Mahdi Ahmad, Mahdi Mahmoud, Ters Patrick
Internal Medicine, University of Kansas School of Medicine, Wichita, USA.
Cardiology, University of Kansas School of Medicine, Wichita, USA.
Cureus. 2023 May 2;15(5):e38439. doi: 10.7759/cureus.38439. eCollection 2023 May.
Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that arises from the intimal layer of the pulmonary artery. It typically presents with nonspecific symptoms such as dyspnea, chest pain, and hemoptysis, making early diagnosis challenging. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful in identifying the tumor's location and extent. A definitive diagnosis is established by biopsy, either via surgical resection or percutaneous needle biopsy. However, diagnosis can be difficult due to the rarity of the disease and the need for specialized expertise in interpreting pathology specimens. Treatment of primary intimal sarcoma of the pulmonary artery involves surgical resection, followed by adjuvant chemotherapy and radiation therapy. Despite aggressive treatment, the prognosis remains poor, with a median survival of approximately two years. However, early detection and aggressive multimodal therapy can improve outcomes. We hereby report a rare case of primary intimal sarcoma of the pulmonary artery and discuss its pathophysiology, presentation, diagnostic approach, and treatment options.
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，起源于肺动脉的内膜层。它通常表现为呼吸困难、胸痛和咯血等非特异性症状，这使得早期诊断具有挑战性。计算机断层扫描（CT）和磁共振成像（MRI）有助于确定肿瘤的位置和范围。通过手术切除或经皮针吸活检进行活检来确立明确诊断。然而，由于该疾病罕见且解读病理标本需要专业知识，诊断可能会很困难。肺动脉原发性内膜肉瘤的治疗包括手术切除，随后进行辅助化疗和放疗。尽管进行了积极治疗，预后仍然很差，中位生存期约为两年。然而，早期发现和积极的多模式治疗可以改善治疗效果。我们在此报告一例罕见的肺动脉原发性内膜肉瘤病例，并讨论其病理生理学、临床表现、诊断方法和治疗选择。
