ABSTRACT
El sarcoma de arteria pulmonar es un tumor infrecuente que afecta en mayor medida a mujeres y con muy mal pronóstico. El diagnóstico suele ser difícil, por su insidioso crecimiento y clínica inespecífica, lo que hace confundir frecuentemente su diagnóstico con una enfermedad tromboembólica pulmonar. Así mismo, ha sido descrito como una causa poco frecuente de hipertensión pulmonar. Presentamos a continuación el caso de una mujer de 66 años de edad, con diagnóstico inicial de tromboembolismo pulmonar (TEP) e hipertensión pulmonar (HTP) secundaria al mismo, siendo diagnosticada finalmente mediante biopsia quirúrgica, de sarcoma intimal de arteria pulmonar. Lo que hace diferente a nuestra paciente con respecto a casos previos es la afectación metastásica a nivel de abdomen, que se objetivó a los pocos meses del diagnóstico definitivo
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
