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病例报告
乳房外佩吉特病并肝转移瘤应用磁共振脂肪及铁定量技术一例
郭茜 尚全良

Cite this article as: Guo X, Shang QL. Extramammary Paget's disease complicated with liver metastases by magnetic resonance fat and iron quantification: One case report[J]. Chin J Magn Reson Imaging, 2022, 13(9): 110-111.本文引用格式:郭茜, 尚全良. 乳房外佩吉特病并肝转移瘤应用磁共振脂肪及铁定量技术一例[J]. 磁共振成像, 2022, 13(9): 110-111. DOI:10.12015/issn.1674-8034.2022.09.024.


[摘要] 本文为回顾性研究,经过中南大学湘雅二医院伦理委员会批准,免除受试者知情同意,批准文号:LYF2022023。
[关键词] 乳房外佩吉特病;肝脏转移瘤;磁共振成像;新型定量技术;脂肪及铁定量;病例报告
[Keywords] extramammary Paget's disease;liver metastases;magnetic resonance imaging;new quantitative technique;fat and iron quantification;case report

郭茜    尚全良 *  

中南大学湘雅二医院放射科,长沙 410011

*尚全良,E-mail:sqljsdh@163.com

作者利益冲突声明:全部作者均声明无利益冲突。


收稿日期:2022-02-16
接受日期:2022-09-06
中图分类号:R445.2  R739.5 
文献标识码:B
DOI: 10.12015/issn.1674-8034.2022.09.024
本文引用格式:郭茜, 尚全良. 乳房外佩吉特病并肝转移瘤应用磁共振脂肪及铁定量技术一例[J]. 磁共振成像, 2022, 13(9): 110-111. DOI:10.12015/issn.1674-8034.2022.09.024

       本文为回顾性研究,经过中南大学湘雅二医院伦理委员会批准,免除受试者知情同意,批准文号:LYF2022023。

       患者男,55岁,2016年发现右侧腋窝肿块至湖南省第二人民医院行肿块切除术,术后病理回报汗腺恶性肿瘤,后未予以进一步治疗。2019年7月因右侧腋窝再发肿块入我院治疗,予以右侧腋窝淋巴结清扫术,术后病理回报右侧腋窝淋巴结有腺癌转移,以后均在我院治疗及复查。2019年9月开始予以TP方案(紫杉醇及顺铂)化疗,术后定期回院常规复查。于2020年12月肝右叶新发两处环形强化灶(图1A1B),结合病史,考虑转移,遂行右肝病变切除术,病理提示肝脏转移性乳腺癌伴神经内分泌分化(图1I1K),免疫组化:CAM5.2(+)、EMA(+)、CEA(+)、GCDFP15(个别细胞)、ER(95%,染色强度:强)、PR(95%,染色强度:强)、CK7(+)、Ki-67(热点区50%)、Syn(+)、PD-L1(CPS;1)、GATA3(+)、CK5/6(-)、CK20(-)、CD56(-)、TTF-(-)、p40(-)、p63(-)。2021年4月开始予以EP(VP-16和顺铂)方案化疗。2021年10月MRI发现肝脏新发转移灶(图1C),继续常规化疗及肝脏MRI定期复查。术后两次复查MRI均使用国产联影uMR790 MRI设备行常规序列+新型定量FACT(fat analysis calculation technique)序列扫描,对肝脏无肿瘤实质区行肝脂肪及铁定量。2022年1月MRI常规复查时发现肝脏转移灶较2021年10月缩小(图1C1D),且2022年1月的非肿瘤区肝实质质子密度脂肪分数(proton density fat fraction, PDFF)及反映铁沉积的R2*值均较前次下降(2021年10月平均PDFF为5.0%,R2*值为66.3 Hz;2022年1月PDFF为4.1%,R2*值为61.5 Hz)(图1E1H)。

图1  男,55 岁,乳房外佩吉特病并肝转移瘤。1A、1B:2020 年12 月肝脏MRI T1 增强序列,肝S6、S5 环形强化灶(箭),结合病史,考虑肝转移瘤;1C:2021 年10 月肝脏MRI T1 增强序列,残肝新发直径18 mm异常强化灶(箭);1D:2022 年1 月肝脏MRI T1 增强序列,异常强化灶较前缩小(箭),约11 mm;1E:2021 年10 月MRI 定量技术质子密度脂肪分数(PDFF)对残肝非肿瘤实质区勾画感兴趣区(ROI),多区PDFF=3.81%、4.55%、6.71%(圆圈);1F:2021 年10 月MRI 定量技术R2*对残肝非肿瘤实质区勾画ROI,多区R2*=65.2 Hz、69.7 Hz、63.9 Hz(圆圈);1G:2022 年1 月PDFF 技术对残肝MRI 定量,多区PDFF=3.46%、2.19%、6.69%(圆圈);1H:2022 年1 月残肝MRI R2*定量,多区R2*=54.3 Hz、52.1 Hz、78.1 Hz(圆圈);1I~1K:分别为2020 年12 月环形强化灶病理(HE ×100),免疫组化(CK+ ×40),免疫组化(Syn+ ×40)图。
Fig. 1  Male, 55 years old, extramammary Paget's disease complicated with liver metastases. 1A, 1B: Liver MRI in December 2020, two nodules were observed in liver S6 and S5 (arrow), respectively, with ring enhancement, combined with the medical history, liver metastases were considered; 1C: In October 2021, liver MRI T1 showed a new 18 mm abnormal enhancement lesion in the residual liver (arrow); 1D: In January 2022, the abnormal enhancement foci were smaller than before (arrow), about 11 mm; 1E: In October 2021, the new magnetic resonance quantitative technology proton density fat fraction (PDFF) delineated the region of interest (ROI) in the non-tumor parenchymal region of the residual liver, PDFF is 3.81%, 4.55%, 6.71%, respectively (circle); 1F: In October 2021, the new magnetic resonance quantitative technology R2* delineated the ROI in the non-tumor parenchymal region of the residual liver, R2* is 65.2 Hz, 69.7 Hz, 63.9 Hz, respectively (circles); 1G: PDFF quantification of residual liver in January 2022, PDFF is 3.46%, 2.19%, 6.69%, respectively (circle); 1H: R2* quantification of residual liver in January 2022, R2* is 54.3 Hz, 52.1 Hz, 78.1Hz, respectively (circles); 1I-1K: Pathological images of ring enhancement foci in December 2020 (HE ×100), immunohistochemical staining (CK+ ×40), immunohistochemical staining (Syn+ ×40), respectively.

讨论

       James·Paget在1874年首次报道乳头周围湿疹样病变与潜在的乳房恶性肿瘤相关,将其命名为佩吉特病[1],后来将乳腺外皮肤类似病变命名为乳房外佩吉特病(extramammary Paget's disease, EMPD)。EMPD是一种少见的皮肤低度恶性肿瘤,其病理特征为病变组织内发现表皮腺癌细胞(Paget样细胞)[2]。EMPD多分布于皮肤汗腺分泌发达的部位,以外生殖器和肛周为常见部位,但腋窝较为少见[3, 4]。此例男性患者最初以腋窝肿块就诊,病理诊断为EMPD,术后发生肝转移,遂行肝转移瘤切除,术后病理支持转移性乳腺癌伴神经内分泌分化。

       EMPD生长缓慢,可无症状或呈现不同程度的灼热感和瘙痒,由于其症状非特异性和病程进展缓慢,常被误诊,此例患者最初被误诊为“皮肤软组织感染”。EMPD的病因尚未明确,可分为原发性和继发性,原发性通常局限于表皮,继发性与毗邻恶性肿瘤的延伸或扩散有关[5]。大多数EMPD病例早期病理为原位癌,一旦发生转移,往往表现出很强的侵袭性特征[6]。继发性EMPD最常见起源于消化系统或泌尿系统的潜在肿瘤,临床罕见,Yamaura等[7]报道了一例以神经内分泌为特征的肛管腺癌伴继发性EMPD。Cao等[8]报道了一例肛周佩吉特病并肝转移瘤。原发性EMPD可发展为侵袭性腺癌,具有向区域淋巴结和远处器官转移的内在潜力[7]。此例患者病理提示病变浸润真皮深层,伴神经内分泌分化,故较易转移。

       研究表明,放化疗可引起肝脂肪变性及铁沉积[9, 10],肿瘤出现远处转移也与体内铁等物质代谢异常相关,特别会引起铁在肝脏异常沉积[11]。近年来肝脏多参数MRI定量技术的发展为肝脏脂肪变性及铁沉积的无创精准测量提供了可能,目前已推向市场的肝脏MRI脂肪及铁定量的多回波水脂分离技术序列有Q-Dixon、IDEAL-IQ,使用联影uMR790 MRI仪器运用FACT序列的PDFF及R2*可对患者的脂肪变性及铁沉积情况进行较准确的评估[12, 13]。MRI通过PDFF测定可直接量化肝脏脂肪,PDFF定义为脂肪的信号强度与脂肪和水的总信号的比值,利用MRI化学位移编码(chemical shift encoding, CSE)技术所得的PDFF是目前公认的可较精准地反映肝脏脂肪变性的定量参数;R2*在反映肝脏铁沉积上较为直观,我们用该技术对此例EMPD伴肝转移患者进行追踪复查。为减少测量误差,我们在肝实质近似相同层面避开大血管的相似区域选取三个感兴趣区进行测量并取其平均值,发现规律化疗后肝转移灶逐渐缩小,同时,肝脏非肿瘤区肝实质的平均PDFF及R2*呈现降低趋势,并没有因持续放化疗而增高,这提示我们用MRI定量技术去无创追踪观察肝内脂肪及铁的沉积变化有可能间接反映肝脏转移瘤的治疗疗效,后期将加大样本量进一步探索。

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