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==Differentiating Hepatoblastoma from other diseases==
==Differentiating Hepatoblastoma from other diseases==
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]].
==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==


Line 44: Line 44:


===Physical examination===
===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>
Common physical examination findings of hepatoblastoma include [[hepatomegaly]], [[abdominal distension]], [[Abdominal mass|asymptomatic palpable abdominal mass]], [[hemihypertrophy]], [[jaundice]], [[pyrexia]], and [[anemia]].
*Most tumors are [[solitary]]; but can be multifocal as well.
 
===Laboratory Findings===
===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>
Laboratory tests for liver function are usually normal among patients. An elevated concentration of [[alpha-fetoprotein]] is present in patients with hepatoblastoma.
**[[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)
**High levels of [[Alpha-fetoprotein]] ([[AFP]])
**Mild [[liver]] function disturbances


===Xray===
===Xray===
*[[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>
Findings on abdominal xray are nonspecific for hepatoblastoma and include [[Abdominal mass|right upper quadrant abdominal mass]] and/or calcifications in 10% of cases. [[Chest-X-Ray|Chest]] [[Chest X-ray|x-rays]] can be useful especially since this [[tumor]] has the affinity to [[metastasize]] to [[lungs]].


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


===MRI===
[[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.


===MRI===
*[[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>
===Ultrasound===
===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]].
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]]. Findings on ultrasound suggestive of hepatoblastoma include echogenic soft tissue mass, well defined lesion, and areas of shadowing due to intralesional calcifications.


===Biopsy===
===Biopsy===
Biopsy is the gold standard for the diagnosis of hepatoblastoma.  
Biopsy is the gold standard for the diagnosis of hepatoblastoma.  
===Other Imaging Findings===
===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>
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.


==Treatment==
==Treatment==
===Medical Therapy===
===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>
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]]. [[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.
*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>


===Surgery===
===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>
[[Surgery]] is the mainstay of treatment for hepatoblastoma. Liver [[transplantation]] can be considered for [[tumors]] that cannot be removed by surgery. The feasibility of [[surgery]] depends on the resectability of [[tumor]] at diagnosis.
*The feasibility of [[surgery]] depends on the resectability of [[tumor]] at diagnosis.


===Primary Prevention===
===Primary Prevention===
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===Secondary Prevention===
===Secondary Prevention===
*There are no established measures for the [[secondary prevention]] of hepatoblastoma.
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.
 
==References==
{{reflist|2}}


[[Category:Up-To-Date]]
[[Category:Up-To-Date]]

Latest revision as of 21:32, 13 March 2019

Hepatoblastoma Microchapters

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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

Classification

Hepatoblastoma can be divided into major and minor categories based on histology. Major categories constitutes epithelial, small cell undifferentiated and rhabdoid while minor constitutes cholangioblastic (ductal), keratinising squamous epithelium, intestinal glandular epithelium, teratoid (neuroid-melanocytic), rhabdomyoblastic, chondroid, and osteoid.

Pathophysiology

Development of hepatoblastoma is the result of multiple genetic mutations. Genes involved in the pathogenesis of hepatoblastoma include CTNNB1, CAPRIN2, SPOP, OR5I1, and CDC20B. On gross pathology, hepatoblastoma is characterized by a solitary, large, well circumscribed mass with heterogeneous cut surface. On microscopic histopathological analysis, hepatoblastoma is characterized by small round cell tumor, fetal hepatocytes ~ 1:3 nuclear-cytoplasmic ratio, eosinophilic cytoplasm (mesenchymal component), and immature fibrous tissue osteoid or cartilage. Hepatoblastoma is demonstrated by positivity to alpha-fetoprotein, hepatocyte specific antigen (especially in fetal component), and beta-catenin (cytoplasmic and nuclear).

Hepatoblastoma H&Esource:wikipedia


Causes

There are no specific causes of hepatoblastoma and most tumors are sporadic but there are some risk factors and conditions that have a strong association with this tumor such as beckwith-Weidemann syndrome, familial adenomatous polyposis (FAP), down syndrome, edward syndrome (trisomy 18).

Epidemiology and Demographics

Hepatoblastoma is a common tumor that tends to affect children aged less than five years. Males are more commonly affected with hepatoblastoma than females. The annual incidence of hepatoblastoma in the United States appears to have doubled from 8 (1975-1983) to 16 (2002-2009) per 100,000 children aged 19 years and younger.

Risk factors

Common risk factors in the development of hepatoblastoma include 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

According to the American Association for the Study of Liver Diseases and United States Preventive Services Task Force, there is insufficient evidence to recommend routine screening for hepatoblastoma. However, ultrasound is the recommended for suspected hepatic lesions in children.

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.

Natural History, Complications and Prognosis

If left untreated, patients with hepatoblastoma may progress to develop failure to thrive, weight loss, rapidly enlarging abdominal mass, spontaneous rupture, hemorrhage. Common complications of hepatoblastoma include paraneoplastic features, erythrocytosis, thrombocytosis, hypocalcemia, isosexual precocious puberty, and hypoglycemia. The 5-year survival rate of children with hepatoblastoma is approximately 70%.

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.

Screening

The staging of hepatoblastoma is based on the Intergroup staging system.

History and Symptoms

The majority of patients with hepatoblastoma have an abdominal mass or abdominal distension. Other symptoms include abdominal pain, weight loss, loss of appetite, early puberty in boys, jaundice, nausea, vomiting, back pain, failure to thrive, and rapidly enlarging abdominal mass.

Physical examination

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

Laboratory Findings

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

Xray

Findings on abdominal xray are nonspecific for hepatoblastoma and include right upper quadrant abdominal mass and/or calcifications in 10% of cases. Chest x-rays can be useful especially since this tumor has the affinity to metastasize to lungs.

CT

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

MRI

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.

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. Findings on ultrasound suggestive of hepatoblastoma include echogenic soft tissue mass, well defined lesion, and areas of shadowing due to intralesional calcifications.

Biopsy

Biopsy is the gold standard for the diagnosis of hepatoblastoma.

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.

Treatment

Medical Therapy

The mainstay of therapy for hepatoblastoma is surgery. However, the vast majority of the tumors cannot be completely resected because of their large size or metastasis. 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.


Surgery

Surgery is the mainstay of treatment for hepatoblastoma. Liver transplantation can be considered for tumors that cannot be removed by surgery. The feasibility of surgery depends on the resectability of tumor at diagnosis.

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.

References


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