Department of Nuclear Medicine, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, China.
Department of Pathology, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, China.
Ann Palliat Med. 2021 Jun;10(6):7013-7018. doi: 10.21037/apm-20-630. Epub 2020 Nov 18.
Primary pulmonary artery sarcoma is an extremely rare and highly aggressive malignant tumor of cardiovascular system. It is usually misdiagnosed as pulmonary thromboembolism due to its atypical clinical features and similar clinical symptoms. Different from published reports, our case received both enhanced CT and 18F-FDG PET/CT examination before the pathologic result, and lung metastases had already occurred at the time of diagnosis. We herein reported a case of 41-year-old female patient who suffered from cough and chest tightness for more than a month. Laboratory examination indicated that both blood routine and tumor markers were within the normal range, and only the D-dimer slightly elevated. Contrast-enhanced chest computed tomography showed right pulmonary artery lesion and multiple nodular located right upper lung, the lesion was mild heterogeneous enhancement. No obvious abnormalities were found in deep vein of bilateral lower extremities on ultrasonography. In order to confirm the nature of these lesions, PET/CT scan was performed, which revealed stripe hypermetabolism in right pulmonary artery and nodular hypermetabolism in right upper lung, and the rest of the whole-body PET imaging were negative, a diagnosis of primary pulmonary artery malignancy with pulmonary metastases was made, and pulmonary thromboembolism was ruled out. Biopsy of right pulmonary lesions was performed and histopathological examination indicated pulmonary artery sarcoma. She only received palliative conservative medical treatment because the disease was late stage according to the tumor-node-metastasis (TNM) staging system, and did not acceptable surgical treatment, and was in good health during recent follow-up. Our study suggested that 18F-FDG PET/CT image is a good approach for the diagnosis of pulmonary artery sarcoma and could provide adjunct value for further treatment.
原发性肺动脉肉瘤是一种极为罕见且高度侵袭性的心血管系统恶性肿瘤。由于其非典型的临床特征和相似的临床症状，通常被误诊为肺血栓栓塞症。与已发表的报告不同，我们的病例在获得病理结果之前同时接受了增强 CT 和 18F-FDG PET/CT 检查，并且在诊断时已经发生了肺转移。我们在此报告一例 41 岁女性患者，她因咳嗽和胸闷超过一个月而就诊。实验室检查表明血常规和肿瘤标志物均在正常范围内，只有 D-二聚体略有升高。增强胸部 CT 显示右肺动脉病变和多个位于右上肺的结节状病变，病变呈轻度不均匀增强。超声检查未发现双侧下肢深静脉明显异常。为了明确这些病变的性质，进行了 PET/CT 扫描，显示右肺动脉条纹状高代谢和右上肺结节状高代谢，全身其余部位的 PET 成像均为阴性，诊断为原发性肺动脉恶性肿瘤伴肺转移，并排除了肺血栓栓塞症。对右肺病变进行了活检，组织病理学检查提示肺动脉肉瘤。由于根据肿瘤-淋巴结-转移（TNM）分期系统，疾病已处于晚期，她无法接受手术治疗，仅接受姑息性保守治疗，在最近的随访中身体状况良好。我们的研究表明，18F-FDG PET/CT 图像是诊断肺动脉肉瘤的一种很好的方法，可以为进一步治疗提供附加价值。
