Department of Pathology and Laboratory Medicine, The University of Texas Medical School at Houston, The University of Texas Health Science Center at Houston (UTHealth), Houston, TX.
Cardiovasc Pathol. 2013 Sep-Oct;22(5):351-6. doi: 10.1016/j.carpath.2013.03.005. Epub 2013 Apr 28.
Primary vascular neoplasms are rare entities. They were first described as arising spontaneously in the aorta and other vessels. However, in the past several decades, a number of systemic artery-derived vascular neoplasms, mostly sarcomas, have been reported as arising in intimate association with synthetic grafts. We describe two additional cases of intimal sarcoma seen at our institution. The first is an invasive intimal sarcoma detected in a thoracoabdominal aortic aneurysm at the time of surgical intervention. The second is a superficial spreading intimal sarcoma associated with a Dacron-coated graft, in place for 9 years, detected when the graft was replaced. When the patient died 3 months later, a metastatic subcutaneous sarcomatous lesion was detected at autopsy. In these cases, we studied selective molecular pathways that may be involved in the transformation of benign endothelium to malignant endothelium, with implications for possible therapeutic targets. These cases are presented in order to contribute additional data to the literature involving these vascular neoplasms and to potentially provide a spectrum of disease seen in the vasculature tissues that may arise spontaneously or after placement of a synthetic graft.
原发性血管肿瘤是罕见的实体瘤。它们最初被描述为自发发生在主动脉和其他血管中。然而，在过去的几十年中，许多与合成移植物密切相关的系统性动脉来源的血管肿瘤，主要是肉瘤，已经被报道。我们在本院描述了另外两例内膜肉瘤病例。第一个是在手术干预时在胸腹主动脉瘤中检测到的侵袭性内膜肉瘤。第二个是与 Dacron 涂层移植物相关的浅表扩散性内膜肉瘤，放置 9 年后，在更换移植物时发现。3 个月后患者死亡时，尸检时发现了一个转移性皮下肉瘤病变。在这些病例中，我们研究了可能参与良性内皮向恶性内皮转化的选择性分子途径，这可能为可能的治疗靶点提供依据。这些病例的提出是为了为涉及这些血管肿瘤的文献提供更多的数据，并可能为可能自发发生或在放置合成移植物后发生的血管组织中的疾病谱提供依据。
