Skip to main content
SpringerLink
Log in

Hepatic Tumors

  • Chapter
  • First Online:
Diseases of the Liver in Children

  • 1741 Accesses

Abstract

Primary hepatic neoplasms are rare in childhood and their presentation is usually as a palpable mass with few if any other symptoms. Despite the asymptomatic presentation, the majority that do present in this young age group are malignant. Serum alpha-fetoprotein, although characteristically elevated in some hepatic tumors, is not elevated in all malignant liver tumors of childhood. Radiologic and histological assessments are vital for making accurate diagnoses and for guiding appropriate therapy. This chapter will review the most common of the benign and malignant primary hepatic masses that present in childhood.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Abbreviations

AFP:

Serum alpha-fetoprotein

CT:

Computed tomography

EHE:

Epithelioid hemangioendothelioma

ESL:

Embryonal (undifferentiated) sarcoma of the liver

FLC:

Fibrolamellar carcinoma

FNH:

Focal nodular hyperplasia

HA:

Hepatocellular adenoma

HB:

Hepatoblastoma

HCC:

Hepatocellular carcinoma

HR:

Hepatobiliary rhabdomyosarcoma

IHH:

Infantile hepatic hemangioma

MH:

Mesenchymal hamartoma

MRI:

Magnetic resonance imaging

NRH:

Nodular regenerative hyperplasia

RICH:

Rapidly involuting congenital hemangioma

US:

Ultrasound

References

  1. Boon LM, Burrows PE, Paltiel HJ, Lund DP, Ezekowitz RA, Folkman J, et al. Hepatic vascular anomalies in infancy: a twenty-seven-year experience. J Pediatr. 1996;129(3):346–54. S0022-3476(96)70065-3 [pii].

    Article  CAS  PubMed  Google Scholar 

  2. Selby DM, Stocker JT, Waclawiw MA, Hitchcock CL, Ishak KG. Infantile hemangioendothelioma of the liver. Hepatology. 1994;20(1 Pt 1):39–45. S0270913994002120 [pii].

    CAS  PubMed  Google Scholar 

  3. Dehner LP, Ishak KG. Vascular tumors of the liver in infants and children. A study of 30 cases and review of the literature. Arch Pathol. 1971;92(2):101–11.

    CAS  PubMed  Google Scholar 

  4. Burrows PE, Dubois J, Kassarjian A. Pediatric hepatic vascular anomalies. Pediatr Radiol. 2001;31(8):533–45.

    Article  CAS  PubMed  Google Scholar 

  5. Chung EM, Cube R, Lewis RB, Conran RM. From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation part 1. Benign tumors. Radiographics. 2010;30(3):801–26. doi:10.1148/rg.303095173. 30/3/801.

    Article  PubMed  Google Scholar 

  6. Kassarjian A, Zurakowski D, Dubois J, Paltiel HJ, Fishman SJ, Burrows PE. Infantile hepatic hemangiomas: clinical and imaging findings and their correlation with therapy. AJR Am J Roentgenol. 2004;182(3):785–95.

    Article  PubMed  Google Scholar 

  7. Christison-Lagay ER, Burrows PE, Alomari A, Dubois J, Kozakewich HP, Lane TS, et al. Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Pediatr Surg. 2007;42(1):62–7. doi:10.1016/j.jpedsurg.2006.09.041; S0022-3468(06)00654-3 [pii].

    Article  PubMed  Google Scholar 

  8. Ohama K, Nagase H, Ogino K, Tsuchida K, Tanaka M, Kubo M, et al. Alpha-fetoprotein (AFP) levels in normal children. Eur J Pediatr Surg. 1997;7(5):267–9. doi:10.1055/s-2008-1071168.

    Article  CAS  PubMed  Google Scholar 

  9. Keslar PJ, Buck JL, Selby DM. From the archives of the AFIP. Infantile hemangioendothelioma of the liver revisited. Radiographics. 1993;13(3):657–70.

    Article  CAS  PubMed  Google Scholar 

  10. Kuroda T, Kumagai M, Nosaka S, Nakazawa A, Takimoto T, Hoshino K. Critical infantile hepatic hemangioma: results of a nationwide survey by the Japanese infantile hepatic hemangioma study group. J Pediatr Surg. 2011;46(12):2239–43. doi:10.1016/j.jpedsurg.2011.09.007; S0022-3468(11)00746-9 [pii].

    Article  PubMed  Google Scholar 

  11. Mhanna A, Franklin WH, Mancini AJ. Hepatic infantile hemangiomas treated with oral propranolol – a case series. Pediatr Dermatol. 2011;28(1):39–45. doi:10.1111/j.1525-1470.2010.01355.x.

    Article  PubMed  Google Scholar 

  12. Storch CH, Hoeger PH. Propranolol for infantile haemangiomas: insights into the molecular mechanisms of action. Br J Dermatol. 2010;163(2):269–74.

    Article  CAS  PubMed  Google Scholar 

  13. Bosemani T, Puttgen KB, Huisman TA, Tekes A. Multifocal infantile hepatic hemangiomas – imaging strategy and response to treatment after propranolol and steroids including review of the literature. Eur J Pediatr. 2012;171(7):1023–8.

    Article  PubMed  Google Scholar 

  14. Yeh I, Bruckner AL, Sanchez R, Jeng MR, Newell BD, Frieden IJ. Diffuse infantile hepatic hemangiomas: a report of four cases successfully managed with medical therapy. Pediatr Dermatol. 2011;28(3):267–75.

    Article  PubMed  Google Scholar 

  15. Barlow CF, Priebe CJ, Mulliken JB, Barnes PD, Mac DD, Folkman J, et al. Spastic diplegia as a complication of interferon alfa-2a treatment of hemangiomas of infancy. J Pediatr. 1998;132(3 Pt 1):527–30.

    Article  CAS  PubMed  Google Scholar 

  16. Stringer MD, Alizai NK. Mesenchymal hamartoma of the liver: a systematic review. J Pediatr Surg. 2005;40(11):1681–90. doi:10.1016/j.jpedsurg.2005.07.052; S0022-3468(05)00622-6 [pii].

    Article  PubMed  Google Scholar 

  17. Koumanidou C, Vakaki M, Papadaki M, Pitsoulakis G, Savvidou D, Kakavakis K. New sonographic appearance of hepatic mesenchymal hamartoma in childhood. J Clin Ultrasound. 1999;27(3):164–7. 10.1002/(SICI)1097-0096(199903/04)27:3<164::AID-JCU12>3.0.CO;2-J [pii].

    Article  CAS  PubMed  Google Scholar 

  18. Kitano Y, Ruchelli E, Weiner S, Adzick NS. Hepatic mesenchymal hamartoma associated with mesenchymal stem villous hyperplasia of the placenta. Fetal Diagn Ther. 2000;15(3):134–8. [pii] 20992.

    Article  CAS  PubMed  Google Scholar 

  19. Yen JB, Kong MS, Lin JN. Hepatic mesenchymal hamartoma. J Paediatr Child Health. 2003;39(8):632–4. 250 [pii].

    Article  PubMed  Google Scholar 

  20. Srouji MN, Chatten J, Schulman WM, Ziegler MM, Koop CE. Mesenchymal hamartoma of the liver in infants. Cancer. 1978;42(5):2483–9.

    Article  CAS  PubMed  Google Scholar 

  21. Kamata S, Nose K, Sawai T, Hasegawa T, Kuroda S, Sasaki T, et al. Fetal mesenchymal hamartoma of the liver: report of a case. J Pediatr Surg. 2003;38(4):639–41. doi:10.1053/jpsu.2003.50142; S0022346802631513 [pii].

    Article  PubMed  Google Scholar 

  22. Meinders AJ, Simons MP, Heij HA, Aronson DC. Mesenchymal hamartoma of the liver: failed management by marsupialization. J Pediatr Gastroenterol Nutr. 1998;26(3):353–5.

    Article  CAS  PubMed  Google Scholar 

  23. Luciani A, Kobeiter H, Maison P, Cherqui D, Zafrani ES, Dhumeaux D, et al. Focal nodular hyperplasia of the liver in men: is presentation the same in men and women? Gut. 2002;50(6):877–80. Pubmed PMCID:PM:PMC1773241.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  24. Scalori A, Tavani A, Gallus S, La VC, Colombo M. Oral contraceptives and the risk of focal nodular hyperplasia of the liver: a case–control study. Am J Obstet Gynecol. 2002;186(2):195–7. S0002937802382498 [pii].

    Article  PubMed  Google Scholar 

  25. Mathieu D, Kobeiter H, Maison P, Rahmouni A, Cherqui D, Zafrani ES, et al. Oral contraceptive use and focal nodular hyperplasia of the liver. Gastroenterology. 2000;118(3):560–4. S0016508500442605 [pii].

    Article  CAS  PubMed  Google Scholar 

  26. Kubota T, Shimizu K, Sonoyama T, Ikeda E, Kurioka H, Ouchi T, et al. Enlarged focal nodular hyperplasia of the liver under the influence of oral contraceptives. Hepatogastroenterology. 2001;48(42):1736–9.

    CAS  PubMed  Google Scholar 

  27. Wanless IR, Mawdsley C, Adams R. On the pathogenesis of focal nodular hyperplasia of the liver. Hepatology. 1985;5(6):1194–200. S0270913985001525 [pii].

    Article  CAS  PubMed  Google Scholar 

  28. Golli M, Mathieu D, Anglade MC, Cherqui D, Vasile N, Rahmouni A. Focal nodular hyperplasia of the liver: value of color Doppler US in association with MR imaging. Radiology. 1993;187(1):113–7.

    CAS  PubMed  Google Scholar 

  29. Wang LY, Wang JH, Lin ZY, Yu ML, Lu SN, Chuang WL, et al. Hepatic focal nodular hyperplasia: findings on color Doppler ultrasound. Abdom Imaging. 1997;22(2):178–81.

    Article  CAS  PubMed  Google Scholar 

  30. Carlson SK, Johnson CD, Bender CE, Welch TJ. CT of focal nodular hyperplasia of the liver. AJR Am J Roentgenol. 2000;174(3):705–12.

    Article  CAS  PubMed  Google Scholar 

  31. Bieze M, van den Esschert JW, Nio CY, Verheij J, Reitsma JB, Terpstra V, et al. Diagnostic accuracy of MRI in differentiating hepatocellular adenoma from focal nodular hyperplasia: prospective study of the additional value of gadoxetate disodium. AJR Am J Roentgenol. 2012;199(1):26–34.

    Article  PubMed  Google Scholar 

  32. Meyers RL. Tumors of the liver in children. Surg Oncol. 2007;16(3):195–203. doi:10.1016/j.suronc.2007.07.002; S0960-7404(07)00068-0 [pii].

    Article  PubMed  Google Scholar 

  33. Resnick MB, Kozakewich HP, Perez-Atayde AR. Hepatic adenoma in the pediatric age group. Clinicopathological observations and assessment of cell proliferative activity. Am J Surg Pathol. 1995;19(10):1181–90.

    Article  CAS  PubMed  Google Scholar 

  34. Bagia S, Hewitt PM, Morris DL. Anabolic steroid-induced hepatic adenomas with spontaneous haemorrhage in a bodybuilder. Aust N Z J Surg. 2000;70(9):686–7.

    Article  CAS  PubMed  Google Scholar 

  35. Toso C, Rubbia-Brandt L, Negro F, Morel P, Mentha G. Hepatocellular adenoma and polycystic ovary syndrome. Liver Int. 2003;23(1):35–7.

    Article  PubMed  Google Scholar 

  36. Marie-Cardine A, Schneider P, Greene V, Metayer J, Bachy B, Vannier JP. Erythrocytosis in a child with a hepatic adenoma. Med Pediatr Oncol. 2001;36(6):659–61. doi:10.1002/mpo.1148.

    Article  CAS  PubMed  Google Scholar 

  37. Musthafa CP, Chettupuzha AP, Syed AA, Ayshabi KA, Aravindan KP, Shobhana DR. Liver adenomatosis. Indian J Gastroenterol. 2003;22(1):30–1.

    CAS  PubMed  Google Scholar 

  38. Rubin RA, Mitchell DG. Evaluation of the solid hepatic mass. Med Clin North Am. 1996;80(5):907–28.

    Article  CAS  PubMed  Google Scholar 

  39. Grazioli L, Federle MP, Brancatelli G, Ichikawa T, Olivetti L, Blachar A. Hepatic adenomas: imaging and pathologic findings. Radiographics. 2001;21(4):877–92.

    Article  CAS  PubMed  Google Scholar 

  40. Micchelli ST, Vivekanandan P, Boitnott JK, Pawlik TM, Choti MA, Torbenson M. Malignant transformation of hepatic adenomas. Mod Pathol. 2008;21(4):491–7. doi:10.1038/modpathol.2008.8; modpathol20088 [pii].

    Article  CAS  PubMed  Google Scholar 

  41. Farges O, Dokmak S. Malignant transformation of liver adenoma: an analysis of the literature. Dig Surg. 2010;27(1):32–8. doi:10.1159/000268405; 000268405 [pii].

    Article  PubMed  Google Scholar 

  42. Hartleb M, Gutkowski K, Milkiewicz P. Nodular regenerative hyperplasia: evolving concepts on underdiagnosed cause of portal hypertension. World J Gastroenterol. 2011;17(11):1400–9.

    Article  PubMed Central  PubMed  Google Scholar 

  43. Giardiello FM, Petersen GM, Brensinger JD, Luce MC, Cayouette MC, Bacon J, et al. Hepatoblastoma and APC gene mutation in familial adenomatous polyposis. Gut. 1996;39(6):867–9. Pubmed PMCID:PM:PMC1383462.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  44. Steenman M, Westerveld A, Mannens M. Genetics of Beckwith-Wiedemann syndrome-associated tumors: common genetic pathways. Genes Chromosomes Cancer. 2000;28(1):1–13. 10.1002/(SICI)1098-2264(200005)28:1<1::AID-GCC1>3.0.CO;2-# [pii].

    Article  CAS  PubMed  Google Scholar 

  45. Van Tornout JM, Buckley JD, Quinn JJ, Feusner JH, Krailo MD, King DR, et al. Timing and magnitude of decline in alpha-fetoprotein levels in treated children with unresectable or metastatic hepatoblastoma are predictors of outcome: a report from the Children’s Cancer Group. J Clin Oncol. 1997;15(3):1190–7.

    PubMed  Google Scholar 

  46. Chung EM, Lattin Jr GE, Cube R, Lewis RB, Marichal-Hernandez C, Shawhan R, et al. From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors. Radiographics. 2011;31(2):483–507. doi:10.1148/rg.312105201. 31/2/483 [pii].

    Article  PubMed  Google Scholar 

  47. Otte JB. Progress in the surgical treatment of malignant liver tumors in children. Cancer Treat Rev. 2010;36(4):360–71. doi:10.1016/j.ctrv.2010.02.013; S0305-7372(10)00033-2 [pii].

    Article  PubMed  Google Scholar 

  48. Meyers RL, Czauderna P, Otte JB. Surgical treatment of hepatoblastoma. Pediatr Blood Cancer. 2012;59(5):800–8. doi:10.1002/pbc.24220.

    Article  PubMed  Google Scholar 

  49. Trobaugh-Lotrario AD, Katzenstein HM. Chemotherapeutic approaches for newly diagnosed hepatoblastoma: past, present, and future strategies. Pediatr Blood Cancer. 2012;59(5):809–12. doi:10.1002/pbc.24219.

    Article  PubMed  Google Scholar 

  50. Herzog CE, Andrassy RJ, Eftekhari F. Childhood cancers: hepatoblastoma. Oncologist. 2000;5(6):445–53.

    Article  CAS  PubMed  Google Scholar 

  51. Brown J, Perilongo G, Shafford E, Keeling J, Pritchard J, Brock P, et al. Pretreatment prognostic factors for children with hepatoblastoma – results from the International Society of Paediatric Oncology (SIOP) study SIOPEL 1. Eur J Cancer. 2000;36(11):1418–25. S0959-8049(00)00074-5 [pii].

    Article  CAS  PubMed  Google Scholar 

  52. Czauderna P. Adult type vs. childhood hepatocellular carcinoma – are they the same or different lesions? Biology, natural history, prognosis, and treatment. Med Pediatr Oncol. 2002;39(5):519–23. doi:10.1002/mpo.10178.

    Article  CAS  PubMed  Google Scholar 

  53. Ferenci P, Fried M, Labrecque D, Bruix J, Sherman M, Omata M, et al. Hepatocellular carcinoma (HCC): a global perspective. J Clin Gastroenterol. 2010;44(4):239–45. doi:10.1097/MCG.0b013e3181d46ef2.

    Article  PubMed  Google Scholar 

  54. Yu SB, Kim HY, Eo H, Won JK, Jung SE, Park KW, et al. Clinical characteristics and prognosis of pediatric hepatocellular carcinoma. World J Surg. 2006;30(1):43–50. 10.1007/s00268-005-7965-z.

    Article  PubMed  Google Scholar 

  55. European Association for the Study of the Liver, European Organisation for Research and Treatment of Cancer. EASL-EORTC clinical practice guidelines: management of hepatocellular carcinoma. J Hepatol. 2012;56(4):908–43. doi:10.1016/j.jhep.2011.12.001; S0168-8278(11)00873-7 [pii].

    Google Scholar 

  56. Hanish SI, Knechtle SJ. Liver transplantation for the treatment of hepatocellular carcinoma. Oncology (Williston Park). 2011;25(8):752–7.

    Google Scholar 

  57. Mazzaferro V, Regalia E, Doci R, Andreola S, Pulvirenti A, Bozzetti F, et al. Liver transplantation for the treatment of small hepatocellular carcinomas in patients with cirrhosis. N Engl J Med. 1996;334(11):693–9. doi:10.1056/NEJM199603143341104.

    Article  CAS  PubMed  Google Scholar 

  58. McLarney JK, Rucker PT, Bender GN, Goodman ZD, Kashitani N, Ros PR. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics. 1999;19(2):453–71.

    Article  CAS  PubMed  Google Scholar 

  59. Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. J Am Coll Surg. 2012;215(6):820–30.

    Article  PubMed  Google Scholar 

  60. Crider MH, Hoggard E, Manivel JC. Undifferentiated (embryonal) sarcoma of the liver. Radiographics. 2009;29(6):1665–8. doi:10.1148/rg.296085237. 29/6/1665 [pii].

    Article  PubMed  Google Scholar 

  61. Noguchi K, Yokoo H, Nakanishi K, Kakisaka T, Tsuruga Y, Kamachi H, et al. A long-term survival case of adult undifferentiated embryonal sarcoma of liver. World J Surg Oncol. 2012;10:65. doi:10.1186/1477-7819-10-65. Pubmed PMCID:PM:PMC3407002 DOI: 1477-7819-10-65 [pii].

    Article  PubMed Central  PubMed  Google Scholar 

  62. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer. 1999;85(3):562–82. 10.1002/(SICI)1097-0142(19990201)85:3<562::AID-CNCR7>3.0.CO;2-T [pii].

    Article  CAS  PubMed  Google Scholar 

  63. Lyburn ID, Torreggiani WC, Harris AC, Zwirewich CV, Buckley AR, Davis JE, et al. Hepatic epithelioid hemangioendothelioma: sonographic, CT, and MR imaging appearances. AJR Am J Roentgenol. 2003;180(5):1359–64.

    Article  PubMed  Google Scholar 

  64. Buetow PC, Buck JL, Ros PR, Goodman ZD. Malignant vascular tumors of the liver: radiologic-pathologic correlation. Radiographics. 1994;14(1):153–66.

    Article  CAS  PubMed  Google Scholar 

  65. Spunt SL, Lobe TE, Pappo AS, Parham DM, Wharam Jr MD, Arndt C, et al. Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma. J Pediatr Surg. 2000;35(2):309–16. S0022-3468(00)90030-7 [pii].

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Pediatric Gastroenterology and Hepatology, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA, USA

    Rohit Gupta MD, PhD & Karen F. Murray MD

  2. Division of Pediatric Transplantation, Department of Surgery, University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, WA, USA

    André A. Dick MD, MPH

Authors
  1. Rohit Gupta MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. André A. Dick MD, MPH
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Karen F. Murray MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to André A. Dick MD, MPH .

Editor information

Editors and Affiliations

  1. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Karen F. Murray

  2. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Simon Horslen

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer Science+Business Media New York

About this chapter

Cite this chapter

Gupta, R., Dick, A.A., Murray, K.F. (2014). Hepatic Tumors. In: Murray, K., Horslen, S. (eds) Diseases of the Liver in Children. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9005-0_22

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-9005-0_22

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-9004-3

  • Online ISBN: 978-1-4614-9005-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation