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四肢静脉源性平滑肌肉瘤。一项具有诊断和手术意义的临床、放射学及形态学相关性研究。

Leiomyosarcomas of venous origin in the extremities. A correlated clinical, roentgenologic, and morphologic study with diagnostic and surgical implications.

作者信息

Berlin O, Stener B, Kindblom L G, Angervall L

出版信息

Cancer. 1984 Nov 15;54(10):2147-59. doi: 10.1002/1097-0142(19841115)54:10<2147::aid-cncr2820541015>3.0.co;2-9.

DOI:10.1002/1097-0142(19841115)54:10<2147::aid-cncr2820541015>3.0.co;2-9
PMID:6488138
Abstract

This article presents a correlated clinical, roentgenologic, and morphologic study of six cases of leiomyosarcoma of large veins in the extremities. Growth of the tumor within the lumen of the affected vein was demonstrated by phlebography in one case and by angiography and computed tomography in one case. In all six cases, such growth was demonstrated by dissection of the surgical specimen. In four cases, arteriography showed both neovascularity at the site of the lesion and compression of the artery accompanying the affected vein. The compression apparently was caused by entrapment of the artery within the same preformed fibrous sheath (conjunctiva vasorum) that enclosed the tumor and the vein from which it had arisen. The histologic diagnosis was based on the light microscopic appearance in all cases and was supported by electron microscopic findings in three cases by the demonstration of ultrastructural features of leiomyoblasts. All six tumors were light microscopically of high-grade malignancy. Five of the patients died of metastatic tumor disease and one is alive with lung metastases. The information obtained by a careful physical examination combined with phlebography, arteriography, and computed tomography may be helpful in the clinical diagnosis and the planning of adequate surgical treatment.

摘要

本文介绍了对6例四肢大静脉平滑肌肉瘤进行的相关临床、放射学和形态学研究。1例通过静脉造影、1例通过血管造影和计算机断层扫描证实肿瘤在受累静脉腔内生长。在所有6例中,通过手术标本解剖证实了这种生长情况。4例动脉造影显示病变部位有新生血管形成,且受累静脉伴行的动脉受压。这种压迫显然是由于动脉被困在包裹肿瘤及其起源静脉的同一预制纤维鞘(血管结膜)内所致。所有病例的组织学诊断均基于光镜表现,3例通过显示成肌纤维细胞的超微结构特征得到电镜检查结果的支持。所有6个肿瘤在光镜下均为高级别恶性。5例患者死于转移性肿瘤疾病,1例存活但有肺转移。通过仔细的体格检查结合静脉造影、动脉造影和计算机断层扫描所获得的信息,可能有助于临床诊断和制定适当的手术治疗方案。

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