[摘要]目的 探讨肝脏局灶性结节性增生(FNH)的临床病理学特点、病因、治疗及预后。方法 收集2013年5月~2017年8月我院收治的5例FNH患者的临床资料,观察其病理学形态及免疫组化染色,并结合国内外最新文献对该病变的特点进行总结分析。结果 典型的FNH大体有特征性的中央瘢痕,镜下见肿块由结节状增生的肝细胞构成,结节内肝细胞呈1~2层排列,结节间由杂乱的纤维分隔,肝细胞无明显异型。不典型FNH需要与肝细胞腺瘤、肝细胞癌鉴别。结论 FNH是一种少见的良性病变,临床切除后,预后良好。
http://
[关键词]肝局灶性结节性增生;肝细胞腺瘤;肝细胞癌
[中图分类号] R575 [文献标识码] A [文章编号] 1674-4721(2017)12(c)-0013-04
[Abstract]Objective To explore the clinicopathologic character,pathogen,treatment and prognosis of liver focal nodular hyperplasia (FNH).Methods The clinical data of 5 patients with FNH of the liver from May 2013 to August 2017 in our hospital were collected.The pathological morphology and immunohistochemical staining were observed.The clinical features of FNH were analyzed by review of the latest foreign and chinese literature.Reslults Macroscopically,characteristic center scar was found in classic type of FNH.Microscopically,nodes composed of heptocytes without atypia weres divided by fibrosis septa.The liver plate was consisted of one or two layer of hepatocyts.Non-classical type of FNH should be distinguished from hepatic adenoma and highly differentiated hepatic cancer.Conclusion FNH was a rare benign lesion occurred in liver,which had good prognosis after clinical excision.
[Key words]Focal nodular hyperplasia;Heptic adenoma;Hepatic cancer
肝脏局灶性结节性增生(focal nodular hyperplasia,FNH)是较少见的肝脏良性病变,发生率约为0.8%[1]。因术前诊断困难,本文回顾性分析我院2013年5月~2017年8月收治的5例FNH的临床病理资料,结合国内外最新文献,对其病理诊断、鉴别诊断、病因、治疗及预后进行总结,以提高临床和病理医生对FNH的认识。
1资料与方法
1.1一般资料
病例的临床信息均通过查阅病案资料获得,并与临床医师及患者核实。随访资料通过电话联系。
1.1.1病例1 患者,女,41岁,左上腹疼痛半月余,伴腰部?可嫱矗?胃纳差,乏力,体重减轻,于2013年5月就诊于我院。腹部CT检查示脾脏占位,恶性可能,肝肾囊肿。术中见脾脏肿瘤10 cm×10 cm大小,球状,侵犯胃结肠韧带、前腹壁,肝左外叶见黄色小结节,行脾脏及肿瘤+肝左外叶小结节切除术。术后病理示(脾脏)弥漫大B细胞淋巴瘤,(肝左外叶)局灶性结节性增生。患者术后行化疗,随访至今,病情稳定,肝脏结节无复发。
1.1.2病例2 患者,男,28岁,体检B超发现肝占位2年,考虑血管瘤可能,自觉无明显症状。未予重视,第2年体检B超提示占位有所增大,进一步检查CT,示肝左内叶4.9 cm×3.9 cm大小占位,考虑血管瘤可能性大,局灶性结节状增生不排除。三月余前再次B超检查提示:肝左内叶等回声区,大小约54 mm×36 mm,考虑肝内血管瘤可能,于2015年10月就诊我院。全身麻醉下行肝左外叶切除术,术后病理示肝左外叶局灶性结节状增生,汇管区小胆管增生,炎细胞浸润。于2017年9月26日随访,体健。
1.1.3病例3 患者,男,65岁,右上腹隐痛伴便血8 d,2016年5月19日就诊于我院,门诊结肠镜示腺瘤性息肉。CT示肝血管瘤,肝囊肿,右肾囊肿,肝膈下低密度灶,转移灶可能。MRI提示肝左叶及右叶多发血管瘤,肝左内叶包膜下见异常信号,转移性恶性肿瘤可能。于2016年5月20日入我院治疗,复查肠镜,病理示绒毛状腺瘤伴低级别上皮内瘤变。于5月27日全身麻醉腹腔镜下行左半结肠+肝左内叶部分切除术,术后病理示左半结肠腺瘤伴腺体中-重度不典型增生,肝组织脂肪变性伴局灶结节状增生。于2017年9月20日随访,体健,无明显不适。
1.1.4病例4 患者,女,64岁,右上腹持续性隐痛不适1个月,剑突下为主,不放射,无锡中医院查胃镜,示贲门炎。B超提示肝右叶见一异常回声区,37 mm×32 mm,肝实质点状回声不均匀增粗,CT示肝右叶类圆形占位,边缘强化,恶性肿瘤?肝右叶囊肿?于2016年7月就诊我院,全身麻醉下行“肝癌根治术”,术后病理示肝局灶性结节性增生。于2017年9月21日随访,体健,一般状况良好。 1.1.5病例5 患者,女,37?q,常规体检发现肝左叶占位4 d,复旦大学附属中山医院行MRI提示,肝左外叶见一圆形异常信号灶,T1W1为等低信号,超T2W1为等高信号,边界欠清,大小5.5 cm×4.3 cm,动态增强后病灶大部早期明显强化,中央有星形强化阴影,诊断提示:FNH。于2017年8月22日收治我院,腹腔镜下行肝部分切除+肝肿瘤切除术,术后病理示,肝局灶性结节状增生。于2017年9月26日电话随访,患者情况良好。
1.2研究方法
收集江南大学附属医院2013年5月~2017年8月肝胆外科肝脏占位手术切除、术后病理科诊断为肝局灶性结节性增生的5例标本,所有病例资料经由查阅病案获得,随访数据由电话联系患者获得。手术标本经10%中性缓冲甲醛固定过夜(>6 h,致谢:特别感谢复旦大学中山医院病理科尚果果友情检测GS。
[参考文献]
[1]Wanless IR,Albrecht S,Bilbao J,et al.Multiple focal nodular hyperplasia of the liver associated with vascular malformations of various organs and neoplasia of the brain:a new syndrome[J].Mod Pathol,1989,2(5):456-462. [2]Ji Y,Chen S,Xiang B,et al.Clinical features of focal nodular hyperplasia of the liver in children[J].J Pediatr Gastroenterol Nutr,2016,62(6):813-818.
[3]Ma IT,Rojas Y,Masand PM,et al.Focal nodular hyperplasia in children:an institutional experience with review of the literature[J].J Pediatr Surg,2015,50(3):382-387.
[4]Romano A,Grassia M,Esposito G,et al.An unusual case of left hepatectomy for focal nodular hyperplasia (FNH) linked to the use of anabolic androgenic steroids (AASs)[J].Int J Surg Case Rep,2017,30:169-171.
[5]Ijichi H,Taketomi A,Yoshizumi T,et al.Hyperbaric oxygen induces vascular endothelial growth factor and reduces liver injury in regenerating rat liver after partial hepatectomy[J].J Hepatol,2006,45(1):28-34.
[6]Sato Y,Harada K,Ikeda H,et al.Hepatic stellate cells are activated around central scars of focal nodular hyperplasia of the liver-a potential mechanism of central scar formation[J].Hum Pathol,2009,40(2):181-188.
[7]Nguyen BN,Flejou JF,Terris B,et al.Focal nodular hyperplasia of the liver:a comprehensive pathologic study of 305 lesions and recognition of new histologic forms[J].Am J Surg Pathol,1999,23(12):1441-154.
[8]?S成,孙惠川.2016年欧洲肝病学会临床实践指南:肝脏良性肿瘤的管理[J].临床肝胆病杂志,2016,32(8):1439-1445.
[9]Perrakis A,Vassos N,Grutzmann R,et al.What is Changing in Indications and Treatment of Focal Nodular Hyperplasia of the Liver.Is There Any Place for Surgery?[J].Ann Hepatol,2017,16(3):333-341.
[10]Rao PN.Nodule in liver:investigations,differential diagnosis and follow-up[J].J Clin Exp Hepatol,2014,4(Suppl 3):S57-S62.
[11]Roux M,Pigneur F,Calderaro J,et al.Differentiation of focal nodular hyperplasia from hepatocellular adenoma:role of the quantitative analysis of gadobenate dimeglumine-enhanced hepatobiliary phase MRI[J].J Magn Reson Imaging,2015,42(5):1249-1258.
[12]Tajiri K,Tsuneyama K,Kawai K,et al.A case of progressing focal nodular hyperplasia and its molecular expression pattern[J].Clin J Gastroenterol,2014,7(3):271-277.
[13]Roncalli M,Sciarra A,Tommaso LD.Benign hepatocellular nodules of healthy liver:focal nodular hyperplasia and hepatocellular adenoma[J].Clin Mol Hepatol,2016,22(2):199-211.
[14]Paradis V,Laurent A,Flejou JF,et al.Evidence for the polyclonal nature of focal nodular hyperplasia of the liver by the study of X-chromosome inactivation[J].Hepatology,1997,26(4):891-895.
[15]Bioulac-Sage P.Angiopoietines play a physiopathological role in focal nodular hyperplasia[J].Gastroenterol Clin Biol,2004,28(2):200-201.