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Vol. 42. Issue 3.
Pages 148-150 (March 2006)
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Vol. 42. Issue 3.
Pages 148-150 (March 2006)
Case Report
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Intimal Sarcoma of the Pulmonary Artery: a Rare Cause of Pulmonary Hypertension
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I. Furesta,
Corresponding author
ifc01v@saludalia.com

Correspondence: Dr. I. Furest-Carrasco. Servicio de Neumología. Hospital Universitario Dr. Peset. Avda. Gaspar Aguilar, 90. 46017 Valencia. España
, M. Marína, P. Escribanob, M.A. Gómezb, J. Cortinac, R. Blanquera
a Servicio de Neumología, Hospital Universitario Dr. Peset, Valencia, Spain
b Servicio de Cardiología, Hospital 12 de Octubre, Madrid, Spain
c Servicio de Cirugía Cardiovascular, Hospital 12 de Octubre, Madrid, Spain
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Intimal sarcoma of the pulmonary artery is a rare tumor that is usually diagnosed during surgery or autopsy. Such tumors are characterized by local growth, with only slight ability to metastasize. Diagnosis is difficult and often delayed owing to the nonspecific nature of the symptoms. Since intimal sarcoma of the pulmonary artery is so rare and insidious it is often confused with pulmonary thromboembolism and is therefore treated inappropriately with prolonged anticoagulation or thrombolysis. With a mean survival of 12 months from the onset of symptoms, the prognosis is poor. We present the case of a woman who was preoperatively diagnosed with intimal sarcoma of the pulmonary artery and who underwent surgical resection with no apparent recurrence at long term follow-up. A review of the literature is also included.

Key words:
Sarcoma
Pulmonary artery
Pulmonary hypertension

El sarcoma intimal de la arteria pulmonar es un tumor infrecuente que habitualmente se diagnostica en el acto quirúrgico o la autopsia. Por lo general crece regionalmente, con poca capacidad de metastatizar. El diagnóstico es difícil y con frecuencia se retrasa debido a la naturaleza inespecífica de los síntomas. Su baja frecuencia y el crecimiento insidioso hacen que a menudo se confunda con una tromboembolia pulmonar, y por ello se trata inadecuadamente con anticoagulación prolongada o trombólisis. El pronóstico es malo, con una media de supervivencia de 12 meses desde el inicio de los síntomas. Presentamos el caso de una paciente a quien se diagnosticó preoperatoriamente un sarcoma intimal de la arteria pulmonar que se sometió a resección quirúgica, sin que se evidenciase recurrencia en el seguimiento a largo plazo. También se realiza una revisión de la bibliografía.

Palabras clave:
Sarcoma
Arteria pulmonar
Hipertensión pulmonar
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Copyright © 2006. Sociedad Española de Neumología y Cirugía Torácica (SEPAR)
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