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肺动脉内膜肉瘤伴多发肺转移:长期生存病例

Intimal sarcoma of the pulmonary artery with multiple lung metastases: Long-term survival case.

作者信息

García-Cabezas Sonia, Centeno-Haro Macarena, Espejo-Pérez Simona, Carmona-Asenjo Elvira, Moreno-Vega Alberto L, Ortega-Salas Rosa, Palacios-Eito Amalia

机构信息

Sonia García-Cabezas, Amalia Palacios-Eito, Department of Radiation Oncology, Reina Sofia University Hospital, 14004 Cordoba, Spain.

出版信息

World J Clin Oncol. 2017 Aug 10;8(4):366-370. doi: 10.5306/wjco.v8.i4.366.

Abstract

Pulmonary artery intimal sarcoma (PAIS) is a rare tumor with a very poor prognosis. Clinical and radiological findings usually mimic thromboembolic disease, leading to diagnostic delays. The treatment of choice is surgery, and adjuvant chemotherapy and radiotherapy have limited results. We report the case of a 48-year-old male patient, initially suspected with pulmonary thromboembolism. The angio-CT revealed a filling defect in the pulmonary artery trunk. The patient underwent surgery, resulting in with complete resection of the mass with a diagnosis of PAIS. The tumor progressed rapidly in the lung, requiring surgery of multiple lung metastases. The patient was treated with stereotactic body radiation therapy (SBRT) on two occasions for new pulmonary lesions. In the last followup (4 years after initial diagnosis), the patient was disease-free. In conclusion, SBRT proved to be an alternative treatment to metastasectomy, allowing palliative chemotherapy to be delayed or omitted, which may result in improved quality of life.

摘要

肺动脉内膜肉瘤(PAIS)是一种罕见的肿瘤,预后极差。临床和影像学表现通常类似血栓栓塞性疾病,导致诊断延误。首选治疗方法是手术,辅助化疗和放疗效果有限。我们报告一例48岁男性患者,最初怀疑患有肺血栓栓塞症。血管CT显示肺动脉主干有充盈缺损。患者接受了手术,肿块被完整切除,诊断为PAIS。肿瘤在肺部迅速进展,需要对多个肺转移灶进行手术。患者因新出现的肺部病变两次接受立体定向体部放疗(SBRT)。在最后一次随访(初次诊断后4年)时,患者无疾病。总之,SBRT被证明是转移灶切除术的替代治疗方法,可延迟或省略姑息性化疗,这可能会改善生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1139/5554881/b2684b64e529/WJCO-8-366-g001.jpg

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