Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/01/08 06:46:15」(JST)

wiki en

Hepatoblastoma is an uncommon malignant liver cancer occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells. They usually present with an abdominal mass. The disease is most commonly diagnosed during a child's first three years of life.^[1] Alpha-fetoprotein (AFP) levels are commonly elevated, but when AFP is not elevated at diagnosis the prognosis is poor.^[2]

Symptoms

Pathophysiology

Micrograph showing a hepatoblastoma (right of image) and normal liver (left of image). H&E stain.

Hepatoblastomas originate from immature liver precursor cells, usually unifocal and affect the right lobe of the liver more often than the left lobe, can metastasize. They are categorized into two types: "Epithelial Type" and "Mixed Epithelial / Mesenchymal Type."

Individuals with familial adenomatous polyposis (FAP), a syndrome of early-onset colonic polyps and adenocarcinoma, frequently develop hepatoblastomas.^[3]^[4] Also beta-catenin mutations have been shown to be common in sporadic hepatoblastomas, occurring in as many as 67% of patients.^[5]^[6]

Recently on other components of the Wnt signaling pathway have also demonstrated a likely role for constitutive activation of this pathway in the etiology of hepatoblastoma.^[6]^[7]Accumulating evidence suggests that hepatoblastoma is derived from a pluripotent stem cell.^[8]

Diagnosis

Treatment

Human liver(transplantation is treatment option)

The most common method of testing for hepatoblastoma is a blood test checking the alpha-fetoprotein level. Alpha-fetoprotein (AFP) is used as a biomarker to help determine the presence of liver cancer in children. At birth, infants have relatively high levels of AFP, which fall to normal adult levels by the first year of life. The normal level for AFP in children has been reported as lower than 50 nanograms per milliliter (ng/ml) and 10 ng/ml. An AFP level greater than 500 (ng/ml) is a significant indicator of hepatoblastoma. AFP is also used as an indicator of treatment success. If treatments are successful in removing the cancer, the AFP level is expected to return to normal.^[9]

Surgical removal of the tumor, adjuvant chemotherapy prior to tumor removal, and liver transplantation have been used to treat these cancers.^[10]^[11] Primary liver transplantation provides high, long term, disease-free survival rate in the range of 80%, in cases of complete tumor removal and adjuvant chemotherapy survival rates approach 100%.^[12]^[13] The presence of metastases is the strongest predictor of a poor prognosis.^[14]

References

^ http://hepatoblastoma.mditv.com/generalinfo^{[full citation needed]}
^ 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 (4): 545–50. doi:10.1016/j.ejca.2007.11.022. PMID 18166449.
^ Hirschman BA, Pollock BH, Tomlinson GE (August 2005). "The spectrum of APC mutations in children with hepatoblastoma from familial adenomatous polyposis kindreds". J. Pediatr. 147 (2): 263–6. doi:10.1016/j.jpeds.2005.04.019. PMID 16126064.
^ Sanders RP, Furman WL (November 2006). "Familial adenomatous polyposis in two brothers with hepatoblastoma: implications for diagnosis and screening". Pediatr Blood Cancer. 47 (6): 851–4. doi:10.1002/pbc.20556. PMID 16106429.
^ Anna CH, Sills RC, Foley JF, Stockton PS, Ton TV, Devereux TR (June 2000). "Beta-catenin mutations and protein accumulation in all hepatoblastomas examined from B6C3F1 mice treated with anthraquinone or oxazepam". Cancer Res. 60 (11): 2864–8. PMID 10850429.
^ ^a ^b Tan, Xinping; Apte, Udayan; Micsenyi, Amanda; Kotsagrelos, Emorphia; Luo, Jian-Hua; Ranganathan, Sarangarajan; Monga, Dulabh K.; Bell, Aaron; Michalopoulos, George K.; Monga, Satdarshan P.S. (2005). "Epidermal Growth Factor Receptor: A Novel Target of the Wnt/β-Catenin Pathway in Liver". Gastroenterology. 129 (1): 285–302. doi:10.1053/j.gastro.2005.04.013. PMC 1821080. PMID 16012954.
^ Koch A, Waha A, Hartmann W, et al. (June 2005). "Elevated expression of Wnt antagonists is a common event in hepatoblastomas". Clin. Cancer Res. 11 (12): 4295–304. doi:10.1158/1078-0432.CCR-04-1162. PMID 15958610.
^ Ruck P, Xiao JC (November 2002). "Stem-like cells in hepatoblastoma". Med. Pediatr. Oncol. 39 (5): 504–7. doi:10.1002/mpo.10175. PMID 12228907.
^ Sarto, I.; Klausberger, T.; Ehya, N.; Mayer, B.; Fuchs, K.; Sieghart, W. (2002). "A novel site on gamma 3 subunits important for assembly of GABA(A) receptors". The Journal of Biological Chemistry. 277 (34): 30656–64. doi:10.1074/jbc.M203597200. PMID 12065588.
^ Ang JP, Heath JA, Donath S, Khurana S, Auldist A (February 2007). "Treatment outcomes for hepatoblastoma: an institution's experience over two decades". Pediatr. Surg. Int. 23 (2): 103–9. doi:10.1007/s00383-006-1834-1. PMID 17119981.
^ Otte JB, Pritchard J, Aronson DC, et al. (January 2004). "Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience". Pediatr Blood Cancer. 42 (1): 74–83. doi:10.1002/pbc.10376. PMID 14752798.
^ Pediatric Hepatoblastoma at eMedicine
^ Otte JB, de Ville de Goyet J, Reding R (October 2005). "Liver transplantation for hepatoblastoma: indications and contraindications in the modern era". Pediatr Transplant. 9 (5): 557–65. doi:10.1111/j.1399-3046.2005.00354.x. PMID 16176410.
^ Czauderna P, Mackinlay G, Perilongo G, et al. (June 2002). "Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group". J. Clin. Oncol. 20 (12): 2798–804. doi:10.1200/JCO.2002.06.102. PMID 12065556.

External links

humpath #2775 (Pathology images)
University of Minnesota hepatoblastoma epidemiology study
What is Hepatoblastoma?

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 悪性肝腫瘍の病理 pathology of malignant liver tumors
2. ベックウィズ・ヴィーデマン症候群 beckwith wiedemann syndrome
3. ガードナー症候群 gardner syndrome
4. Clinical presentation, diagnosis, and staging evaluation of neuroblastoma
5. 固形肝病変：鑑別診断および評価 solid liver lesions differential diagnosis and evaluation

English Journal

Cholesterol homeostasis and autophagic flux in perifosine-treated human hepatoblastoma HepG2 and glioblastoma U-87 MG cell lines.

Ríos-Marco P1, Ríos A2, Jiménez-López JM1, Carrasco MP3, Marco C4.
Biochemical pharmacology.Biochem Pharmacol.2015 Jul 1;96(1):10-9. doi: 10.1016/j.bcp.2015.04.015. Epub 2015 Apr 28.
Perifosine exerts an antiproliferative effect on HepG2 and U-87 MG cells and also interferes with the transport of cholesterol from the plasma membrane to the endoplasmic reticulum (ER). Recently we demonstrated that exposure of U-87 MG cells to perifosine causes an accumulation of autophagosomes. W
PMID 25934232

Pyrrole-imidazole polyamide-mediated silencing of KCNQ1OT1 expression induces cell death in Wilms' tumor cells.

Yoshizawa S1, Fujiwara K2, Sugito K1, Uekusa S1, Kawashima H1, Hoshi R1, Watanabe Y1, Hirano T1, Furuya T1, Masuko T1, Ueno T3, Fukuda N3, Soma M2, Ozaki T4, Koshinaga T1, Nagase H5.
International journal of oncology.Int J Oncol.2015 Jul;47(1):115-21. doi: 10.3892/ijo.2015.3018. Epub 2015 May 22.
KvDMR (an intronic CpG island within the KCNQ1 gene) is one of the imprinting control regions on human chromosome 11p15.5. Since KvDMR exists within the promoter region of KCNQ1OT1 (antisense transcript of KCNQ1), it is likely that genomic alterations of this region including deletion, paternal unip
PMID 25998555

Associating liver partition with portal vein ligation and staged hepatectomy (ALPPS) for the treatment of liver tumors in children.

Wiederkehr JC1, Avilla SG2, Mattos E2, Coelho IM2, Ledesma JA3, Conceição AF2, Wiederkehr HA2, Wiederkehr BA2.
Journal of pediatric surgery.J Pediatr Surg.2015 Jul;50(7):1227-31. doi: 10.1016/j.jpedsurg.2014.10.019.
Resection is the only curative treatment option for primary and secondary malignant tumors of the liver. Although curative resection is associated with long-term survival rates, it can only be performed in 10% of patients with primary tumors and 25% of patients with liver metastases. Liver insuffici
PMID 25783345

Japanese Journal

超低出生体重児にWilson-Mikity症候群とBeckwith-Wiedemann症候群を伴った肝芽腫の1例

吉澤信輔,杉藤公信,蘇我晶子,植草省太,金田英秀,古屋武史,大橋研介,井上幹也,池田太郎,越永従道
日本小児外科学会雑誌 48(6), 956-960, 2012-10-20
超低出生体重児にWilson-Mikity症候群とBeckwith-Wiedemann症候群(BWS)を伴った肝芽腫の1例を報告する.症例は,8か月の女児.BWSに伴う腫瘍のスクリーニング検査で,肝腫瘤と血清alpha-fetoprotein (AFP)高値を指摘され紹介となった.Wilson-Mikity症候群による肺気腫と肺高血圧症により呼吸循環動態が不安定であり,開腹腫瘍生検は行わずに化学療 …
NAID 110009544285

症例報告超低出生体重児にWilson-Mikity症候群とBeckwith-Wiedemann症候群を伴った肝芽腫の1例

吉澤信輔,杉藤公信,蘇我晶子 [他]
日本小児外科学会雑誌 48(6), 956-960, 2012-10-00
NAID 40019466188

ピラルビシン(THP)の小児固形がんにおける保険適応外使用に関するアンケート調査の結果

池田均,森川康英,福澤正洋 [他],檜山英三
日本小児外科学会雑誌 48(4), 727-730, 2012-06-20
【目的】ピラルビシン(THP)の小児固形がんにおける保険適応外使用の状況を把握する.【対象と方法】日本小児外科学会認定施設および教育関連施設を対象とし,無記名(匿名)のアンケート調査を実施した.【結果】対象施設144施設のうち92施設(63.9%)から回答を得た.過去5年間に小児固形がんの治療経験があったのは62施設(67.4%)で,腫瘍別のTHP使用施設の割合は神経芽腫が56施設中47施設(83 …
NAID 110009470926

Related Pictures

★リンクテーブル★

リンク元

「肝芽腫」

Hepatoblastoma
	Micrograph of a hepatoblastoma. H&E stain.
Classification and external resources
Specialty	oncology
ICD-10	C22.0
ICD-O	M8970/3
DiseasesDB	5799
eMedicine	ped/982 radio/331
MeSH	D018197
	[edit on Wikidata]

　　[★]

英: hepatoblastoma
同: 肝芽細胞腫、ヘパトブラストーマ
関: 肝細胞癌

[1] ttp://hepatoblastoma.mditv.com/generalinfo^{[full citation needed]}

[pmid18166449-2] 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 (4): 545–50. doi:10.1016/j.ejca.2007.11.022. PMID 18166449.

[pmid16126064-3] Hirschman BA, Pollock BH, Tomlinson GE (August 2005). "The spectrum of APC mutations in children with hepatoblastoma from familial adenomatous polyposis kindreds". J. Pediatr. 147 (2): 263–6. doi:10.1016/j.jpeds.2005.04.019. PMID 16126064.

[journal-4] Sanders RP, Furman WL (November 2006). "Familial adenomatous polyposis in two brothers with hepatoblastoma: implications for diagnosis and screening". Pediatr Blood Cancer. 47 (6): 851–4. doi:10.1002/pbc.20556. PMID 16106429.

[pmid10850429-5] Anna CH, Sills RC, Foley JF, Stockton PS, Ton TV, Devereux TR (June 2000). "Beta-catenin mutations and protein accumulation in all hepatoblastomas examined from B6C3F1 mice treated with anthraquinone or oxazepam". Cancer Res. 60 (11): 2864–8. PMID 10850429.

[pmid16012954-6] Tan, Xinping; Apte, Udayan; Micsenyi, Amanda; Kotsagrelos, Emorphia; Luo, Jian-Hua; Ranganathan, Sarangarajan; Monga, Dulabh K.; Bell, Aaron; Michalopoulos, George K.; Monga, Satdarshan P.S. (2005). "Epidermal Growth Factor Receptor: A Novel Target of the Wnt/β-Catenin Pathway in Liver". Gastroenterology. 129 (1): 285–302. doi:10.1053/j.gastro.2005.04.013. PMC 1821080. PMID 16012954.

[pmid15958610-7] Koch A, Waha A, Hartmann W, et al. (June 2005). "Elevated expression of Wnt antagonists is a common event in hepatoblastomas". Clin. Cancer Res. 11 (12): 4295–304. doi:10.1158/1078-0432.CCR-04-1162. PMID 15958610.

[pmid12228907-8] Ruck P, Xiao JC (November 2002). "Stem-like cells in hepatoblastoma". Med. Pediatr. Oncol. 39 (5): 504–7. doi:10.1002/mpo.10175. PMID 12228907.

[pmid12065588-9] Sarto, I.; Klausberger, T.; Ehya, N.; Mayer, B.; Fuchs, K.; Sieghart, W. (2002). "A novel site on gamma 3 subunits important for assembly of GABA(A) receptors". The Journal of Biological Chemistry. 277 (34): 30656–64. doi:10.1074/jbc.M203597200. PMID 12065588.

[pmid17119981-10] Ang JP, Heath JA, Donath S, Khurana S, Auldist A (February 2007). "Treatment outcomes for hepatoblastoma: an institution's experience over two decades". Pediatr. Surg. Int. 23 (2): 103–9. doi:10.1007/s00383-006-1834-1. PMID 17119981.

[pmid14752798-11] Otte JB, Pritchard J, Aronson DC, et al. (January 2004). "Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience". Pediatr Blood Cancer. 42 (1): 74–83. doi:10.1002/pbc.10376. PMID 14752798.

[12] Pediatric Hepatoblastoma at eMedicine

[pmid16176410-13] Otte JB, de Ville de Goyet J, Reding R (October 2005). "Liver transplantation for hepatoblastoma: indications and contraindications in the modern era". Pediatr Transplant. 9 (5): 557–65. doi:10.1111/j.1399-3046.2005.00354.x. PMID 16176410.

[pmid12065556-14] Czauderna P, Mackinlay G, Perilongo G, et al. (June 2002). "Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group". J. Clin. Oncol. 20 (12): 2798–804. doi:10.1200/JCO.2002.06.102. PMID 12065556.

匿名

検索

案内

案内

hepatoblastoma