Burke A P, Virmani R
Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000.
Cancer. 1993 Mar 1;71(5):1761-73. doi: 10.1002/1097-0142(19930301)71:5<1761::aid-cncr2820710510>3.0.co;2-7.
There are few reports comparing the clinicopathologic features of sarcomas of the aorta (SA), sarcomas of the inferior vena cava (SIVC), and sarcomas of the pulmonary artery (SPA).
The authors retrospectively reviewed 11 SA, 16 SIVC, and 16 SPA, and compared clinical, pathologic, and immunohistochemical findings.
The mean age at presentation for SA was 62.3 +/- 17.3 years versus 41.3 +/- 17.1 for SPA; mean age for SIVC was 49.9 +/- 18.8. Nine of 11 SA and 14 of 16 SPA were grossly confined to the lumen, compared with only two SIVC. Luminal sarcomas were classified as poorly differentiated (intimal), angiosarcoma or leiomyosarcoma. Eight SA, 13 SPA, and one SIVC were of the intimal type and were composed of fibroblastic or myofibroblastic cells; five had "storiform" areas typical of malignant fibrous histiocytoma, and all had areas of necrosis. Intimal SPA were more likely myxoid than SA, and osteosarcomatous differentiation was present only in SPA (three cases). Intimal sarcomas were negative for desmin, Factor VIII-related antigen, S-100 protein, and CD34/QBend; all were positive for vimentin and most showed positive cells for smooth muscle actin. One luminal SA and one luminal SPA were histologically typical of angiosarcoma. Two SPA, 2 SA, and 14 SIVC were predominantly mural, most of which were leiomyosarcomas. The mean survival of intimal SA was poor (5 months), compared with 37 months for SIVC and 23 months for intimal SPA.
SA, SPA, and SIVC differ in their clinical presentation and survival. Most SA and SPA sarcomas are aggressive, probably derived from intimal cells that show myofibroblastic differentiation. SIVC are usually derived from medial smooth muscle and are relatively well differentiated leiomyosarcomas.
关于主动脉肉瘤（SA）、下腔静脉肉瘤（SIVC）和肺动脉肉瘤（SPA）的临床病理特征比较的报道较少。
作者回顾性分析了11例SA、16例SIVC和16例SPA，并比较了其临床、病理和免疫组化结果。
SA患者的平均就诊年龄为62.3±17.3岁，而SPA为41.3±17.1岁；SIVC的平均年龄为49.9±18.8岁。11例SA中有9例和16例SPA中有14例大体上局限于管腔内，而SIVC仅有2例。管腔内肉瘤分为低分化（内膜型）、血管肉瘤或平滑肌肉瘤。8例SA、13例SPA和1例SIVC为内膜型，由成纤维细胞或肌成纤维细胞组成；5例有恶性纤维组织细胞瘤典型的“席纹状”区域，且均有坏死区域。内膜型SPA比SA更易呈黏液样，骨肉瘤分化仅见于SPA（3例）。内膜型肉瘤结蛋白、因子VIII相关抗原、S-100蛋白和CD34/QBend均为阴性；波形蛋白均为阳性，大多数平滑肌肌动蛋白呈阳性细胞。1例管腔内SA和1例管腔内SPA在组织学上为典型的血管肉瘤。2例SPA、2例SA和14例SIVC主要位于壁内，其中大多数为平滑肌肉瘤。内膜型SA的平均生存期较差（5个月），而SIVC为37个月，内膜型SPA为23个月。
SA、SPA和SIVC在临床表现和生存期方面存在差异。大多数SA和SPA肉瘤具有侵袭性，可能起源于显示肌成纤维细胞分化的内膜细胞。SIVC通常起源于中膜平滑肌，是分化相对良好的平滑肌肉瘤。
