Wyler von Ballmoos Moritz C, Chan Edward Y, Reardon Michael J
Houston Methodist DeBakey Heart and Vascular Center, Houston, TX, USA.
Department of Cardiovascular Surgery, Houston Methodist DeBakey Heart & Vascular Center, Houston, TX, 77030, USA.
Int J Cardiovasc Imaging. 2019 Aug;35(8):1429-1433. doi: 10.1007/s10554-018-1489-8. Epub 2018 Dec 8.
Primary pulmonary artery sarcoma is a rare tumor originating from the pulmonary artery tree. Given the low incidence, few centers have reported on more than a handful of cases. Because of its rarity it is also commonly misdiagnosed as pulmonary embolism. Multi-modality diagnostic imaging and recognition of specific imaging characteristics along with a high index of suspicion is required to make the correct diagnosis and expedite treatment. The primary imaging modality for most cardiac tumors such as primary pulmonary artery sarcoma is now MRI. It provides superb spatial resolution as well as functional assessment of the heart and pulmonary circulation. CT imaging also is part of routing imaging and remains as the most pertinent imaging modality to evaluate the lung parenchyma and presence of metastatic disease. Here we review the pertinent imaging modalities and tissue characteristics that facilitate recognition of primary pulmonary artery sarcoma. We also provide a short overview of surgical resection and reconstruction, which is the mainstay therapy, for this rare tumor.
原发性肺动脉肉瘤是一种起源于肺动脉树的罕见肿瘤。鉴于其发病率低，很少有中心报道过超过少数几例病例。由于其罕见性，它也常被误诊为肺栓塞。需要多模态诊断成像、识别特定的成像特征以及高度的怀疑指数才能做出正确诊断并加快治疗。对于大多数心脏肿瘤，如原发性肺动脉肉瘤，目前主要的成像方式是MRI。它提供了出色的空间分辨率以及对心脏和肺循环的功能评估。CT成像也是常规成像的一部分，并且仍然是评估肺实质和转移疾病存在的最相关成像方式。在此，我们回顾有助于识别原发性肺动脉肉瘤的相关成像方式和组织特征。我们还简要概述了手术切除和重建，这是这种罕见肿瘤的主要治疗方法。
