Department of Hematology-Oncology, Hotel Dieu de France University Hospital, Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.
Department of Hematology-Oncology, Hotel Dieu de France University Hospital, Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.
Crit Rev Oncol Hematol. 2020 Mar;147:102889. doi: 10.1016/j.critrevonc.2020.102889. Epub 2020 Jan 30.
Only a few hundred cases of intimal sarcomas of pulmonary artery (ISPA) were reported on the literature. Diagnosis of this rare entity is a challenging dilemma with the need for a high expertise in the radiological and pathological identification of ISPA. Treatment strategies rely initially on an early aggressive surgery aiming for complete surgical resection with clear margins while no clear recommendations guiding the choice for additional drug therapy or radiotherapy exist. In this article, we perform an extensive review of the literature on ISPA with details on the clinical presentation, diagnosis and management strategies. An additional goal of this paper is to make practicing oncologists aware of this rare entity with clear idea on the initial management.
仅有几百例肺动脉内膜肉瘤（ISPA）的病例在文献中报道过。诊断这种罕见的实体瘤具有挑战性，需要在放射学和病理学方面具有很高的专业知识来识别 ISPA。治疗策略最初依赖于早期积极的手术，目的是进行完全的手术切除，并保证有清晰的切缘，而目前尚无明确的建议来指导选择额外的药物治疗或放疗。本文对 ISPA 的文献进行了广泛的回顾，详细介绍了其临床表现、诊断和管理策略。本文的另一个目的是让肿瘤学医生了解这种罕见的实体瘤，对其初始管理有清晰的认识。
