Hepatoblastoma: Difference between revisions

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{{Infobox_Disease
__NOTOC__
| Name          = {{PAGENAME}}
| Image          =
| Caption        =
| DiseasesDB    = 5799
| ICD10          = {{ICD10|C|22|0|c|15}}
| ICD9          =
| ICDO          = 8970/3
| OMIM          =
| MedlinePlus    =
| eMedicineSubj  = ped
| eMedicineTopic = 982
| eMedicine_mult = {{eMedicine2|radio|331}}
| MeshID        = D018197
}}
{{SI}}


{{CMG}}
'''For patient information, click [[Hepatoblastoma (patient information)|here]]'''
{{Hepatoblastoma}}
{{CMG}}; {{AE}} {{Nnasiri}}  


{{EH}}
{{SK}} Epithelial hepatoblastoma; Fetal hepatoblastoma; Embryonal hepatoblatoma; Macrotrabecular hepatoblastoma; Mixed hepatoblastoma; Rhabdoid hepatoblastoma; Small cell hepatoblastoma; Undifferentiated hepatoblastoma; Cholangioblastic hepatoblastoma; Teratoid hepatoblastoma; Chondroid hepatoblastoma; Osteoid hepatoblastoma; Rhabdomyoblastic hepatoblastoma; Neuroid-melanocytic hepatoblastoma; Well differentiated hepatoblastoma; HB


==Overview==
==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|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 (pathology)|staging]], and recurrence of the [[tumor]]. The mainstay of treatment is [[surgery]], but the [[adjuvant]] [[Chemotherapeutic agent|chemotherapeutic]] agents and [[liver transplantation]] also are helpful.


'''Hepatoblastoma''' is an uncommon malignant liver neoplasm occurring in infants and children and composed of tissue resembling fetal or mature liver cells or [[bile duct]]s. They usually present with an [[abdominal mass]]. [[Alpha-fetoprotein]] (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.<ref name="pmid18166449">{{cite journal
==Historical Perspective==
| author = De Ioris M, Brugieres L, Zimmermann A, Keeling J, Brock P, Maibach R, Pritchard J, Shafford L, Zsiros J, Czaudzerna P, Perilongo G
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 [[Embryo|embryonal]] origin <ref name="pmid25336801">{{cite journal |vauthors=Aronson DC, Czauderna P, Maibach R, Perilongo G, Morland B |title=The treatment of hepatoblastoma: Its evolution and the current status as per the SIOPEL trials |journal=J Indian Assoc Pediatr Surg |volume=19 |issue=4 |pages=201–7 |date=October 2014 |pmid=25336801 |pmc=4204244 |doi=10.4103/0971-9261.142001 |url=}}</ref>
| title = Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience.
 
| journal = Eur J Cancer
==Classification==
| volume = 44
*Hepatoblastoma are divided into two broad categories:<ref name="pmid12228903">{{cite journal |vauthors=Rowland JM |title=Hepatoblastoma: assessment of criteria for histologic classification |journal=Med. Pediatr. Oncol. |volume=39 |issue=5 |pages=478–83 |date=November 2002 |pmid=12228903 |doi=10.1002/mpo.10171 |url=}}</ref><ref name="pmid24322718">{{cite journal |vauthors=Czauderna P, Lopez-Terrada D, Hiyama E, Häberle B, Malogolowkin MH, Meyers RL |title=Hepatoblastoma state of the art: pathology, genetics, risk stratification, and chemotherapy |journal=Curr. Opin. Pediatr. |volume=26 |issue=1 |pages=19–28 |date=February 2014 |pmid=24322718 |doi=10.1097/MOP.0000000000000046 |url=}}</ref>
| issue =  
**[[Epithelial]] type (E-HB)
| pages = 545
***[[Fetal]], which has four subtypes:
| year = 2007
****Well differentiated
| pmid = 18166449
****Crowded or [[mitotic]]<nowiki/>ally active
| doi = 10.1016/j.ejca.2007.11.022
****[[Pleomorphic]]
| issn =  
****Poorly differentiated
}}</ref>
****[[Anaplastic]]
***[[Embryo]]<nowiki/>nal
***Macrotubular small cell [[undifferentiated]] (SCU)
***Cholangioblastic
**Mixed [[epithelial]] and [[mesenchymal]] type (MEM-HB). The mixed type is subdivided into:
***[[Stromal]] derivatives 
***[[Teratoma|Teratoid]] variants
 
==Pathophysiology==
*The exact [[pathogenesis]] of hepatoblastoma is not fully understood. <ref name="pmid19619488">{{cite journal |vauthors=MacDonald BT, Tamai K, He X |title=Wnt/beta-catenin signaling: components, mechanisms, and diseases |journal=Dev. Cell |volume=17 |issue=1 |pages=9–26 |date=July 2009 |pmid=19619488 |pmc=2861485 |doi=10.1016/j.devcel.2009.06.016 |url=}}</ref>
*Loss of function [[mutations]] in [[APC]] leads to [[intracellular]] accumulation of the [[protooncogene]] [[beta-catenin]], which leads to [[germline]] [[mutation]] of [[Wnt signalling pathway|Wnt signal]] [[transduction]] and pathway.
*Hepatoblastomas originate from primitive [[hepatic]] [[stem cells]].
*[[Beta-catenin|B-catenin]] [[mutations]] have been shown to be common in the majority of [[sporadic]] hepatoblastomas.
*Studies revealed that [[tumor]] occurs more often in families affected by [[familial adenomatous polyposis]]([[FAP]]), which is caused by inactivation of the [[adenomatous polyposis coli]] ([[APC]]), a [[tumor-suppressor gene]] that [[down-regulate]] the amount of [[beta-catenin]].
*[[Immunohistochemical]] markers such as expression of CK19, [[beta-catenin]] and EpCAM were correlated with [[tumor]] behaviour, response to [[chemotherapy]] and survival.<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>
 
[[File:Hepatoblastoma.jpg|150px|thumb|left|Hepatoblastoma H&E[https://commons.wikimedia.org/wiki source:wikipedia]]]<br style="clear:left" />
 
==Causes==
*Underlying causes of hepatoblastoma poorly understood and most tumors are sporadic but there are some [[risk factors]] and conditions that have a strong association with this [[tumor]] such as:<ref name="pmid23558166">{{cite journal |vauthors=Heck JE, Meyers TJ, Lombardi C, Park AS, Cockburn M, Reynolds P, Ritz B |title=Case-control study of birth characteristics and the risk of hepatoblastoma |journal=Cancer Epidemiol |volume=37 |issue=4 |pages=390–5 |date=August 2013 |pmid=23558166 |pmc=3679264 |doi=10.1016/j.canep.2013.03.004 |url=}}</ref><ref name="pmid17425379">{{cite journal |vauthors=Finegold MJ, Lopez-Terrada DH, Bowen J, Washington MK, Qualman SJ |title=Protocol for the examination of specimens from pediatric patients with hepatoblastoma |journal=Arch. Pathol. Lab. Med. |volume=131 |issue=4 |pages=520–9 |date=April 2007 |pmid=17425379 |doi=10.1043/1543-2165(2007)131[520:PFTEOS]2.0.CO;2 |url=}}</ref><ref name="pmid30270492">{{cite journal |vauthors=Mussa A, Duffy KA, Carli D, Ferrero GB, Kalish JM |title=Defining an optimal time window to screen for hepatoblastoma in children with Beckwith-Wiedemann syndrome |journal=Pediatr Blood Cancer |volume=66 |issue=1 |pages=e27492 |date=January 2019 |pmid=30270492 |doi=10.1002/pbc.27492 |url=}}</ref>
**[[Beckwith-Weidemann Syndrome|Beckwith-Weidemann syndrome]]
**[[Familial adenomatous polyposis]] (FAP)
**[[Down syndrome]]
**[[Edward's syndrome|Edward syndrome]] ([[trisomy 18]])
**[[Nephroblastoma]],
**[[Low birth weight]] infants are at higher risk of developing a hepatoblastoma
**[[Preeclampsia]] 
**Parental [[tobacco]] [[smoking]]
**[[Oxygen therapy]]
**Certain medication ([[furosemide]])
**[[Radiation]]
**[[Total parenteral nutrition]] ([[TPN]])
 
*The most common [[genetic]] [[mutation]] which plays role in [[etiology]] of sporadic cases include:<ref name="pmid17962810">{{cite journal |vauthors=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 |title=Sporadic childhood hepatoblastomas show activation of beta-catenin, mismatch repair defects and p53 mutations |journal=Mod. Pathol. |volume=21 |issue=1 |pages=7–14 |date=January 2008 |pmid=17962810 |doi=10.1038/modpathol.3800977 |url=}}</ref>
**The [[Wnt signaling pathway]] which results in the accumulation of [[beta-catenin]].
 
==Differentiating hepatoblastoma from Other Diseases==
*Hepatoblastoma must be differentiated from other diseases that cause rapidly enlarging abdominal mass in pediatrics such as:<ref name="pmid26835349">{{cite journal |vauthors=Hiyama E |title=Pediatric hepatoblastoma: diagnosis and treatment |journal=Transl Pediatr |volume=3 |issue=4 |pages=293–9 |date=October 2014 |pmid=26835349 |pmc=4728840 |doi=10.3978/j.issn.2224-4336.2014.09.01 |url=}}</ref>
**[[Hepatocellular carcinoma]] ([[HCC]]) of liver
**[[Focal]] [[nodular]] [[hyperplasia]] of liver
**[[Hepatic adenoma]] of liver
**[[Lymphoma]], and [[metastases]] to liver
 
==Epidemiology and Demographics==
*Hepatoblastoma is the most common primary liver [[cancer]] in infants and children, [[tumor]] involves right lobe of [[liver]] more often.<ref name="pmid12939582">{{cite journal |vauthors=Darbari A, Sabin KM, Shapiro CN, Schwarz KB |title=Epidemiology of primary hepatic malignancies in U.S. children |journal=Hepatology |volume=38 |issue=3 |pages=560–6 |date=September 2003 |pmid=12939582 |doi=10.1053/jhep.2003.50375 |url=}}</ref>
*The incidence/[[prevalence]] of hepatoblastoma is approximately 0.05–0.15 patients per 100000 population in children younger than 15 years.<ref name="pmid23600968">{{cite journal |vauthors=Allan BJ, Parikh PP, Diaz S, Perez EA, Neville HL, Sola JE |title=Predictors of survival and incidence of hepatoblastoma in the paediatric population |journal=HPB (Oxford) |volume=15 |issue=10 |pages=741–6 |date=October 2013 |pmid=23600968 |pmc=3791112 |doi=10.1111/hpb.12112 |url=}}</ref>
*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==
==Risk Factors==


Risk factors for hepatoblastoma including the following:
*Common risk factors in the development of hepatoblastoma include:<ref name="pmid23558166">{{cite journal |vauthors=Heck JE, Meyers TJ, Lombardi C, Park AS, Cockburn M, Reynolds P, Ritz B |title=Case-control study of birth characteristics and the risk of hepatoblastoma |journal=Cancer Epidemiol |volume=37 |issue=4 |pages=390–5 |date=August 2013 |pmid=23558166 |pmc=3679264 |doi=10.1016/j.canep.2013.03.004 |url=}}</ref>
**[[Low birth weight]] infants
**[[Preeclampsia]]
**[[Fetal distress]]
**[[Premature labor]]
**[[Chromosomal anomalies]] such as [[Down syndrome]], [[Edwards syndrome]].
**Parental [[tobacco]] [[smoking]] before and during [[pregnancy]]
**[[Oxygen therapy]]
**Certain medication ([[furosemide]]) 
**[[Total parenteral nutrition]] ([[TPN]])
 
==Screening==
*There is no screening method for detecting hepatoblastoma.
*[[Ultrasound]] is the main tool for suspected [[hepatic]] lesions in children.<ref name="pmid27526937">{{cite journal |vauthors=Shelmerdine SC, Roebuck DJ, Towbin AJ, McHugh K |title=MRI of paediatric liver tumours: How we review and report |journal=Cancer Imaging |volume=16 |issue=1 |pages=21 |date=August 2016 |pmid=27526937 |pmc=4986178 |doi=10.1186/s40644-016-0083-3 |url=}}</ref>
*Serum [[alpha-fetoprotein]] ([[AFP]]) is the most important [[clinical]] marker of hepatoblastoma and helps to estimate [[malignant]] change, response to the treatment, and [[relapse]]. <ref name="pmid26835349">{{cite journal |vauthors=Hiyama E |title=Pediatric hepatoblastoma: diagnosis and treatment |journal=Transl Pediatr |volume=3 |issue=4 |pages=293–9 |date=October 2014 |pmid=26835349 |pmc=4728840 |doi=10.3978/j.issn.2224-4336.2014.09.01 |url=}}</ref>
 
==Natural History, Complications, and Prognosis==
*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==
===Diagnostic Study of Choice===
*The diagnosis of hepatoblastoma is made when [[abdominal mass]] is detected on [[ultrasound]] or [[spiral CT scan]], but a definitive diagnosis requires the histological evaluation of [[biopsy]] specimen after surgery.<ref name="pmid26835349">{{cite journal |vauthors=Hiyama E |title=Pediatric hepatoblastoma: diagnosis and treatment |journal=Transl Pediatr |volume=3 |issue=4 |pages=293–9 |date=October 2014 |pmid=26835349 |pmc=4728840 |doi=10.3978/j.issn.2224-4336.2014.09.01 |url=}}</ref>
 
===History and Symptoms===
*The majority of patients with hepatoblastoma have an [[abdominal mass]] or [[abdominal distension]]. <ref name="pmid26835349">{{cite journal |vauthors=Hiyama E |title=Pediatric hepatoblastoma: diagnosis and treatment |journal=Transl Pediatr |volume=3 |issue=4 |pages=293–9 |date=October 2014 |pmid=26835349 |pmc=4728840 |doi=10.3978/j.issn.2224-4336.2014.09.01 |url=}}</ref>
*Other symptoms of hepatoblastoma include:  
**[[Abdominal discomfort]]
**Generalized [[Fatigue (medical)|fatigue]]
**Loss of appetite ([[Anorexia]])
**[[Nausea]] and [[vomiting]]


* Being male.
*Ruptured tumor can cause symptoms of [[peritoneal]] irritation due to [[intraperitoneal]] bleeding such as severe [[abdominal pain]], nausea, vomiting, and severe [[anemia]].<ref name="pmid16273660">{{cite journal |vauthors=Ke HY, Chen JH, Jen YM, Yu JC, Hsieh CB, Chen CJ, Liu YC, Chen TW, Chan DC |title=Ruptured hepatoblastoma with massive internal bleeding in an adult |journal=World J. Gastroenterol. |volume=11 |issue=39 |pages=6235–7 |date=October 2005 |pmid=16273660 |pmc=4436650 |doi= |url=}}</ref>
* Having familial adenomatous polyposis (FAP).
* Having Beckwith-Wiedemann syndrome.
* Having had a very low weight at birth.


==Signs and Symptoms==
===Physical Examination===
*Physical examination of patients with hepatoblastoma is usually remarkable for single, mildly painful, rapidly enlarging [[abdominal mass]] that is found in the right lobe of the [[liver]].<ref name="pmid23330017">{{cite journal |vauthors=Zhang Q, Ming J, Zhang S, Guo D, Qiu X |title=A rare case of adult hepatoblastoma with neuroendocrine differentiation misdiagnosed as neuroendocrine tumor |journal=Int J Clin Exp Pathol |volume=6 |issue=2 |pages=308–13 |date=2013 |pmid=23330017 |pmc=3544231 |doi= |url=}}</ref><ref name="pmid26835349">{{cite journal |vauthors=Hiyama E |title=Pediatric hepatoblastoma: diagnosis and treatment |journal=Transl Pediatr |volume=3 |issue=4 |pages=293–9 |date=October 2014 |pmid=26835349 |pmc=4728840 |doi=10.3978/j.issn.2224-4336.2014.09.01 |url=}}</ref>
*Most tumors are [[solitary]]; but can be multifocal as well.


Possible signs of childhood liver cancer include a lump or pain in the abdomen.
===Laboratory Findings===
*Laboratory findings that help with the diagnosis of hepatoblastoma includes:<ref name="pmid49416">{{cite journal |vauthors=Exelby PR, Filler RM, Grosfeld JL |title=Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical Section Survey--1974 |journal=J. Pediatr. Surg. |volume=10 |issue=3 |pages=329–37 |date=June 1975 |pmid=49416 |doi= |url=}}</ref>
**High levels of [[Alpha-fetoprotein]] ([[AFP]])
**Mild [[liver]] function disturbances
**[[Anemia]] on [[Complete blood counts|complete blood count]]
**[[Thrombocytosis]] (more frequent because of the effect of [[thrombopoietin]])<ref name="pmid6153151">{{cite journal |vauthors=Nickerson HJ, Silberman TL, McDonald TP |title=Hepatoblastoma, thrombocytosis, and increased thrombopoietin |journal=Cancer |volume=45 |issue=2 |pages=315–7 |date=January 1980 |pmid=6153151 |doi= |url=}}</ref>
**[[Thrombocytopenia]] (less common)


Symptoms are more common after the tumor becomes large. Other conditions can cause the same symptoms.  
===Electrocardiogram===
*There are no [[ECG]] findings associated with hepatoblastoma.


* A painless lump in the abdomen.
===X-ray===
* Swelling or pain in the abdomen.
*[[Chest-X-Ray|Chest]] [[Chest X-ray|x-rays]] can be useful especially since this [[tumor]] has the affinity to [[metastasize]] to [[lungs]].<ref name="pmid11042582">{{cite journal |vauthors=Perilongo G, Brown J, Shafford E, Brock P, De Camargo B, Keeling JW, Vos A, Philips A, Pritchard J, Plaschkes J |title=Hepatoblastoma presenting with lung metastases: treatment results of the first cooperative, prospective study of the International Society of Paediatric Oncology on childhood liver tumors |journal=Cancer |volume=89 |issue=8 |pages=1845–53 |date=October 2000 |pmid=11042582 |doi= |url=}}</ref>
* Weight loss for no known reason.
* Loss of appetite.
* Early puberty in boys.
* Nausea and vomiting.


==Diagnosis of Childhood Liver Cancer==
===Ultrasound===
*Imaging studies play an important role in the diagnosis, [[Staging (pathology)|staging]], and treatment of disease, most tumors can be resected surgically and [[ultrasound]] is often used in order to detect [[tumor]] size, also the initial diagnosis is made by [[abdominal]] [[ultrasound]].


The following tests and procedures may be used:
===CT scan===
*[[CT scan]] is very helpful to diagnose the disease, children with hepatoblastoma undergo either a CT or [[MRI]] scan at diagnosis. <ref name="pmid27955729">{{cite journal |vauthors=Aronson DC, Meyers RL |title=Malignant tumors of the liver in children |journal=Semin. Pediatr. Surg. |volume=25 |issue=5 |pages=265–275 |date=October 2016 |pmid=27955729 |doi=10.1053/j.sempedsurg.2016.09.002 |url=}}</ref>
*Surgeons prefer an [[angiographic]] or [[biphasic]] [[CT scan]] because of better depiction of the [[hepatic]] arterial, [[portal]] venous and [[hepatic vein]] and other [[liver]] structures.
*Concerns about [[radiation]] exposures in [[pediatrics]] has changed this modality in favor of [[MRI]], although [[MRI]] is much lengthy exam than [[CT]] has the advantage of multiplanar [[soft-tissue]] characterization, and when [[diffusion-weighted imaging]] techniques are used, [[MRI]] is exquisitely sensitive for detecting tiny [[liver]] lesions.
*[[Spiral CT scan]] findings of hypervascular lesions in the [[liver]] with delayed contrast excretion are highly suggestive of a [[malignant]] [[liver]] [[tumor]].


* Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
===MRI===
* Serum tumor marker test: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The blood of children who have liver cancer may have increased amounts of a protein called alpha-fetoprotein (AFP) or a hormone called beta-human chorionic gonadotropin (β-hCG). Other cancers and certain noncancerous conditions, including cirrhosis and hepatitis, may also increase AFP levels.
*[[MRI]] has the advantage of multiplanar [[soft-tissue]] characterization, lack of harmful [[ionizing radiation]] and when [[diffusion-weighted imaging]] techniques are used, [[MRI]] is exquisitely sensitive for detecting tiny [[liver]] lesions.<ref name="pmid27526937">{{cite journal |vauthors=Shelmerdine SC, Roebuck DJ, Towbin AJ, McHugh K |title=MRI of paediatric liver tumours: How we review and report |journal=Cancer Imaging |volume=16 |issue=1 |pages=21 |date=August 2016 |pmid=27526937 |pmc=4986178 |doi=10.1186/s40644-016-0083-3 |url=}}</ref>
* Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
:* The number of red blood cells, white blood cells, and platelets.
:* The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
:* The portion of the blood sample made up of red blood cells.
* Liver function tests: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver cancer.
* Abdominal x-ray: An x-ray of the organs in the abdomen. An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body.
* Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
* CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. In childhood liver cancer, a CT scan of the chest and abdomen is usually done.
* MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
* Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. The sample may be taken during surgery to remove or view the tumor. A pathologist views the sample under a microscope to determine the type of liver cancer.


==Treatment==
===Other Imaging Findings===
*Other imaging studies such as [[PET scan]] or even [[bone scan]] when there is evidence of [[metastasis]] to [[bone]] may be helpful in the diagnosis of hepatoblastoma. PET scan can be helpful in localizing recurrent hepatoblastoma.<ref name="pmid16170514">{{cite journal |vauthors=Figarola MS, McQuiston SA, Wilson F, Powell R |title=Recurrent hepatoblastoma with localization by PET-CT |journal=Pediatr Radiol |volume=35 |issue=12 |pages=1254–8 |date=December 2005 |pmid=16170514 |doi=10.1007/s00247-005-1568-6 |url=}}</ref><ref name="pmid8293620">{{cite journal |vauthors=Archer D, Babyn P, Gilday D, Greenberg MA |title=Potentially misleading bone scan findings in patients with hepatoblastoma |journal=Clin Nucl Med |volume=18 |issue=12 |pages=1026–31 |date=December 1993 |pmid=8293620 |doi= |url=}}</ref>


Surgical resection, adjuvant chemotherapy prior to resection, and [[liver transplantation]] have been used to treat these neoplasms.
===Other Diagnostic Studies===


==Factors that Affect Prognosis (chance of recovery) and Treatment Options==
*Other diagnostic studies for hepatoblastoma include evaluation of [[serum]] level of [[alpha-fetoprotein]], serum [[hCG]] level in rare cases of [[precocious puberty]].<ref name="pmid2411379">{{cite journal |vauthors=Nakagawara A, Ikeda K, Tsuneyoshi M, Daimaru Y, Enjoji M, Watanabe I, Iwafuchi M, Sawada T |title=Hepatoblastoma producing both alpha-fetoprotein and human chorionic gonadotropin. Clinicopathologic analysis of four cases and a review of the literature |journal=Cancer |volume=56 |issue=7 |pages=1636–42 |date=October 1985 |pmid=2411379 |doi= |url=}}</ref><ref name="pmid6187430">{{cite journal |vauthors=Morinaga S, Yamaguchi M, Watanabe I, Kasai M, Ojima M, Sasano N |title=An immunohistochemical study of hepatoblastoma producing human chorionic gonadotropin |journal=Cancer |volume=51 |issue=9 |pages=1647–52 |date=May 1983 |pmid=6187430 |doi= |url=}}</ref>


The prognosis (chance of recovery) and treatment options depend on the following:
==Treatment==
===Medical Therapy===
*The mainstay of therapy for hepatoblastoma is [[Surgery|surger]]<nowiki/>y, however, the vast majority of the tumors cannot be completely resected because of their large size or [[metastasis]].<ref name="pmid26308249">{{cite journal |vauthors=Pham TA, Gallo AM, Concepcion W, Esquivel CO, Bonham CA |title=Effect of Liver Transplant on Long-term Disease-Free Survival in Children With Hepatoblastoma and Hepatocellular Cancer |journal=JAMA Surg |volume=150 |issue=12 |pages=1150–8 |date=December 2015 |pmid=26308249 |doi=10.1001/jamasurg.2015.1847 |url=}}</ref>
*Liver [[transplantation]] can be considered for [[tumors]] that cannot be removed by surgery.<ref name="pmid10839879">{{cite journal |vauthors=Reyes JD, Carr B, Dvorchik I, Kocoshis S, Jaffe R, Gerber D, Mazariegos GV, Bueno J, Selby R |title=Liver transplantation and chemotherapy for hepatoblastoma and hepatocellular cancer in childhood and adolescence |journal=J. Pediatr. |volume=136 |issue=6 |pages=795–804 |date=June 2000 |pmid=10839879 |doi= |url=}}</ref>
*[[Chemotherapy]] is an important [[adjuvant therapy]], and [[cisplatin]] is the most commonly used [[chemotherapeutic agent]], it can reduce the volume of [[tumors]] that are too big for surgical removal.<ref name="pmid12778356">{{cite journal |vauthors=Häberle B, Bode U, von Schweinitz D |title=[Differentiated treatment protocols for high- and standard-risk hepatoblastoma--an interim report of the German Liver Tumor Study HB99] |language=German |journal=Klin Padiatr |volume=215 |issue=3 |pages=159–65 |date=2003 |pmid=12778356 |doi=10.1055/s-2003-39375 |url=}}</ref>


* The stage of the cancer (the size of the tumor, whether it affects part or all of the liver, and whether it has spread to other places in the body, such as the lungs).
===Surgery===
* Whether the cancer can be removed completely by surgery.
*[[Surgery]] is the mainstay of treatment for hepatoblastoma.<ref name="pmid30084209">{{cite journal |vauthors=Uchida H, Sakamoto S, Sasaki K, Takeda M, Hirata Y, Fukuda A, Hishiki T, Irie R, Nakazawa A, Miyazaki O, Nosaka S, Kasahara M |title=Surgical treatment strategy for advanced hepatoblastoma: Resection versus transplantation |journal=Pediatr Blood Cancer |volume=65 |issue=12 |pages=e27383 |date=December 2018 |pmid=30084209 |doi=10.1002/pbc.27383 |url=}}</ref>
* The type of liver cancer (hepatoblastoma or hepatocellular).
*The feasibility of [[surgery]] depends on the resectability of [[tumor]] at diagnosis.
* Whether the cancer has just been diagnosed or has recurred.


Prognosis may also depend on:
===Primary Prevention===
*There are no established measures for the primary [[Prevention (medical)|prevention]] of hepatoblastoma.


* Certain features of the cancer cell (how it looks under a microscope).
===Secondary Prevention===
* Whether the AFP blood levels go down after chemotherapy begins.
*There are no established measures for the [[secondary prevention]] of hepatoblastoma.


==References==
==References==
{{reflist}}
{{reflist|2}}


== External links ==
[[Category:Up-To-Date]]
*[http://www.cancer.umn.edu/hopestudy University of Minnesota hepatoblastoma epidemiology study]
[[Category:Oncology]]
*[http://survivor-support.rare-cancer.org/forum/ Hepatoblastoma Support Group]
[[Category:Medicine]]
*[http://www.lindseyann.net One Little Girl's Fight against Hepatoblastoma]
[[Category:Gastroenterology]]
{{SIB}}
[[Category:Surgery]]
[[Category:Hepatology]]
[[Category:Hepatology]]
[[Category:Oncology]]
</br>
[[de:Hepatoblastom]]
[[pt:Hepatoblastoma]]
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Latest revision as of 19:27, 13 March 2019


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Hepatoblastoma Microchapters

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Overview

Historical Perspective

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Epidemiology and Demographics

Risk Factors

Screening

Differentiating Hepatoblastoma from other Diseases

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

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Treatment

Medical Therapy

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

Synonyms and keywords: Epithelial hepatoblastoma; Fetal hepatoblastoma; Embryonal hepatoblatoma; Macrotrabecular hepatoblastoma; Mixed hepatoblastoma; Rhabdoid hepatoblastoma; Small cell hepatoblastoma; Undifferentiated hepatoblastoma; Cholangioblastic hepatoblastoma; Teratoid hepatoblastoma; Chondroid hepatoblastoma; Osteoid hepatoblastoma; Rhabdomyoblastic hepatoblastoma; Neuroid-melanocytic hepatoblastoma; Well differentiated hepatoblastoma; HB

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

Differentiating hepatoblastoma from Other Diseases

Epidemiology and Demographics

  • Hepatoblastoma is the most common primary liver cancer in infants and children, tumor involves right lobe of liver more often.[11]
  • The incidence/prevalence of hepatoblastoma is approximately 0.05–0.15 patients per 100000 population in children younger than 15 years.[12]
  • 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

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

  • Physical examination of patients with hepatoblastoma is usually remarkable for single, mildly painful, rapidly enlarging abdominal mass that is found in the right lobe of the liver.[18][10]
  • Most tumors are solitary; but can be multifocal as well.

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with hepatoblastoma.

X-ray

Ultrasound

  • Imaging studies play an important role in the diagnosis, staging, and treatment of disease, most tumors can be resected surgically and ultrasound is often used in order to detect tumor size, also the initial diagnosis is made by abdominal ultrasound.

CT scan

MRI

Other Imaging Findings

  • Other imaging studies such as PET scan or even bone scan when there is evidence of metastasis to bone may be helpful in the diagnosis of hepatoblastoma. PET scan can be helpful in localizing recurrent hepatoblastoma.[23][24]

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

  • Surgery is the mainstay of treatment for hepatoblastoma.[30]
  • The feasibility of surgery depends on the resectability of tumor at diagnosis.

Primary Prevention

  • There are no established measures for the primary prevention of hepatoblastoma.

Secondary Prevention

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. ↑ 10.0 10.1 10.2 10.3 10.4 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.
  11. ↑ 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.
  12. ↑ 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.
  13. ↑ 13.0 13.1 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.
  14. ↑ Musick SR, Babiker HM. PMID 30521216. Missing or empty |title= (help)
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ Ke HY, Chen JH, Jen YM, Yu JC, Hsieh CB, Chen CJ, Liu YC, Chen TW, Chan DC (October 2005). "Ruptured hepatoblastoma with massive internal bleeding in an adult". World J. Gastroenterol. 11 (39): 6235–7. PMC 4436650. PMID 16273660.
  18. ↑ Zhang Q, Ming J, Zhang S, Guo D, Qiu X (2013). "A rare case of adult hepatoblastoma with neuroendocrine differentiation misdiagnosed as neuroendocrine tumor". Int J Clin Exp Pathol. 6 (2): 308–13. PMC 3544231. PMID 23330017.
  19. ↑ Exelby PR, Filler RM, Grosfeld JL (June 1975). "Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical Section Survey--1974". J. Pediatr. Surg. 10 (3): 329–37. PMID 49416.
  20. ↑ Nickerson HJ, Silberman TL, McDonald TP (January 1980). "Hepatoblastoma, thrombocytosis, and increased thrombopoietin". Cancer. 45 (2): 315–7. PMID 6153151.
  21. ↑ Perilongo G, Brown J, Shafford E, Brock P, De Camargo B, Keeling JW, Vos A, Philips A, Pritchard J, Plaschkes J (October 2000). "Hepatoblastoma presenting with lung metastases: treatment results of the first cooperative, prospective study of the International Society of Paediatric Oncology on childhood liver tumors". Cancer. 89 (8): 1845–53. PMID 11042582.
  22. ↑ Aronson DC, Meyers RL (October 2016). "Malignant tumors of the liver in children". Semin. Pediatr. Surg. 25 (5): 265–275. doi:10.1053/j.sempedsurg.2016.09.002. PMID 27955729.
  23. ↑ Figarola MS, McQuiston SA, Wilson F, Powell R (December 2005). "Recurrent hepatoblastoma with localization by PET-CT". Pediatr Radiol. 35 (12): 1254–8. doi:10.1007/s00247-005-1568-6. PMID 16170514.
  24. ↑ Archer D, Babyn P, Gilday D, Greenberg MA (December 1993). "Potentially misleading bone scan findings in patients with hepatoblastoma". Clin Nucl Med. 18 (12): 1026–31. PMID 8293620.
  25. ↑ Nakagawara A, Ikeda K, Tsuneyoshi M, Daimaru Y, Enjoji M, Watanabe I, Iwafuchi M, Sawada T (October 1985). "Hepatoblastoma producing both alpha-fetoprotein and human chorionic gonadotropin. Clinicopathologic analysis of four cases and a review of the literature". Cancer. 56 (7): 1636–42. PMID 2411379.
  26. ↑ Morinaga S, Yamaguchi M, Watanabe I, Kasai M, Ojima M, Sasano N (May 1983). "An immunohistochemical study of hepatoblastoma producing human chorionic gonadotropin". Cancer. 51 (9): 1647–52. PMID 6187430.
  27. ↑ Pham TA, Gallo AM, Concepcion W, Esquivel CO, Bonham CA (December 2015). "Effect of Liver Transplant on Long-term Disease-Free Survival in Children With Hepatoblastoma and Hepatocellular Cancer". JAMA Surg. 150 (12): 1150–8. doi:10.1001/jamasurg.2015.1847. PMID 26308249.
  28. ↑ Reyes JD, Carr B, Dvorchik I, Kocoshis S, Jaffe R, Gerber D, Mazariegos GV, Bueno J, Selby R (June 2000). "Liver transplantation and chemotherapy for hepatoblastoma and hepatocellular cancer in childhood and adolescence". J. Pediatr. 136 (6): 795–804. PMID 10839879.
  29. ↑ Häberle B, Bode U, von Schweinitz D (2003). "[Differentiated treatment protocols for high- and standard-risk hepatoblastoma--an interim report of the German Liver Tumor Study HB99]". Klin Padiatr (in German). 215 (3): 159–65. doi:10.1055/s-2003-39375. PMID 12778356.
  30. ↑ Uchida H, Sakamoto S, Sasaki K, Takeda M, Hirata Y, Fukuda A, Hishiki T, Irie R, Nakazawa A, Miyazaki O, Nosaka S, Kasahara M (December 2018). "Surgical treatment strategy for advanced hepatoblastoma: Resection versus transplantation". Pediatr Blood Cancer. 65 (12): e27383. doi:10.1002/pbc.27383. PMID 30084209.
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