Alizadehasl Azin, Najdaghi Soroush, Mohseni Salehi Maryam, Meshgi Shahla, Hosseini Jebelli Seyedeh Fatemeh, Yalameh Aliabadi Azam, Hakimian Hoda, Forati Sara, Safavirad Amineh, Narimani Davani Delaram
Cardio-Oncology Research Center Rajaie Cardiovascular Medical and Research Institute Tehran Iran.
Heart Failure Research Center, Cardiovascular Research Institute Isfahan University of Medical Science Isfahan Iran.
Clin Case Rep. 2024 Nov 18;12(11):e9580. doi: 10.1002/ccr3.9580. eCollection 2024 Nov.
Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that presents significant diagnostic and therapeutic challenges due to its nonspecific symptoms and propensity for late detection. This case report aimed to elucidate the diagnostic journey, surgical intervention, and multidisciplinary management of this rare entity. In September 2023, a 42-year-old male presented with dyspnea on exertion and retrosternal chest pain, classified as NYHA FC II. Initial investigations, including ECG and lab tests, indicated tachycardia and elevated troponin and NT-pro-BNP levels. Transthoracic and transesophageal echocardiography identified a multilobulated mass in the right ventricular outflow tract and main pulmonary artery. Cardiac MRI and CT angiography confirmed a high-grade pleomorphic spindle cell tumor, leading to surgical resection in October 2023. Histopathology confirmed intimal sarcoma. Postsurgery, the patient underwent chemotherapy and radiotherapy, showing significant clinical improvement and no recurrence on follow-up PET-CT. This case highlights the importance of a multidisciplinary approach in diagnosing and managing primary intimal sarcoma of the pulmonary artery, emphasizing the role of advanced imaging, timely surgical intervention, and combined chemotherapy with radiotherapy in improving patient outcomes.
肺动脉原发性内膜肉瘤是一种罕见且侵袭性强的恶性肿瘤，因其症状不具特异性且易于晚期才被发现，故而在诊断和治疗方面带来了重大挑战。本病例报告旨在阐明这一罕见病症的诊断过程、手术干预及多学科管理。2023年9月，一名42岁男性因劳力性呼吸困难和胸骨后胸痛就诊，纽约心脏协会（NYHA）心功能分级为II级。包括心电图和实验室检查在内的初步检查显示心动过速，肌钙蛋白及N末端脑钠肽前体（NT-pro-BNP）水平升高。经胸和经食管超声心动图检查发现右心室流出道和主肺动脉内有一个分叶状肿块。心脏磁共振成像（MRI）和CT血管造影证实为高级别多形性梭形细胞瘤，遂于2023年10月进行了手术切除。组织病理学确诊为内膜肉瘤。术后，患者接受了化疗和放疗，临床症状显著改善，随访PET-CT未发现复发。本病例凸显了多学科方法在肺动脉原发性内膜肉瘤诊断和管理中的重要性，强调了先进影像学检查、及时手术干预以及化疗联合放疗在改善患者预后方面的作用。
