Hepatoblastoma natural history: Difference between revisions

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__NOTOC__
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{{Hepatoblastoma}}
{{Hepatoblastoma}}
{{CMG}} {{AE}}{{Faizan}} {{NM}}
{{CMG}} {{AE}}{{Faizan}} {{NM}}
==Overview==
==Overview==
 
If left untreated, patients with hepatoblastoma may progress to develop [[failure to thrive]], [[weight loss]], [[Abdominal mass|rapidly enlarging abdominal mass]], spontaneous rupture, [[hemorrhage]]. Common complications of hepatoblastoma include paraneoplastic features, [[erythrocytosis]], [[thrombocytosis]], [[hypocalcemia]], [[Precocious puberty|isosexual precocious puberty]], and [[hypoglycemia]]. The 5-year survival rate of children with hepatoblastoma is approximately 70%.
==Natural History==
==Natural History==
Hepatoblastoma tends to grow rapidly. If left untreated, patients with hepatoblastoma may progress to develop [[failure to thrive]], [[weight loss]], [[Abdominal mass|rapidly enlarging abdominal mass]], spontaneous rupture, hemorrhage.<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><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>
==Complications==
==Complications==
Paraneoplastic features of hepatoblastoma are not uncommon at presentation and include:<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>
*[[Erythrocytosis]]
*[[Thrombocytosis]]
*[[Hypocalcemia]]
*[[Precocious puberty|Isosexual precocious puberty]]
*[[Hypoglycemia]]
==Prognosis==
==Prognosis==
* 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>
* 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>
 
* The table below lists prognosis of hepatoblastma based on staging:<ref name="differential">Treatment and prognosis of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref>
* The table below lists prognosis of hepatoblastma based on staging:<ref name=prognosis>Treatment and prognosis of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref>


{| style="border: 100px; font-size: 90%; margin: 3px; width: 400px"
{| style="border: 100px; font-size: 90%; margin: 3px; width: 400px"
|valign=top|
| valign="top" |
|+
|+
! style="background: #4479BA; width: 250px; color: #FFFFFF;"|'''Subtype'''
! style="background: #4479BA; width: 250px; color: #FFFFFF;" |'''Subtype'''


! style="background: #4479BA; width: 600px; color: #FFFFFF;"|'''Description'''
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |'''Description'''


|-
|-


| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Stage I'''|| style="padding: 5px 5px; background: #F5F5F5;"|
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Stage I'''|| style="padding: 5px 5px; background: #F5F5F5;" |
* Up to 100%
* Up to 100%


|-
|-


| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Stage II'''|| style="padding: 5px 5px; background: #F5F5F5;"|
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Stage II'''|| style="padding: 5px 5px; background: #F5F5F5;" |
* 75-80%
* 75-80%
|-
|-


| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Stage III'''|| style="padding: 5px 5px; background: #F5F5F5;"|
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Stage III'''|| style="padding: 5px 5px; background: #F5F5F5;" |
* 65%
* 65%
|-
|-


| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Stage IV'''|| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Stage IV'''|| style="padding: 5px 5px; background: #F5F5F5;" |
* 0-27%  
* 0-27%  


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|}
|}
 
:
====Factors that Affect Prognosis (chance of recovery) and Treatment Options====
:*Prognosis is based on different factors including:<ref name="pmid30521216">{{cite journal |vauthors=Musick SR, Babiker HM |pmid=30521216|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><ref name="pmid18166449">{{cite journal
* The 5-year survival rate of children with hepatoblastoma is approximately 70%.
| author = De Ioris M, Brugieres L, Zimmermann A, Keeling J, Brock P, Maibach R, Pritchard J, Shafford L, Zsiros J, Czaudzerna P, Perilongo G
* The prognosis (chance of recovery) and treatment options depend on the following:
| title = Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience.
 
| journal = Eur J Cancer
:* The stage of the cancer (the size of the [[tumor]]
| volume = 44
:* Whether it affects part or all of the [[liver]]
| issue =
:* Whether it has spread to other places in the body, such as the [[lungs]]).
| pages = 545
:* Whether the cancer can be removed completely by surgery.
| year = 2007
:* The type of liver cancer (hepatoblastoma or hepatocellular).
| pmid = 18166449
:* Whether the cancer has just been diagnosed or has recurred.
| doi = 10.1016/j.ejca.2007.11.022
 
| issn =
* Prognosis may also depend on:
}}</ref>
 
:**Age of diagnosis, younger children have a better prognosis.
:* Certain features of the cancer cell (determined microscopically).
:**[[Metastases]]
:* Whether the [[AFP]] blood levels go down after [[chemotherapy]] is initiated.
:**[[Alpha-fetoprotein]] ([[AFP]]) levels
:***Drop in [[Alpha-fetoprotein|AFP]] level after [[chemotherapy]] means better response to treatment.
:**[[Histologic]] subtype
:***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>


==References==
==References==
{{reflist|1}}
{{reflist|2}}


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Latest revision as of 20:35, 13 March 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2] Nawal Muazam M.D.[3]

Overview

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%.

Natural History

Hepatoblastoma tends to grow rapidly. If left untreated, patients with hepatoblastoma may progress to develop failure to thrive, weight loss, rapidly enlarging abdominal mass, spontaneous rupture, hemorrhage.[1][2]

Complications

Paraneoplastic features of hepatoblastoma are not uncommon at presentation and include:[1]

Prognosis

  • The 5-year survival rate of children with hepatoblastoma is approximately 70%.[2]
  • The table below lists prognosis of hepatoblastma based on staging:[3]
Subtype Description
Stage I
  • Up to 100%
Stage II
  • 75-80%
Stage III
  • 65%
Stage IV
  • 0-27%

References

  1. 1.0 1.1 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.
  2. 2.0 2.1 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
  3. Treatment and prognosis of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015
  4. Musick SR, Babiker HM. PMID 30521216. Missing or empty |title= (help)
  5. 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. 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.
  7. 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.


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