小儿局灶性结节性肝脏增生临床诊治经验探讨
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摘要
目的探讨小儿局灶性结节性肝脏增生的临床特点、诊断方法及治疗经验,提高其诊治水平。方法回顾性分析2006年1月至2018年1月由首都医科大学附属北京儿童医院经手术切除及病理检查证实为局灶性结节性肝脏增生的患儿临床资料,其中男童9例,女童13例,发病年龄7个月至11岁1个月,中位年龄4岁6个月,所有病灶为单发,均经不规则性肝切除术治疗。结果临床表现:22例局灶性结节性肝脏增生(focal nodular hyperplasia,FNH)患儿中腹痛7例,腹部膨隆或包块4例,体检发现11例;实验室检验:肝功异常8例,AFP升高3例,均于术后恢复正常;影像学检查:所有22例均行超声检查,10例拟诊断为FNH,拟诊断正确率为45. 5%;误诊为肝脏血管瘤5例,其中肝母细胞瘤1例,间叶错构瘤1例。20例行CT(含增强)检查,11例拟诊断为FNH,拟诊断正确率为55%。3例术前行MRI检查,1例拟诊断为FNH,2例未予明确诊断。术后肿瘤最长径线5～15 cm,中位长度8 cm,其中≥10 cm者5例,最大肿物体积为15 cm×10 cm×8 cm。所有病例为单发病灶,均行手术切除,病理提示肿物中央灰白色放射样瘢痕特征11例(50%)。术后随访0. 5～10. 8年,未见复发或严重并发症发生。结论小儿局灶性结节性肝脏增生在临床及影像学上有一定特征,联合应用CT等影像学检查和AFP水平及肝功能实验室检查指标能提高其诊断水平,最终确诊需术后病理检查,手术切除治疗能够有效解除病灶,远期预后良好。
Objective To review the clinical features and management experiences of children with focal nodular hyperplasia( FNH) of liver. Methods A review of medical records was conducted for 22 children pathologically diagnosed as FNH between January 2006 and January 2018. There were 9 boys and 13 girls with a median age of 54( 7 ～ 133) months. All lesions were single and surgical resections performed. Results Clinical manifestations included abdominal pain( n = 7),abdominal bulge or mass( n = 4) and detection by physical examination( n = 11). And laboratory findings of abnormal liver function( n = 8) and elevated alpha fetoprotein( AFP,n = 3) were both restored postoperatively. Ultrasonography confirmed the diagnosis of FNH( n =10) with a correct rate of 45. 5%. The misdiagnoses were hepatic hemangioma( n = 5),hepatoblastoma( n =1) and mesenchymal hamartoma( n = 1). Plain and enhanced computed tomography( CT) revealed 11/20 cases of FNH with a correct rate of proposed diagnosis at 55%. Three cases received preoperative magnetic resonance imaging( MRI). One case was diagnosed definitely as FNH while another two were diagnosed non-definitely. The longest diameters of tumor were 5 to 15 cm with a median length of 8 cm. The maximal diameter of tumor was > 10 cm( n = 5) and the volume of the largest mass 15 cm × 10 cm × 8 cm. Central fibrovascular scar was found( n = 11,50%). After a follow-up period of 0. 5 to 10. 8 years,all cases survived without recurrence or serious complications. Conclusion The clinical and imaging features of FNH in children have some characteristics. The combined application of imaging methods,AFP and liver function test may improve the diagnostic accuracy. A definite diagnosis is dependent upon postoperative pathology examination and surgical resection can effectively remove the lesion.
Objective To review the clinical features and management experiences of children with focal nodular hyperplasia( FNH) of liver. Methods A review of medical records was conducted for 22 children pathologically diagnosed as FNH between January 2006 and January 2018. There were 9 boys and 13 girls with a median age of 54( 7 ～ 133) months. All lesions were single and surgical resections performed. Results Clinical manifestations included abdominal pain( n = 7),abdominal bulge or mass( n = 4) and detection by physical examination( n = 11). And laboratory findings of abnormal liver function( n = 8) and elevated alpha fetoprotein( AFP,n = 3) were both restored postoperatively. Ultrasonography confirmed the diagnosis of FNH( n =10) with a correct rate of 45. 5%. The misdiagnoses were hepatic hemangioma( n = 5),hepatoblastoma( n =1) and mesenchymal hamartoma( n = 1). Plain and enhanced computed tomography( CT) revealed 11/20 cases of FNH with a correct rate of proposed diagnosis at 55%. Three cases received preoperative magnetic resonance imaging( MRI). One case was diagnosed definitely as FNH while another two were diagnosed non-definitely. The longest diameters of tumor were 5 to 15 cm with a median length of 8 cm. The maximal diameter of tumor was > 10 cm( n = 5) and the volume of the largest mass 15 cm × 10 cm × 8 cm. Central fibrovascular scar was found( n = 11,50%). After a follow-up period of 0. 5 to 10. 8 years,all cases survived without recurrence or serious complications. Conclusion The clinical and imaging features of FNH in children have some characteristics. The combined application of imaging methods,AFP and liver function test may improve the diagnostic accuracy. A definite diagnosis is dependent upon postoperative pathology examination and surgical resection can effectively remove the lesion.
引文
1 Von SD.Management of liver tumors in childhood[J].Semin Pediatr Surg,2006,15(1):17-24.DOI:10.1053/j.sempedsurg.2005.11.004.
2 Nguyen BN,Fléjou JF,Terris B,et al.Focal nodular hyperplasia of the liver:a comprehensive pathologic study of 305lesions and recognition of new histologic forms[J].Am JSurg Pathol,1999,23(12):1441.
3 Luca GD,Zamparelli M,Fadda C,et al.Focal nodular hyperplasia of the liver in infancy:a case report[J].J Pediatr Surg,2006,41(2):456.DOI:10.1016/j.jpedsurg.2005.11.026.
4 Masetti R,Zama D,Gasperini P,et al.Focal nodular hyperplasia of the liver in children after hematopoietic stem cell transplantation[J].Pediatr Transplant,2013,17(5):479-486.DOI:10.1111/petr.12091.
5 Scalori A,Tavani A,Gallus S.Oral contraceptives and the risk of focal nodular hyperplasia of the liver:a case-control study[J].Am J Obstet Gynecol,2002,186(2):195-197.
6 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.DOI:10.1016/j.jpedsurg.2014.06.016.
7 Nguyen BN,Fléjou JF,Terris B,et al.Focal nodular hyperplasia of the liver:a comprehensive pathologic study of 305lesions and recognition of new histologic forms[J].Am JSurg Pathol,1999,23(12):1441.
8 Yang Y,Fu S,Li A,et al.Management and surgical treatment for focal nodular hyperplasia in children[J].Pediatr Surg Int,2008,24(6):699-703.DOI:10.1007/s00383-008-2150-8.
9 Hussain SM,Terkivatan TZ,Ondervan PE,et al.Focal nodular hyperplasia:findings at state-of-the-art MR imaging,US,CT and pathologic analysis[J].Radiographics,2004,24(1):3-17.DOI:10.1148/rg.241035050.
10 Towbin AJ,Luo GG,Yin H,et al.Focal nodular hyperplasia in children,adolescents and young adults[J].Pediatr Radiol,2011,41(3):341-349.DOI:10.1007/s00247-010-1839-8.
11 Benz-Bohm G,Hero B,Gossmann A,et al.Focal nodular hyperplasia of the liver in longterm survivors of neuroblastoma:how much diagnostic imaging is necessary?[J].Eur J Radiol,2010,74(3):e1-e5.DOI:10.1016/j.ejrad.2009.03.011.
12 Ungermann L,Eliás P,Zizka J,et al.Focal nodular hyperplasia:spoke-wheel arterial pattern and other signs on dynamic contrast-enhanced ultrasonography[J].Eur J Radiol,2007,63(2):290.DOI:10.1016/j.ejrad.2007.01.026.
13孙健,王捷.肝脏局灶性结节性增生诊断及规范化治疗[J].中国实用外科杂志,2013,33(9):742-745.Sun J,Wang J.Diagnosis and standardized management of hepatic focal nodular hyperplasia[J].Chin J Pract Surg,2013,9(33):742-745.
14 Cha DI,Yoo SY,Kim JH,et al.Clinical and imaging features of focal nodular hyperplasia in children[J].Ajr Am JRoentgenol,2014,202(5):960-965.DOI:10.2214/AJR.13.11856.
15 Trillaud H,Bruel JM,Valette PJ,et al.Characterization of focal liver lesions with Sono Vue-enhanced sonography:international multicenter-study in comparison to CT and MRI[J].World J Gastroenterol,2009,15(30):3748-3756.
16阎洁羽,刘凤永,王茂强.肝脏局灶性结节增生影像表现及研究进展[J].中国医学影像学杂志,2012,20(8):635-637.DOI:10.3969/j.issn.1005-5185.2012.08.023.Yan JY,Liu FY,Wang MQ.Imaging findings and research advances of hepatic focal nodular hyperplasia[J].Chin JMed Imag,2012,20(8):635-637.DOI:10.3969/j.issn.1005-5185.2012.08.023.
17 Bartolotta TV,Taibbi A,Brancatelli G,et al.Imaging findings of hepatic focal nodular hyperplasia in men and women:are they really different?[J].Radiol Med,2014,119(4):222-230.DOI:10.1007/s11547-013-0333-4.
18 Kuo PH,Lai HS,Huang SY,et al.Focal nodular hyperplasia of the liver in an 8-year-old boy[J].Surgery,2007,142(3):422-423.DOI:10.1016/j.surg.2006.10.011.
19汪洵理,靳曙光,杨周建,等.小儿肝脏局灶性结节状增生临床分析[J].临床小儿外科杂志,2012,11(4):292-293.DOI:10.3969/j.issn.1671-6353.2012.04.017.Wang XL,Jin SG,Yang ZJ,et al.Clinical analysis of focal nodular hyperplasia of liver in children[J].J Clin Ped Sur,2012,11(4):292-293.DOI:10.3969/j.issn.1671-6353.2012.04.017.
20沈英皓,樊嘉,吴志全,等.肝脏局灶性结节性增生60例临床分析[J].中华普通外科杂志,2005,20(7):397-399.DOI:10.3760/j.issn:1007-631X.2005.07.003.Shen YH,Fan J,Wu ZQ,et al.Focal nodular hyperplasia of the liver:a report of 60 cases[J].Chin J Gener Surg,2005,20(7):397-399.DOI:10.3760/j.issn:1007-631X.2005.07.003.
21 Cristiano A,Dietrich A,Spina JC,et al.Focal nodular hyperplasia and hepatic adenoma:current diagnosis and management[J].Updates Surg,2014,66(1):9-21.DOI:10.1007/s13304-013-0222-3.
22 Mcinnes MD,Hibbert RM,Inácio JR,et al.Focal nodular hyperplasia and hepatocellular adenoma:accuracy of gadoxetic acid-enhanced MR imaging-a systematic review[J].Radiology,2015,277(2):142986.DOI:10.1148/radiol.2015142986.
23 Muguti G,Tait N,Richardson A,et al.Hepatic focal nodular hyperplasia:a benign incidentaloma or a marker of serious hepatic disease?[J].HPB Surg,1992,5(3):171.
24 Gong Y,Chen L,Qiao ZW,et al.Focal nodular hyperplasia coexistent with hepatoblastoma in a 36-d-old infant[J].World J Gastroenterol,2015,21(3):1028-1031.DOI:10.3748/wjg.v21.i3.1028.
25 Petsas T,Tsamandas A,Tsota I,et al.A case of hepatocellular carcinoma arising within large focal nodular hyperplasia with review of the literature[J].World J Gastroenterol,2006,12(40):6567-6571.
26 Makhlouf HR,Abdul-Al HM,Goodman ZD.Diagnosis of focal nodular hyperplasia of the liver by needle biopsy[J].Hum Pathol,2005,36(11):1210-1216.DOI:10.1016/j.humpath.2005.08.014.
27祁付珍,吴健雄,邵永孚,等.肝脏局灶性结节增生的诊断及治疗[J].实用癌症杂志,2004,19(5):501-503.DOI:10.3969/j.issn.1001-5930.2004.05.017.Qi FZ,Wu JX,Shao YF,et al.Diagnosis and treatment of focal nodular hyperplasia of liver[J].Pract J Canc,2004,19(5):501-503.DOI:10.3969/j.issn.1001-5930.2004.05.017.
28 Geschwind JF,Degli MS,Morris JM,et al.Re:Treatment of focal nodular hyperplasia with selective transcatheter arterial embolization using iodized oil and polyvinyl alcohol[J].Cardiovasc Intervent Radiol,2002,25(4):340-341.DOI:10.1007/s00270-001-0068-9.
29 Perrakis A,Vassos N,Grützmann 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.
30 Hau HM,Atanasov G,Tautenhahn HM,et al.The value of liver resection for focal nodular hyperplasia:resection yes or no?[J].Eur J Med Res,2015,20(1):1-11.DOI:10.1186/s40001-015-0181-x.
2 Nguyen BN,Fléjou JF,Terris B,et al.Focal nodular hyperplasia of the liver:a comprehensive pathologic study of 305lesions and recognition of new histologic forms[J].Am JSurg Pathol,1999,23(12):1441.
3 Luca GD,Zamparelli M,Fadda C,et al.Focal nodular hyperplasia of the liver in infancy:a case report[J].J Pediatr Surg,2006,41(2):456.DOI:10.1016/j.jpedsurg.2005.11.026.
4 Masetti R,Zama D,Gasperini P,et al.Focal nodular hyperplasia of the liver in children after hematopoietic stem cell transplantation[J].Pediatr Transplant,2013,17(5):479-486.DOI:10.1111/petr.12091.
5 Scalori A,Tavani A,Gallus S.Oral contraceptives and the risk of focal nodular hyperplasia of the liver:a case-control study[J].Am J Obstet Gynecol,2002,186(2):195-197.
6 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.DOI:10.1016/j.jpedsurg.2014.06.016.
7 Nguyen BN,Fléjou JF,Terris B,et al.Focal nodular hyperplasia of the liver:a comprehensive pathologic study of 305lesions and recognition of new histologic forms[J].Am JSurg Pathol,1999,23(12):1441.
8 Yang Y,Fu S,Li A,et al.Management and surgical treatment for focal nodular hyperplasia in children[J].Pediatr Surg Int,2008,24(6):699-703.DOI:10.1007/s00383-008-2150-8.
9 Hussain SM,Terkivatan TZ,Ondervan PE,et al.Focal nodular hyperplasia:findings at state-of-the-art MR imaging,US,CT and pathologic analysis[J].Radiographics,2004,24(1):3-17.DOI:10.1148/rg.241035050.
10 Towbin AJ,Luo GG,Yin H,et al.Focal nodular hyperplasia in children,adolescents and young adults[J].Pediatr Radiol,2011,41(3):341-349.DOI:10.1007/s00247-010-1839-8.
11 Benz-Bohm G,Hero B,Gossmann A,et al.Focal nodular hyperplasia of the liver in longterm survivors of neuroblastoma:how much diagnostic imaging is necessary?[J].Eur J Radiol,2010,74(3):e1-e5.DOI:10.1016/j.ejrad.2009.03.011.
12 Ungermann L,Eliás P,Zizka J,et al.Focal nodular hyperplasia:spoke-wheel arterial pattern and other signs on dynamic contrast-enhanced ultrasonography[J].Eur J Radiol,2007,63(2):290.DOI:10.1016/j.ejrad.2007.01.026.
13孙健,王捷.肝脏局灶性结节性增生诊断及规范化治疗[J].中国实用外科杂志,2013,33(9):742-745.Sun J,Wang J.Diagnosis and standardized management of hepatic focal nodular hyperplasia[J].Chin J Pract Surg,2013,9(33):742-745.
14 Cha DI,Yoo SY,Kim JH,et al.Clinical and imaging features of focal nodular hyperplasia in children[J].Ajr Am JRoentgenol,2014,202(5):960-965.DOI:10.2214/AJR.13.11856.
15 Trillaud H,Bruel JM,Valette PJ,et al.Characterization of focal liver lesions with Sono Vue-enhanced sonography:international multicenter-study in comparison to CT and MRI[J].World J Gastroenterol,2009,15(30):3748-3756.
16阎洁羽,刘凤永,王茂强.肝脏局灶性结节增生影像表现及研究进展[J].中国医学影像学杂志,2012,20(8):635-637.DOI:10.3969/j.issn.1005-5185.2012.08.023.Yan JY,Liu FY,Wang MQ.Imaging findings and research advances of hepatic focal nodular hyperplasia[J].Chin JMed Imag,2012,20(8):635-637.DOI:10.3969/j.issn.1005-5185.2012.08.023.
17 Bartolotta TV,Taibbi A,Brancatelli G,et al.Imaging findings of hepatic focal nodular hyperplasia in men and women:are they really different?[J].Radiol Med,2014,119(4):222-230.DOI:10.1007/s11547-013-0333-4.
18 Kuo PH,Lai HS,Huang SY,et al.Focal nodular hyperplasia of the liver in an 8-year-old boy[J].Surgery,2007,142(3):422-423.DOI:10.1016/j.surg.2006.10.011.
19汪洵理,靳曙光,杨周建,等.小儿肝脏局灶性结节状增生临床分析[J].临床小儿外科杂志,2012,11(4):292-293.DOI:10.3969/j.issn.1671-6353.2012.04.017.Wang XL,Jin SG,Yang ZJ,et al.Clinical analysis of focal nodular hyperplasia of liver in children[J].J Clin Ped Sur,2012,11(4):292-293.DOI:10.3969/j.issn.1671-6353.2012.04.017.
20沈英皓,樊嘉,吴志全,等.肝脏局灶性结节性增生60例临床分析[J].中华普通外科杂志,2005,20(7):397-399.DOI:10.3760/j.issn:1007-631X.2005.07.003.Shen YH,Fan J,Wu ZQ,et al.Focal nodular hyperplasia of the liver:a report of 60 cases[J].Chin J Gener Surg,2005,20(7):397-399.DOI:10.3760/j.issn:1007-631X.2005.07.003.
21 Cristiano A,Dietrich A,Spina JC,et al.Focal nodular hyperplasia and hepatic adenoma:current diagnosis and management[J].Updates Surg,2014,66(1):9-21.DOI:10.1007/s13304-013-0222-3.
22 Mcinnes MD,Hibbert RM,Inácio JR,et al.Focal nodular hyperplasia and hepatocellular adenoma:accuracy of gadoxetic acid-enhanced MR imaging-a systematic review[J].Radiology,2015,277(2):142986.DOI:10.1148/radiol.2015142986.
23 Muguti G,Tait N,Richardson A,et al.Hepatic focal nodular hyperplasia:a benign incidentaloma or a marker of serious hepatic disease?[J].HPB Surg,1992,5(3):171.
24 Gong Y,Chen L,Qiao ZW,et al.Focal nodular hyperplasia coexistent with hepatoblastoma in a 36-d-old infant[J].World J Gastroenterol,2015,21(3):1028-1031.DOI:10.3748/wjg.v21.i3.1028.
25 Petsas T,Tsamandas A,Tsota I,et al.A case of hepatocellular carcinoma arising within large focal nodular hyperplasia with review of the literature[J].World J Gastroenterol,2006,12(40):6567-6571.
26 Makhlouf HR,Abdul-Al HM,Goodman ZD.Diagnosis of focal nodular hyperplasia of the liver by needle biopsy[J].Hum Pathol,2005,36(11):1210-1216.DOI:10.1016/j.humpath.2005.08.014.
27祁付珍,吴健雄,邵永孚,等.肝脏局灶性结节增生的诊断及治疗[J].实用癌症杂志,2004,19(5):501-503.DOI:10.3969/j.issn.1001-5930.2004.05.017.Qi FZ,Wu JX,Shao YF,et al.Diagnosis and treatment of focal nodular hyperplasia of liver[J].Pract J Canc,2004,19(5):501-503.DOI:10.3969/j.issn.1001-5930.2004.05.017.
28 Geschwind JF,Degli MS,Morris JM,et al.Re:Treatment of focal nodular hyperplasia with selective transcatheter arterial embolization using iodized oil and polyvinyl alcohol[J].Cardiovasc Intervent Radiol,2002,25(4):340-341.DOI:10.1007/s00270-001-0068-9.
29 Perrakis A,Vassos N,Grützmann 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.
30 Hau HM,Atanasov G,Tautenhahn HM,et al.The value of liver resection for focal nodular hyperplasia:resection yes or no?[J].Eur J Med Res,2015,20(1):1-11.DOI:10.1186/s40001-015-0181-x.