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Hepatoblastoma must be differentiated from other diseases such as [[Hamartoma|hepatic mesenchymal hamartoma]], [[hepatocellular carcinoma]], [[Hepatocellular carcinoma|hepatic metastases]], [[infantile hemangioendothelioma]], and [[Rhabdomyosarcoma|rhabdomyosarcoma of biliary tract]].<ref name=differential>Differential diagnosis of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref>
Hepatoblastoma must be differentiated from other diseases such as [[Hamartoma|hepatic mesenchymal hamartoma]], [[hepatocellular carcinoma]], [[Hepatocellular carcinoma|hepatic metastases]], [[infantile hemangioendothelioma]], and [[Rhabdomyosarcoma|rhabdomyosarcoma of biliary tract]].<ref name=differential>Differential diagnosis of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref>
==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
If left untreated, patients with hepatoblastoma may progress to develop [[failure to thrive]], [[weight loss]], spontaneous rupture, hemorrhage, and death.<ref name="MadabhaviPatel2014">{{cite journal|last1=Madabhavi|first1=Irappa|last2=Patel|first2=Apurva|last3=Choudhary|first3=Mukesh|last4=Aagre|first4=Suhas|last5=Revannasiddaiah|first5=Swaroop|last6=Modi|first6=Gaurang|last7=Anand|first7=Asha|last8=Panchal|first8=Harsha|last9=Parikh|first9=Sonia|last10=Raut|first10=Shreeniwas|title=Paraneoplastic Recurrent Hypoglycaemic Seizures: An Initial Presentation of Hepatoblastoma in an Adolescent Male—A Rare Entity|journal=Case Reports in Pediatrics|volume=2014|year=2014|pages=1–5|issn=2090-6803|doi=10.1155/2014/104543}}</ref> Common complications of hepatoblastoma include paraneoplastic features, [[erythrocytosis]], [[thrombocytosis]], [[hypocalcemia]], [[Precocious puberty|isosexual precocious puberty]], [[hypoglycemia]].<ref name="MadabhaviPatel2014">{{cite journal|last1=Madabhavi|first1=Irappa|last2=Patel|first2=Apurva|last3=Choudhary|first3=Mukesh|last4=Aagre|first4=Suhas|last5=Revannasiddaiah|first5=Swaroop|last6=Modi|first6=Gaurang|last7=Anand|first7=Asha|last8=Panchal|first8=Harsha|last9=Parikh|first9=Sonia|last10=Raut|first10=Shreeniwas|title=Paraneoplastic Recurrent Hypoglycaemic Seizures: An Initial Presentation of Hepatoblastoma in an Adolescent Male—A Rare Entity|journal=Case Reports in Pediatrics|volume=2014|year=2014|pages=1–5|issn=2090-6803|doi=10.1155/2014/104543}}</ref> The 5-year survival rate of children with hepatoblastoma is approximately 70%.<ref name=risk>Prognosis and Prognostic Factors. National Cancer Institute 2015. http://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq#link/_570_toc. Accessed on October 28, 2015</ref>
*Prognosis is based on different factors including:<ref name="pmid30521216">{{cite journal |vauthors=Musick SR, Babiker HM |title= |journal= |volume= |issue= |pages= |date= |pmid=30521216 |doi= |url=}}</ref><ref name="pmid29755772">{{cite journal |vauthors=Kiruthiga KG, Ramakrishna B, Saha S, Sen S |title=Histological and immunohistochemical study of hepatoblastoma: correlation with tumour behaviour and survival |journal=J Gastrointest Oncol |volume=9 |issue=2 |pages=326–337 |date=April 2018 |pmid=29755772 |pmc=5934143 |doi=10.21037/jgo.2018.01.08 |url=}}</ref>
**Age of diagnosis, younger children have a better prognosis.
**[[Metastases]],  
**[[Alpha-fetoprotein]] ([[AFP]]) levels, drop in [[Alpha-fetoprotein|AFP]] level after [[chemotherapy]] means better response to treatment.
**[[Histologic]] subtype, the well-differentiated [[fetal]] subtype has a better [[prognosis]] compared with small cell undifferentiated ones.
**Pretreatment extent of disease (PRETEXT) which was developed to stage the [[tumor]] before [[surgical]] removal and compare the [[efficacy]] of varous adjuvant [[chemotherapeutic agents]]. It is based on [[anatomy]] of [[liver]] and depending on [[tumor]] free sectors of [[liver]].<ref name="pmid15718322">{{cite journal |vauthors=Aronson DC, Schnater JM, Staalman CR, Weverling GJ, Plaschkes J, Perilongo G, Brown J, Phillips A, Otte JB, Czauderna P, MacKinlay G, Vos A |title=Predictive value of the pretreatment extent of disease system in hepatoblastoma: results from the International Society of Pediatric Oncology Liver Tumor Study Group SIOPEL-1 study |journal=J. Clin. Oncol. |volume=23 |issue=6 |pages=1245–52 |date=February 2005 |pmid=15718322 |doi=10.1200/JCO.2005.07.145 |url=}}</ref> 
*Complications of hepatoblastoma includes:
**[[Pancytopenia]]
**[[Anemia]]
**[[Intraperitoneal|Intraperitonea]]<nowiki/>l [[tumor]] [[rupture]]
**Complications related to [[chemotherapy]] such as [[renal failure]]
**Post-[[transplant]] complications such as [[hepatic]] ductal [[obstruction]], [[biliary]] leakage, [[thrombosis]].<ref name="pmid25251521">{{cite journal |vauthors=Becker K, Furch C, Schmid I, von Schweinitz D, Häberle B |title=Impact of postoperative complications on overall survival of patients with hepatoblastoma |journal=Pediatr Blood Cancer |volume=62 |issue=1 |pages=24–8 |date=January 2015 |pmid=25251521 |doi=10.1002/pbc.25240 |url=}}</ref>
**[[Psychosocial]] effects of treatment and painful procedures


==Diagnosis==
==Diagnosis==

Revision as of 22:43, 2 January 2019

Hepatoblastoma Microchapters

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Overview

Historical Perspective

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Risk Factors

Screening

Differentiating Hepatoblastoma from other Diseases

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]

Overview

Hepatoblastoma is the most common primary liver tumor occurs in infant and children, usually less than 3 years old, more frequently in male, and accounting for over 1% of pediatric cancers. The etiology is unknown and not well understood, but it has been associated with Beckwith-Weidemann syndrome, familial adenomatous polyposis, and other factors such as low birth weight, preeclampsia, hemihypertrophy. The primary treatment is surgical resection, however, chemotherapy plays an important role by increasing the number of tumors that are respectable, chemotherapeutic agents helps in shrinkage of the tumor and make it easier to be resected by surgery. Prognosis of the tumor depends on various criteria such as resectability of the tumor, distant metastasis, tumor size, PRETEXT staging, and recurrence of the tumor. The mainstay of treatment is surgery, but the adjuvant chemotherapeutic agents and liver transplantation also are helpful.

Historical Perspective

  • In 1898, the first case of hepatoblastoma was published in English literature, the tumor was diagnosed in a 6-week old boy in Prague, by a physician named Misick, who found a large tumor on the autopsy of his liver. Decades later on 1962 Willis used the term, hepatoblastoma for this type of liver tumor because of it's embryonal origin [1]

Classification

Pathophysiology

Hepatoblastoma H&Esource:wikipedia


Causes

Epidemiology and Demographics

  • Hepatoblastoma is the most common primary liver cancer in infants and children, tumor involves right lobe of liver more often.[10]
  • The incidence/prevalence of hepatoblastoma is approximately 0.05–0.15 patients per 100000 population in children younger than 15 years.[11]
  • Peak incidence means of 18 months, mostly in infants and children younger than 3 years old, with a male predilection.
  • Hepatoblastoma accounts for one percent of all primary malignancies in pediatrics.

Risk factors

Screening

Differentiating Hepatoblastoma from other diseases

Hepatoblastoma must be differentiated from other diseases such as hepatic mesenchymal hamartoma, hepatocellular carcinoma, hepatic metastases, infantile hemangioendothelioma, and rhabdomyosarcoma of biliary tract.[14]

Natural History, Complications and Prognosis

Diagnosis

Staging

The staging of hepatoblastoma is based on the Intergroup staging system.[18]

History and Symptoms

Symptoms of hepatoblastoma include an abdominal mass (may be painless or painful), abdominal pain, weight loss, loss of appetite, early puberty in boys, jaundice, nausea, vomiting, back pain, and failure to thrive.[19][14]

Physical examination

Common physical examination findings of hepatoblastoma include hepatomegaly, abdominal distension, asymptomatic palpable abdominal mass, hemihypertrophy, jaundice, pyrexia, and anemia.[19][14]

Laboratory Findings

Laboratory tests for liver function are usually normal. An elevated concentration of alpha-fetoprotein is present in patients with hepatoblastoma.[19]

Xray

Findings on abdominal xray are nonspecific for hepatoblastoma and include right upper quadrant abdominal mass and/or calcifications in 10% of cases.[14]

CT

Abdominal CT scan may be helpful in the diagnosis of hepatoblastoma. Findings on CT scan suggestive of hepatoblastoma include well defined heterogeneous mass, intralesional areas of necrosis and hemorrhage, and dense calcifications.[14]

MRI

Abdominal MRI is helpful in the diagnosis of hepatoblastoma. On MRI, hepatoblastoma is characterized by hypointensity on T1-weighted imaging and hyperintensity compared to liver with areas of necrosis and hemorrhage on T2-weighted imaging.[14]

Ultrasound

Ultrasound may be helpful in the diagnosis of hepatoblastoma. Findings on ultrasound suggestive of hepatoblastoma include echogenic soft tissue mass, well defined lesion, and areas of shadowing due to intralesional calcifications.[14]

Biopsy

Biopsy is the gold standard for the diagnosis of hepatoblastoma. Histopathological findings on biopsy can be found here.

Treatment

Medical Therapy

The predominant therapy for hepatoblastoma is surgical resection. Neoadjuvant and adjuvant chemotherapy may be required.[19]

Surgery

Surgery is the mainstay of treatment for hepatoblastoma.[19]

Primary Prevention

There are no primary preventive measures available for hepatoblastoma.

Secondary Prevention

Effective measures for the secondary prevention of hepatoblastoma include use of abdominal ultrasound and alpha-fetoprotein levels in patients with Beckwith-Wiedemann syndrome or isolated hemihyperplasia.[20]

References

  1. Aronson DC, Czauderna P, Maibach R, Perilongo G, Morland B (October 2014). "The treatment of hepatoblastoma: Its evolution and the current status as per the SIOPEL trials". J Indian Assoc Pediatr Surg. 19 (4): 201–7. doi:10.4103/0971-9261.142001. PMC 4204244. PMID 25336801.
  2. Rowland JM (November 2002). "Hepatoblastoma: assessment of criteria for histologic classification". Med. Pediatr. Oncol. 39 (5): 478–83. doi:10.1002/mpo.10171. PMID 12228903.
  3. Czauderna P, Lopez-Terrada D, Hiyama E, Häberle B, Malogolowkin MH, Meyers RL (February 2014). "Hepatoblastoma state of the art: pathology, genetics, risk stratification, and chemotherapy". Curr. Opin. Pediatr. 26 (1): 19–28. doi:10.1097/MOP.0000000000000046. PMID 24322718.
  4. MacDonald BT, Tamai K, He X (July 2009). "Wnt/beta-catenin signaling: components, mechanisms, and diseases". Dev. Cell. 17 (1): 9–26. doi:10.1016/j.devcel.2009.06.016. PMC 2861485. PMID 19619488.
  5. 5.0 5.1 Kiruthiga KG, Ramakrishna B, Saha S, Sen S (April 2018). "Histological and immunohistochemical study of hepatoblastoma: correlation with tumour behaviour and survival". J Gastrointest Oncol. 9 (2): 326–337. doi:10.21037/jgo.2018.01.08. PMC 5934143. PMID 29755772.
  6. 6.0 6.1 Heck JE, Meyers TJ, Lombardi C, Park AS, Cockburn M, Reynolds P, Ritz B (August 2013). "Case-control study of birth characteristics and the risk of hepatoblastoma". Cancer Epidemiol. 37 (4): 390–5. doi:10.1016/j.canep.2013.03.004. PMC 3679264. PMID 23558166.
  7. Finegold MJ, Lopez-Terrada DH, Bowen J, Washington MK, Qualman SJ (April 2007). "Protocol for the examination of specimens from pediatric patients with hepatoblastoma". Arch. Pathol. Lab. Med. 131 (4): 520–9. doi:10.1043/1543-2165(2007)131[520:PFTEOS]2.0.CO;2. PMID 17425379.
  8. Mussa A, Duffy KA, Carli D, Ferrero GB, Kalish JM (January 2019). "Defining an optimal time window to screen for hepatoblastoma in children with Beckwith-Wiedemann syndrome". Pediatr Blood Cancer. 66 (1): e27492. doi:10.1002/pbc.27492. PMID 30270492.
  9. Curia MC, Zuckermann M, De Lellis L, Catalano T, Lattanzio R, Aceto G, Veschi S, Cama A, Otte JB, Piantelli M, Mariani-Costantini R, Cetta F, Battista P (January 2008). "Sporadic childhood hepatoblastomas show activation of beta-catenin, mismatch repair defects and p53 mutations". Mod. Pathol. 21 (1): 7–14. doi:10.1038/modpathol.3800977. PMID 17962810.
  10. Darbari A, Sabin KM, Shapiro CN, Schwarz KB (September 2003). "Epidemiology of primary hepatic malignancies in U.S. children". Hepatology. 38 (3): 560–6. doi:10.1053/jhep.2003.50375. PMID 12939582.
  11. Allan BJ, Parikh PP, Diaz S, Perez EA, Neville HL, Sola JE (October 2013). "Predictors of survival and incidence of hepatoblastoma in the paediatric population". HPB (Oxford). 15 (10): 741–6. doi:10.1111/hpb.12112. PMC 3791112. PMID 23600968.
  12. Shelmerdine SC, Roebuck DJ, Towbin AJ, McHugh K (August 2016). "MRI of paediatric liver tumours: How we review and report". Cancer Imaging. 16 (1): 21. doi:10.1186/s40644-016-0083-3. PMC 4986178. PMID 27526937.
  13. Hiyama E (October 2014). "Pediatric hepatoblastoma: diagnosis and treatment". Transl Pediatr. 3 (4): 293–9. doi:10.3978/j.issn.2224-4336.2014.09.01. PMC 4728840. PMID 26835349.
  14. 14.0 14.1 14.2 14.3 14.4 14.5 14.6 Differential diagnosis of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015
  15. Musick SR, Babiker HM. PMID 30521216. Missing or empty |title= (help)
  16. Aronson DC, Schnater JM, Staalman CR, Weverling GJ, Plaschkes J, Perilongo G, Brown J, Phillips A, Otte JB, Czauderna P, MacKinlay G, Vos A (February 2005). "Predictive value of the pretreatment extent of disease system in hepatoblastoma: results from the International Society of Pediatric Oncology Liver Tumor Study Group SIOPEL-1 study". J. Clin. Oncol. 23 (6): 1245–52. doi:10.1200/JCO.2005.07.145. PMID 15718322.
  17. Becker K, Furch C, Schmid I, von Schweinitz D, Häberle B (January 2015). "Impact of postoperative complications on overall survival of patients with hepatoblastoma". Pediatr Blood Cancer. 62 (1): 24–8. doi:10.1002/pbc.25240. PMID 25251521.
  18. Intergroup staging system of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma-staging-2. Accessed on November 3, 2015
  19. 19.0 19.1 19.2 19.3 19.4 Madabhavi, Irappa; Patel, Apurva; Choudhary, Mukesh; Aagre, Suhas; Revannasiddaiah, Swaroop; Modi, Gaurang; Anand, Asha; Panchal, Harsha; Parikh, Sonia; Raut, Shreeniwas (2014). "Paraneoplastic Recurrent Hypoglycaemic Seizures: An Initial Presentation of Hepatoblastoma in an Adolescent Male—A Rare Entity". Case Reports in Pediatrics. 2014: 1–5. doi:10.1155/2014/104543. ISSN 2090-6803.
  20. Beckwith-Wiedemann syndrome and hemihyperplasia. National Cancer Institute (2015). http://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq#link/_509_toc Accessed on November 4, 2015


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