokop CM, Duyndam DAC, Zonnenberg BA, Prokop M. Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex. Clinical Radiology. 2011;66:625-8.
- Hohman DW. Noghrehkar D, Ratnayak S. Lymphangioleiomyomatosis: a review. Eur J Int Med. 2008(19):319–24.
- Avila NA, Dwyer AJ, Moss J. Imaging features of lymphangioleiomyomatosis: diagnostic pitfalls. AJR. 2011;196:982–6.
- Liu ME, Branstetter BF, Whetstone J, Escott EJ. Normal appearance of the distal thoracic duct. AJR. 2006;197:1615-20.
- Avila, NA, Kelly JA, Chu SC, Dwyer AJ, Moss J. Lymphangioleiomyomatosis: abdominopelvic CT and US findings. Radiology. 2000;216:147-53.
- Ryu JH, Moss J, Beck GJ, Lee JC, Brown KK, Chapman JT et al. The NHLBI lymphangioleiomyomatosis registry. characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006;173:105-11.
- Hindman N, Ngo L, Genega EM, Melamed J, Wei J. Angiomyolipoma with minimal fat: can it be differentiated from clear cell renal cell carcinoma by using standard MR techniques? Radiology. 2012;265(2):468-77.
- Harari S, Torre O, Moss J. Lymphangioleiomyomatosis: what do we know and what are we looking for? Eur Respir Rev.2011;20(119):34–44.
- Casanova A, Ancochea J. Lymphangioleiomyomatosis: new therapeutic approaches. Arch Bronconeumol. 2011;47:579-80.
- McCormack FX, Inoue Y, Moss J. National Institutes of Health Rare Lung Diseases Consortium; MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011;364(17):1595–606.
- Bissler JJ, Kingswood JC, Radzikowska E. Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangi- oleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet. 2013;381(9869):817–24.
- Taveira-DaSilva AM, Moss J. Management of lymphangioleiomyomatosis. F1000Prime Reports. 2014;6(116):1-16.