ACM  >> Vol. 10 No. 3 (March 2020)

    早期原发性肝脏肉瘤样癌1例报告
    Case Report of Primary Liver Sarcomatous Carcinoma at Early-Stage

  • 全文下载: PDF(696KB) HTML   XML   PP.284-288   DOI: 10.12677/ACM.2020.103045  
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作者:  

鲍 利,黄佳程,陈芝涛,万大龙,林胜璋:浙江大学医学院附属第一医院肝胆胰外科,浙江 杭州

关键词:
肉瘤样癌肝脏乙型肝炎病毒预后Sarcomatous Carcinoma Liver Hepatitis B Virus Prognosis

摘要:

肝脏肉瘤样癌临床上少见,目前病因不明,可能与乙型肝炎病毒感染和术前抗癌治疗有关。患者临床表现和影像学表现缺乏特征性,术前诊断此病比较困难,确诊依赖病理学和免疫组织化学检查。肝脏肉瘤样癌预后差,缺乏有效的治疗方法,目前手术仍是有望延长患者生存时间的治疗方法。本研究报道了1例预后相对较好的早期原发性肝脏肉瘤样癌。

Liver sarcomatous carcinoma is a rare malignant tumor. The pathogenesis remains unknown, hepatitis B virus infection and anti-cancer therapy may be the potential cause of this disease. The atypical clinical symptom and image feature of this disease make it difficult to be accurately diag-nosed without pathology and immunohistology. Liver sarcomatous carcinoma has a poor prognosis and lacks effective treatments. At present, surgery is still a treatment that is expected to extend the survival time of patients. This study reported a rare case of primary liver sarcomatous carcinoma at early-stage with relatively better prognosis.

1. 引言

肉瘤样癌是一种以恶性上皮和间充质成分同时存在为特征的肿瘤,比较少见,可发生于许多脏器,常见于上呼吸道,甲状腺,肺,乳腺,消化道,膀胱和肾脏等 [1]。肝脏肉瘤样癌是发生于肝脏的一种少见的恶性肿瘤,发病的平均年龄在50岁以上,约占所有肝脏恶性肿瘤的0.2% [2]。文献报道肝脏肉瘤样癌的发生与病毒感染和抗癌治疗有关,根据患者术前是否有抗癌治疗经历,可将肝脏肉瘤样癌分成原发性,继发性和混合性三类 [3] [4] [5]。本病进展速度快,术后复发和转移的风险高,预后差,大多数患者在就诊后一年内死亡 [6]。本研究报道一例预后相对较好的早期肝脏肉瘤样癌。

2. 病例报道

患者男,49岁,因“乏力半年,加重8天”入院。入院查体:生命体征平稳,皮肤巩膜无黄染;心肺查体未见异常;腹部稍膨隆,软,无压痛、反跳痛及肌紧张,肝肋下未触及,轻度脾大,肝区无明显叩击痛, 移动性浊音阳性,肠鸣音正常存在,双下肢无水肿。既往病史包括乙型肝炎病史20年,未接受规范的抗病毒治疗。实验室检查:HBsAg阳性;肝功能ALT 53 U/L (5~40 U/L),ALB 25.4 g/L(35~55 g/L);AFP正常水平;血常规,凝血功能及其他肿瘤标志物均未见明显异常。腹部B超示:右肝混合回声团块,恶性肿瘤首先考虑(图1)。上腹部增强CT可见肿瘤位于肝右叶,直径3 cm,肿瘤内部有坏死,动脉期结节状强化,静脉期延迟强化(图2)。诊断为:1) 右肝占位性病变,肝癌首先考虑;2) 肝硬化、腹水、脾大。结合患者乙肝病史和影响学检查结构,考虑为不典型肝癌伴乙肝肝硬化,肝功能A级。患者入院后第5天行右肝部分切除术,肿瘤位于第V,第VIII段,未见其它脏器转移。术后病理示:肝脏肉瘤样癌(图3)。免疫组织化学染色结果显示:CK(+)、Vim(+)、AFP(-)、CD34(+),符合肝脏肉瘤样癌(图4)。患者术后每半年复查B超、CT或者MRI,术后第30个月复查,未见肿瘤复发及转移征象。

3. 讨论

肝脏肉瘤样癌是一种包含癌样和肉瘤样双相成分的恶性肿瘤,约占所有肝脏恶性肿瘤的0.2%。由于肝脏肉瘤样癌比较少见,国内外关于该疾病的文献以个案报道居多。该病的病因至今尚未明确,可能与病毒感染和术前抗癌治疗有关,根据患者术前是否有抗癌治疗经历,可将肝脏肉瘤样癌分成原发性,继发性和混合性三类。本研究报道的病例术前未接受过任何抗癌治疗,肿瘤的发生可能与乙肝病毒感染有关,属于原发性肝脏肉瘤样癌。

肝脏肉瘤样癌具有很强的侵袭性,病程进展较快,大多数患者发现时已经处于晚期,因而错失手术治疗的最佳时期 [7]。该病患者临床表现多不典型,可表现为乏力、消瘦,也可有腹部疼痛不适、腹胀、食欲下降等消化系统症状,APF等肿瘤标志物对该病的提示作用不大 [8]。CT下表现具有肉瘤和普通类型癌的双重特征,由于生长速度快,体积一般较大,滋养血管难以供血,肿瘤内部常常出现坏死灶,平扫期呈不均匀低密度影,囊实性多见;动脉期肿瘤实体部分呈环状或者结节状强化,呈快进快出、延迟强化型,对诊断有一定提示作用 [9]。然而临床上只有部分患者表现出这种典型的影像学特征,术前诊断肝脏肉瘤样癌相对困

Figure 1. Abdominal ultrasonography

图1. 腹部B超

Figure 2. Abdominal enhanced CT A: In the plain scan, the tumor presented a low-density lesion (arrow) with a lower density necrotic area. B: The tumor presented nodular enhancement in the arterial phase except the necrotic area. C: The enhancement prolonged in the portal vein phase. D: The enhancement faded away in the equilibrium phase

图2. 腹部增强CTA:平扫期肿瘤呈低密度灶(箭头所指),内部可见密度低于肿瘤实质的坏死区域;B:动脉期肿瘤呈结节状强化,坏死区域不强化;C:门静脉期肿瘤仍有强化。D:平衡期肿瘤强化减退

Figure 3. Specimen seen under the microscope A: Liver sarcomatous carcinoma, with obvious atypia of tumor cells and multinucleated tumor giant cells (×400); B: The tumor cells presented active growth with pathological mitotic phase visible (×400); C: The tumor cells were spindle-shaped and short spindle-shaped in some areas (×400); D: The tumor cells were diffusely distributed, showing invasive growth, and residual liver cells can be seen (×100)

图3. 标本镜下所见A:肝脏肉瘤样癌,肿瘤细胞异型性明显,可见多核瘤巨细胞(×400);B:细胞生长活跃,可见病理性核分裂相(×400);C:部分区域肿瘤细胞呈梭形和短梭形(×400);D:肿瘤细胞弥漫成片分布,呈浸润性生长,可见残留的肝细胞(×100)

Figure 4. Immunohistology findings The tumor cells were diffusely positive for CK and Vimentin, negative for AFP(×400). A: CK8; B: CK7; C: Vimentin; D: AFP

图4. 免疫组化结果 肿瘤细胞呈CK和Vimentin强阳性,AFP阴性(×400)。A:CK8;B:CK7;C:Vimentin;D:AFP

难,需要结合病理学和免疫组织化学检查确诊。肝脏肉瘤样癌的病理学特点为同时包含恶性上皮成分和肉瘤样成分,并且两者之间存在一定的移行区域,但也有文献报道过完全由肉瘤样成分构成的“纯粹”肝脏肉瘤样癌,分化程度低 [10]。免疫组织化学染色复杂多样,以同时表达上皮性肿瘤标记物(CK, EMA)和间叶性肿瘤标记物(Vimentin)为特点,但是部分肝脏肉瘤样癌可能缺乏Vimentin表达,而表达DES、Actin等 [11]。本例患者发病年龄相对较小,仅有乏力症状,AFP在正常范围内,影像学表现并不典型,但肿瘤内部有坏死,术前诊断为不典型肝癌。术后病理学检查和免疫组织化学检查结果与文献报道一致,确诊为肝脏肉瘤样癌。

目前还没有关于治疗肝脏肉瘤样癌的特定指南,手术治疗包括外科切除和肝移植是目前唯一有望延长患者生存时间的治疗方法,放化疗,消融等介入治疗的效果难以肯定,靶向治疗鲜有报道。对于接受手术治疗的患者,肿瘤分期和手术切缘是影响术后患者生存时间的危险因素,I期(AJCC癌症分期)患者的术后长期生存时间明显高于II期、III期和IV期患者 [12] [13]。本例患者处于I期,根治性切除肿瘤,因此无复发生存期相对较长,预后相对较好。

4. 结论

综上所述,肝脏肉瘤样癌恶性程度高,进展速度快,平均发病年龄在50岁以上,预后差。乙型肝炎病毒感染的患者有一定几率发生肝脏肉瘤样癌,临床上对于年龄较大,病毒感染时间长的乙型肝炎患者有一定的必要行影像学检查,以期早期发现此病和及时的手术治疗,提高患者生存时间。

本病例报道已获得患者家属知情同意。

文章引用:
鲍利, 黄佳程, 陈芝涛, 万大龙, 林胜璋. 早期原发性肝脏肉瘤样癌1例报告[J]. 临床医学进展, 2020, 10(3): 284-288. https://doi.org/10.12677/ACM.2020.103045

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