宫颈大细胞神经内分泌癌一例

2020-05-26 陈晨 程敬亮 罗乐凯 临床放射学杂志

患者女,29岁,5个月前行“剖宫产术”,产一男活婴,恶露持续时间52天;2个月前无明显诱因出现阴道不规则出血,量少,色鲜红,伴褐色,无腹痛、腹胀、发热等不适,无同房接触性出血史

患者女,29岁,5个月前行“剖宫产术”,产一男活婴,恶露持续时间52天;2个月前无明显诱因出现阴道不规则出血,量少,色鲜红,伴褐色,无腹痛、腹胀、发热等不适,无同房接触性出血史。患者于7天前至当地医院就诊,TCT提示:1.炎症反应性细胞改变;2.可见不典型细胞,疑上皮内病变;HPV18(+)。行宫颈赘生物活检术,病理提示:宫颈非角化型鳞癌;HPV18(+)。患者为进一步治疗来我院就诊。妇科检查:宫颈后唇可见直径约2 cm病灶,触血阳性;三合诊右侧骶主韧带缩短,弹性可,左侧骶主韧带未触及异常。余体格检查未见明显异常。

MRI检查:子宫体积形态正常。宫颈后壁肌层增厚,内可见团块状长T1、稍长T2信号(图1、2),DWI上呈稍高信号(图3),病变范围约3.1 cm×2.0 cm×3.7 cm,未见阴道明显受累。S3椎体及骶管内可见片状长T2信号。静脉注入Gd-DTPA增强扫描,宫颈后壁病灶呈明显不均匀强化,延迟期强化程度稍下降,呈略高信号(图4、5),时间-信号强度曲线呈流出型(图6)。CT检查:肝脏内可见多发大小不等类圆形低密度影,边界模糊,增强轻度强化;右肺中下叶可见多发结节。


图1矢状位T1WI示肿块位于宫颈后壁肌层,呈长T1信号(↑);图2矢状位T2WI示肿块位于宫颈后壁肌层,呈稍长T2信号(↑),S3椎体及骶管内也可见片状长T2信号;图3DWI上病灶呈稍高信号;图4轴位增强T1WI示宫颈后壁病灶呈明显不均匀强化;图5轴位增强T1WI延迟期病灶强化程度稍下降,呈略高信号;图6时间-信号强度曲线呈流出型

手术及病理:患者行腹腔镜下子宫广泛切除+双侧附件移位+盆腔淋巴结清扫术,术后将切除的子宫及盆腔双侧淋巴结组织送常规病理检查。镜下可见肿瘤细胞大,排列紧密呈小梁状,胞质丰富,并可见“地图”样坏死灶。免疫组织化学检查:CK、CD56、Syn(+),CK5/6、P40、P63(-),Ki-67(约70%+)。病理诊断:(宫颈)大细胞神经内分泌癌(图7)。


图7病理图。镜下可见肿瘤细胞大,排列紧密呈小梁状,胞质丰富,并可见“地图”样坏死灶(HE×200)

讨论

宫颈大细胞神经内分泌癌(large cell neuroendocrine carcinoma,LCNEC)是一种罕见的宫颈神经内分泌肿瘤,约占宫颈原发肿瘤的0.087%~0.6%,恶性程度极高,在早期就可发生血行转移和淋巴转移,术后复发率高。有文献报道,LCNEC的发生与高危型人乳头状病毒(HPV)感染有关,尤其是HPV18型。本例患者HPV18(+),在肝脏和右肺上发现了多个转移病灶,S3椎体及骶管也可见异常信号,并伴有盆腔淋巴结的转移。

LCNEC病理特征为肿瘤细胞大,排列紧密,胞质丰富,核仁明显,有丝分裂频繁,肿瘤细胞核分裂象活跃(>10个/10HPF),并伴有广泛的坏死。免疫组织化学检查可以发现神经内分泌标记物,如CK、CD56、Syn、CgA和NSE等。本例患者CK、CD56、Syn均(+)。LCNEC临床表现无特异性,主要表现为阴道异常出血。LCNEC罕见,影像描述较少。

曾裕镜等认为肺外神经内分泌癌常为单发实性肿瘤,MRI表现为混杂长T1、混杂长T2信号,增强扫描动脉期肿瘤明显不均匀强化,门静脉期仍呈高信号。薛鹏等也认为动脉期不均匀明显强化、延迟期仍表现轻中度强化可作为肺外神经内分泌癌的重要特征。张弦等认为在宫颈癌进行动态增强时,时间-信号强度曲线呈快速上升及快进慢出型,延迟扫描时肿瘤边缘显示清晰,对肿瘤检出的意义较大。

本例MRI增强检查中肿瘤呈明显不均匀强化,延迟期强化程度稍下降,呈略高信号,时间-信号强度曲线流出型。笔者认为上述影像表现对于LCNEC的术前诊断有一定的价值,在诊断时还需与小细胞神经内分泌癌、低分化腺癌、黑色素瘤等相鉴别。最终确诊仍需靠病理和免疫组织化学检查。

原始出处:

陈晨,程敬亮,罗乐凯.宫颈大细胞神经内分泌癌一例[J].临床放射学杂志,2019,38(02):250-251.

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    2020-05-28 一闲

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