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C. De La Torre Cabrera



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    P3.11 - Poster Session/ Palliative and Supportive Care (ID 231)

    • Event: WCLC 2015
    • Type: Poster
    • Track: Palliative and Supportive Care
    • Presentations: 1
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      P3.11-011 - Mesenchymal Thoracic Neoplasma Presenting as a Thromboembolism (ID 3192)

      09:30 - 09:30  |  Author(s): C. De La Torre Cabrera

      • Abstract
      • Slides

      Background:
      In the screening of the possible etiology of pulmonary embolism rule out the presence of an occult neoplasia. Tumors more often associated with thrombotic phenomena are lung, pancreas and colon. The pulmonary artery sarcoma is a rare entity and its clinical diagnosis is complex.

      Methods:
      We report a case of a non-smoker 71 years old woman. In 1999 suffers first episode of thrombophlebitis. Since then presents several episodes of DVT in the lower limbs so it was anticoagulated with acenocumarol. In October 2014 she was admitted due to costalgia and fever and suspected diagnosis of pneumonia. She told a 3 months history of asthenia and progressive edema of the lower limbs. It is performed thoracic CT, with a massive pulmonary thromboembolism. The doppler sonography of lower limb show a chronic thrombosis. After clinical stabilization she was put under rivaroxaban. At 15th admission day, she starts with dyspnea with chest discomfort, and respiratory failure was found. In an urgent CT was shown a progression of the known embolism. Being a massive thrombosis refractory to treatment and progressive elevation of pulmonary pressure, surgeon was consulted, and is was performed a thromboendarterectomy and a pulmonary artery homograft replacement. In the pathology report, is reported a intermediate-grade sarcoma, suggestive of intramural primary origin and intimal type intimal grade. In January 2015 CT shows progression of local disease, and is discussed in the tumor board, considering unresectability of disesase, and it is proposed to start palliative chemotherapy

      Results:
      The pulmonary artery sarcoma is a rare disease, since its first description in 1923, there have been documented 200 cases. It can be classified according to their location relative to the vessel wall, or by histologic subtype. Usually located in the main pulmonary artery, diagnosis and complications arise from its intraluminal extension. Up to 50% of cases have pulmonary and mediastinal metastases at diagnosis and distant metastases in 16-19% It is an entity with very similar clinical and radiological features a thromboembolism, a fact that probably contribute to underdiagnosis. The symptoms are dyspnea (72-100%), chest pain (35-45%) and hemoptysis (15-24%), weight loss (21%), asthenia (10%) and fever (8%). The prognosis is poor, with survival reported between 6 months and 2 years (median 17 months) The treatment of choice is surgical approach, by pulmonary endarterectomy, lobectomy or pneumonectomy, with or without reconstruction of the pulmonary artery. In most cases R0 resection is not achieved. The role of radiotherapy (RT) and chemotherapy (CT) is not yet well defined. Regarding chemotherapy schedules, it have been used traditionally active drugs for the treatment of sarcomas

      Conclusion:
      The pulmonary artery sarcoma is a rare disease that should be suspected in patients with progressive or refractory pulmonary thromboembolism.

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