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散发性与辐射相关血管肉瘤:48例病例的临床病理与分子比较分析

Sporadic versus Radiation-Associated Angiosarcoma: A Comparative Clinicopathologic and Molecular Analysis of 48 Cases.

作者信息

Hung Jennifer, Hiniker Susan M, Lucas David R, Griffith Kent A, McHugh Jonathan B, Meirovitz Amichay, Thomas Dafydd G, Chugh Rashmi, Herman Joseph M

机构信息

Tunnell Cancer Center, Beebe Medical Center, Rehoboth Beach, DE 19971, USA.

出版信息

Sarcoma. 2013;2013:798403. doi: 10.1155/2013/798403. Epub 2013 Sep 3.

Abstract

Angiosarcomas are aggressive tumors of vascular endothelial origin, occurring sporadically or in association with prior radiotherapy. We compared clinicopathologic and biologic features of sporadic angiosarcomas (SA) and radiation-associated angiosarcomas (RAA). Methods. From a University of Michigan institutional database, 37 SA and 11 RAA were identified. Tissue microarrays were stained for p53, Ki-67, and hTERT. DNA was evaluated for TP53 and ATM mutations. Results. Mean latency between radiotherapy and diagnosis of RAA was 11.9 years: 6.7 years for breast RAA versus 20.9 years for nonbreast RAA (P = 0.148). Survival after diagnosis did not significantly differ between SA and RAA (P = 0.590). Patients with nonbreast RAA had shorter overall survival than patients with breast RAA (P = 0.03). The majority of SA (86.5%) and RAA (77.8%) were classified as high-grade sarcomas (P = 0.609). RAA were more likely to have well-defined vasoformative areas (55.6% versus 27%, P = 0.127). Most breast SA were parenchymal in origin (80%), while most breast RAA were cutaneous in origin (80%). TMA analysis showed p53 overexpression in 25.7% of SA and 0% RAA, high Ki-67 in 35.3% of SA and 44.4% RAA, and hTERT expression in 100% of SA and RAA. TP53 mutations were detected in 13.5% of SA and 11.1% RAA. ATM mutations were not detected in either SA or RAA. Conclusions. SA and RAA are similar in histology, immunohistochemical markers, and DNA mutation profiles and share similar prognosis. Breast RAA have a shorter latency period compared to nonbreast RAA and a significantly longer survival.

摘要

血管肉瘤是起源于血管内皮的侵袭性肿瘤,可散发性发生或与既往放疗相关。我们比较了散发性血管肉瘤(SA)和放疗相关血管肉瘤(RAA)的临床病理及生物学特征。方法:从密歇根大学机构数据库中识别出37例SA和11例RAA。组织微阵列进行p53、Ki-67和hTERT染色。对DNA进行TP53和ATM突变评估。结果:放疗与RAA诊断之间的平均潜伏期为11.9年:乳腺RAA为6.7年,非乳腺RAA为20.9年(P = 0.148)。SA和RAA诊断后的生存率无显著差异(P = 0.590)。非乳腺RAA患者的总生存期短于乳腺RAA患者(P = 0.03)。大多数SA(86.5%)和RAA(77.8%)被归类为高级别肉瘤(P = 0.609)。RAA更可能具有界限清楚的血管形成区域(55.6%对27%,P = 0.127)。大多数乳腺SA起源于实质(80%),而大多数乳腺RAA起源于皮肤(80%)。组织微阵列分析显示,25.7%的SA和0%的RAA中p53过表达,35.3%的SA和44.4%的RAA中Ki-67高表达,100%的SA和RAA中hTERT表达。13.5%的SA和11.1%的RAA中检测到TP53突变。SA和RAA中均未检测到ATM突变。结论:SA和RAA在组织学、免疫组化标志物和DNA突变谱方面相似,预后也相似。与非乳腺RAA相比,乳腺RAA的潜伏期较短,生存期明显更长。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/75d9/3776386/4ab40fa872ad/SRCM2013-798403.001.jpg

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