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原发性肢体淋巴水肿中Stewart-Treves综合征的磁共振成像结果与病理学对比:一项回顾性单中心研究

Magnetic resonance findings of Stewart-Treves Syndrome in primary limb lymphedema compared with pathology: A retrospective single-center study.

作者信息

Li Bin, Li Jiyuan, Hao Kun, Jin Yanfang, Ma Jun, Du Xuemei

机构信息

Department of MRI, Beijing Shijitan Hospital, Capital Medical University, Beijing, China.

Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing, China.

出版信息

Front Oncol. 2023 Feb 9;13:953524. doi: 10.3389/fonc.2023.953524. eCollection 2023.

DOI:10.3389/fonc.2023.953524
PMID:36874095
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9976609/
Abstract

BACKGROUND

Stewart-Treves Syndrome in Primary Limb Lymphedema (STS-PLE) is an extremely rare malignant tumor. A retrospective analysis was conducted to elucidate the relationship between magnetic resonance imaging (MRI) findings and signs compared to pathology.

METHODS

Seven patients with STS-PLE were enrolled at Beijing Shijitan Hospital, Capital Medical University, from June 2008 to March 2022. All cases were examined by MRI. The surgical specimens were subjected to histopathological and immunohistochemical staining for CD31, CD34, D2-40, and Ki-67.

RESULTS

There were two different types of MRI findings. One was mass shape (STS-PLE I type) in three male patients, and the other was the "trash ice" d sign (STS-PLE II type) observed in four female patients. The average duration of lymphedema (DL) of STS-PLE I type (18 months) was shorter than that of STS-PLE II type (31 months). The prognosis for the STS-PLE I type was worse than that for the STS-PLE II type. Regarding overall survival (OS), the STS-PLE I type (17.3 months) was three times shorter than that of the STS-PLE II type (54.5 months). For STS-PLE I type, the older the STS-PLE onset, the shorter the OS. However, there was no significant correlation in STS-PLE II type. MRI was compared to histological results to provide an explanation for the differences in MR signal changes, especially on T2WI. Against a background of dense tumor cells, the richer the lumen of immature vessels and clefts, the higher the T2WI MRI signal (taking muscle signal as the internal reference standard) and the worse the prognosis, and vice versa. We also found that younger patients with a lower Ki-67 index (<16%) had better OS, especially for the STS-PLE I type. Those with stronger positive expression of CD31 or CD34 had shorter OS. However, the expression of D2-40 was positive in nearly all cases, and seemed not to be associated with prognosis.

CONCLUSIONS

In lymphedema, the richer the lumen of immature vessels and clefts based on dense tumor cells, the higher the T2WI signal on the MRI. In adolescent patients, the tumor often showed a "trash ice" sign (STS-PLE II-type) and prognosis was better than for the STS-PLE I type. While in middle-aged and older patients, tumors showed a mass shape (STS-PLE I type). The expression of immunohistochemical indicators (CD31, CD34, and KI-67) correlated with clinical prognosis, especially decreased Ki-67 expression. In this study, we determined it was possible to predict prognosis comparing MRI findings with pathological results.

摘要

背景

原发性肢体淋巴水肿中的斯图尔特 - 特里夫斯综合征(STS - PLE)是一种极其罕见的恶性肿瘤。进行了一项回顾性分析,以阐明磁共振成像(MRI)结果与体征和病理之间的关系。

方法

2008年6月至2022年3月,首都医科大学附属北京世纪坛医院收治了7例STS - PLE患者。所有病例均接受了MRI检查。手术标本进行了组织病理学检查以及CD31、CD34、D2 - 40和Ki - 67的免疫组织化学染色。

结果

MRI表现有两种不同类型。一种是3例男性患者中的肿块型(STS - PLE I型),另一种是4例女性患者中观察到的“碎冰”征(STS - PLE II型)。STS - PLE I型的平均淋巴水肿持续时间(DL)(18个月)短于STS - PLE II型(31个月)。STS - PLE I型的预后比STS - PLE II型差。关于总生存期(OS),STS - PLE I型(17.3个月)比STS - PLE II型(54.5个月)短三倍。对于STS - PLE I型,STS - PLE发病年龄越大,OS越短。然而,STS - PLE II型中无显著相关性。将MRI与组织学结果进行比较,以解释MR信号变化的差异,尤其是在T2WI上。在密集肿瘤细胞的背景下,未成熟血管和裂隙的管腔越丰富,T2WI MRI信号越高(以肌肉信号作为内部参考标准),预后越差,反之亦然。我们还发现,Ki - 67指数较低(<16%)的年轻患者OS较好,尤其是对于STS - PLE I型。CD31或CD34阳性表达越强的患者OS越短。然而,几乎所有病例中D2 - 40的表达均为阳性,似乎与预后无关。

结论

在淋巴水肿中,基于密集肿瘤细胞的未成熟血管和裂隙的管腔越丰富,MRI上的T2WI信号越高。在青少年患者中,肿瘤常表现为“碎冰”征(STS - PLE II型),预后优于STS - PLE I型。而在中老年患者中,肿瘤表现为肿块型(STS - PLE I型)。免疫组织化学指标(CD31、CD34和KI - 67)的表达与临床预后相关,尤其是Ki - 67表达降低。在本研究中,我们确定将MRI结果与病理结果进行比较来预测预后是可行的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/45f6/9976609/7d1a9bfbd1a0/fonc-13-953524-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/45f6/9976609/28e9d8c284c4/fonc-13-953524-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/45f6/9976609/7d1a9bfbd1a0/fonc-13-953524-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/45f6/9976609/28e9d8c284c4/fonc-13-953524-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/45f6/9976609/7d1a9bfbd1a0/fonc-13-953524-g002.jpg

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