Linfangiomatosis torácica difusa: diagnóstico y tratamiento
Diffuse Thoracic Lymphangiomatosis: Diagnosis and Treatment
E.J. Bermejo Casero*, R. Mongil Poce, R. Arrabal Sánchez, A. Fernández de Rota Avecilla, A. Benítez Doménech, J.L. Fernández Bermúdez
Servicio de Cirugía Torácica. Hospital Regional Universitario Carlos Haya. Málaga. España.
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Mongil Poce, R. Arrabal Sánchez, A. Fernández de Rota Avecilla, A. Benítez Doménech, J.L. Fernández Bermúdez" "autores" => array:6 [ 0 => array:3 [ "nombre" => "E.J." "apellidos" => "Bermejo Casero" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "R." "apellidos" => "Mongil Poce" ] 2 => array:2 [ "nombre" => "R." "apellidos" => "Arrabal Sánchez" ] 3 => array:2 [ "nombre" => "A." "apellidos" => "Fernández de Rota Avecilla" ] 4 => array:2 [ "nombre" => "A." "apellidos" => "Benítez Doménech" ] 5 => array:2 [ "nombre" => "J.L." "apellidos" => "Fernández Bermúdez" ] ] "afiliaciones" => array:1 [ 0 => array:1 [ "entidad" => "Servicio de Cirugía Torácica. Hospital Regional Universitario Carlos Haya. Málaga. España." ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "*" "correspondencia" => "Correspondencia: Servicio de Cirugía Torácica. Hospital Regional Universitario Carlos Haya.Avda. Carlos Haya, s/n. 29010 Málaga. España." ] ] ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Diffuse Thoracic Lymphangiomatosis: Diagnosis and Treatment" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2004-03-30" "fechaAceptado" => "2004-05-04" "PalabrasClave" => array:2 [ "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec190025" "palabras" => array:3 [ 0 => "Linfangiomatosis torácica" 1 => "Linfangioma torácico" 2 => "Infiltrados broncovasculares" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec190026" "palabras" => array:3 [ 0 => "Thoracic lymphangiomatosis" 1 => "Thoracic lymphangioma" 2 => "Bronchoalveolar infiltrates" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "es" => array:1 [ "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">La linfangiomatosis constituye una rara clase de tumoración benigna, desde el punto de vista histológico, resultado de un anormal desarrollo y proliferación del sistema linfático. La afectación torácica puede presentarse localizada (linfangioma) o difusa (linfangiomatosis). En la mayoría de los casos el curso evolutivo de la enfermedad es progresivo con aparición de clara morbilidad e incluso mortalidad</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">En las formas localizadas la cirugía, principalmente, o la instilación local de sustancias esclerosantes (antígeno estreptocócico OK-432) son el tratamiento elegido. Ahora bien, en las formas difusas existe un material gelatinoso, infiltrante, sin límites definidos. Aquí la radioterapia constituye la principal opción terapéutica</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Se presentan 2 casos de linfangiomatosis torácica difusa con infiltración pulmonar. Se comprueba que la radioterapia a dosis adecuadas consigue la desaparición de los infiltrados pulmonares y del derrame pleural, así como de la disnea y mal estado general. La cirugía es necesaria en la resolución de las complicaciones paralelas a la enfermedad y como técnica diagnóstica</p>" ] "en" => array:1 [ "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Histologically, lymphangiomatosis is a rare type of benign neoplasm caused by abnormal development and proliferation of the lymphatic system. Thoracic lymphangiomatosis can present in a localized (lymphangioma) or diffuse form (lymphangiomatosis). In most cases the disease progresses to serious morbidity or even death</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The treatment of choice for localized disease is usually surgery or, less frequently, local injection of sclerosing agents (streptococcus antigen OK-432). However, in diffuse forms there is a gelatinous infiltrate without defined limits. In these cases the main treatment option is radiotherapy</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">We report 2 cases of diffuse thoracic lymphangiomatosis with pulmonary infiltrate. In both cases radiotherapy in appropriate doses successfully eliminated pulmonary infiltrates, pleural effusion, dyspnea, and general discomfort. Surgery was needed to resolve complications of the disease and for diagnosis</p>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografía" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis and lymphatic dysplasia syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J.L. Faul" 1 => "G.J. Berry" 2 => "T.V. Colby" 3 => "S.J. Ruoss" 4 => "M.B. Walter" 5 => "G.D. Rosen" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1164/ajrccm.161.3.9904056" "Revista" => array:6 [ "tituloSerie" => "Am J Respir Crit Care Med" "fecha" => "2000" "volumen" => "161" "paginaInicial" => "1037" "paginaFinal" => "1046" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/10712360" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Diffuse pulmonary lymphangiomatosis: CT findings" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "S.J. Swensen" 1 => "T.E. Hartman" 2 => "J.R. Mayo" 3 => "T.V. Colby" 4 => "H.D. Tazelaar" 5 => "N.L. 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"referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Guidelines for the successful treatment of lymphangioma with OK-432" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "B. Banieghbal" 1 => "M.R.Q. Davies" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1055/s-2003-39581" "Revista" => array:6 [ "tituloSerie" => "Eur J Pediatr Surg" "fecha" => "2003" "volumen" => "13" "paginaInicial" => "103" "paginaFinal" => "107" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12776241" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Treatment of thoracic lymphangiomatosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "A.Y. 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