Hepatoblastoma overview: Difference between revisions
Nawal Muazam (talk | contribs) No edit summary |
Nawal Muazam (talk | contribs) No edit summary |
||
Line 21: | Line 21: | ||
Hepatoblastoma may be classified into two subtypes based on histology, major category and minor category<ref name=a>Radiopaedia 2015 Hepatoblastoma>{{cite web | title = Radiopedia 2015 Hepatoblastoma [Dr Frank Gaillard]| url = http://radiopaedia.org/articles/hepatoblastoma-histological-classification }}</ref> | Hepatoblastoma may be classified into two subtypes based on histology, major category and minor category<ref name=a>Radiopaedia 2015 Hepatoblastoma>{{cite web | title = Radiopedia 2015 Hepatoblastoma [Dr Frank Gaillard]| url = http://radiopaedia.org/articles/hepatoblastoma-histological-classification }}</ref> | ||
==Pathophysiology== | ==Pathophysiology== | ||
Development of hepatoblastoma is the result of multiple genetic mutations. Genes involved in the pathogenesis of hepatoblastoma include ''[[Beta-catenin|CTNNB1]]'', ''CAPRIN2'', ''[[SPOP]]'', ''[[OR5I1]]'', ''CDC20B''. On gross pathology, hepatoblastoma is characterized by well circumscribed large masses, usually single, with heterogeneous cut surface.<ref name=pathology>Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref> On microscopic histopathological analysis, hepatoblastoma is characterized by: small round cell tumor, fetal hepatocytes ~ 1:3 NC ratio, eosinophilic cytoplasm (mesenchymal component), immature fibrous tissue osteoid or cartilage.<ref name=microscopy>Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015</ref> Hepatoblastoma is demonstrated by positivity to alpha-fetoprotein, Hepatocyte specific antigen (especially in fetal component), ''Beta-catenin'' (cytoplasmic and nuclear)<ref name=microscopy>IHC of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 4, 2015</ref> | |||
==Causes== | ==Causes== | ||
There are no established causes for hepatoblastoma. The etiology of hepatoblastoma has been elusive.<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> | |||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
==Risk factors== | ==Risk factors== | ||
==Screening== | ==Screening== |
Revision as of 22:15, 4 November 2015
Hepatoblastoma Microchapters |
Diagnosis |
---|
Treatment |
Case Studies |
Hepatoblastoma overview On the Web |
American Roentgen Ray Society Images of Hepatoblastoma overview |
Risk calculators and risk factors for Hepatoblastoma overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]
Overview
Hepatoblastoma is an uncommon malignant liver neoplasm occurring in infants and children and composed of tissue resembling fetal or mature liver cells or bile ducts. 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.[1]
Historical Perspective
Hepatoblastoma was first described in 1958 by I. Bartok.[2]
Classification
Hepatoblastoma may be classified into two subtypes based on histology, major category and minor category[3]
Pathophysiology
Development of hepatoblastoma is the result of multiple genetic mutations. Genes involved in the pathogenesis of hepatoblastoma include CTNNB1, CAPRIN2, SPOP, OR5I1, CDC20B. On gross pathology, hepatoblastoma is characterized by well circumscribed large masses, usually single, with heterogeneous cut surface.[4] On microscopic histopathological analysis, hepatoblastoma is characterized by: small round cell tumor, fetal hepatocytes ~ 1:3 NC ratio, eosinophilic cytoplasm (mesenchymal component), immature fibrous tissue osteoid or cartilage.[5] Hepatoblastoma is demonstrated by positivity to alpha-fetoprotein, Hepatocyte specific antigen (especially in fetal component), Beta-catenin (cytoplasmic and nuclear)[5]
Causes
There are no established causes for hepatoblastoma. The etiology of hepatoblastoma has been elusive.[2]
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
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
References
- ↑ De Ioris M, Brugieres L, Zimmermann A, Keeling J, Brock P, Maibach R, Pritchard J, Shafford L, Zsiros J, Czaudzerna P, Perilongo G (2007). "Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience". Eur J Cancer. 44: 545. doi:10.1016/j.ejca.2007.11.022. PMID 18166449.
- ↑ 2.0 2.1 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.
- ↑ Radiopaedia 2015 Hepatoblastoma>"Radiopedia 2015 Hepatoblastoma [Dr Frank Gaillard]".
- ↑ Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015
- ↑ 5.0 5.1 Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015