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  1. Zainal Abidin H, Mohd Lutfi N, Phang YY, Zarina FMI, Hamidah I, Saedah A, et al.
    A A Pract, 2020 Jul;14(9):e01281.
    PMID: 32909720 DOI: 10.1213/XAA.0000000000001281
    Hepatoblastoma is the most frequently occurring malignant tumor of the liver in children (ages ≤5 years). The formation of bronchobiliary fistula is a rare complication. We present a case report that describes the associated anesthetic challenges that we encountered for the treatment of this pathology.
    Matched MeSH terms: Hepatoblastoma
  2. Oh L, Hafsi H, Hainaut P, Ariffin H
    Curr Opin Oncol, 2019 03;31(2):84-91.
    PMID: 30585860 DOI: 10.1097/CCO.0000000000000504
    PURPOSE OF REVIEW: Childhood blastomas, unlike adult cancers, originate from developing organs in which molecular and cellular features exhibit differentiation arrest and embryonic characteristics. Conventional cancer therapies, which rely on the generalized cytotoxic effect on rapidly dividing cells, may damage delicate organs in young children, leading to multiple late effects. Deep understanding of the biology of embryonal cancers is crucial in reshaping the cancer treatment paradigm for children.

    RECENT FINDINGS: p53 plays a major physiological role in embryonic development, by controlling cell proliferation, differentiation and responses to cellular stress. Tumor suppressor function of p53 is commonly lost in adult cancers through genetic alterations. However, both somatic and germline p53 mutations are rare in childhood blastomas, suggesting that in these cancers, p53 may be inactivated through other mechanisms than mutation. In this review, we summarize current knowledge about p53 pathway inactivation in childhood blastomas (specifically neuroblastoma, retinoblastoma and Wilms' tumor) through various upstream mechanisms. Laboratory evidence and clinical trials of targeted therapies specific to exploiting p53 upstream regulators are discussed.

    SUMMARY: Despite the low rate of inherent TP53 mutations, p53 pathway inactivation is a common denominator in childhood blastomas. Exploiting p53 and its regulators is likely to translate into more effective targeted therapies with minimal late effects for children. (see Video Abstract, Supplemental Digital Content 1, http://links.lww.com/COON/A23).

    Matched MeSH terms: Hepatoblastoma/metabolism*; Hepatoblastoma/pathology
  3. Al-Qubaisi M, Rosli R, Subramani T, Omar AR, Yeap SK, Ali AM, et al.
    Nat Prod Res, 2013;27(23):2216-8.
    PMID: 23767409 DOI: 10.1080/14786419.2013.800979
    Goniothalamin is a biologically active styrylpyrone derivative isolated from various Goniothalamus species. The ability of goniothalamin to induce apoptosis via caspase-3 activation against hepatoblastoma (HepG2) and normal liver cells (Chang cells) was studied using morphological and biochemical evaluations. HepG2 and Chang cells were treated with goniothalamin for 72 h and analysed by TUNEL and Annexin-V/PI staining. Furthermore, the post-mitochondrial caspase-3 was quantified using ELISA. In view of our results, goniothalamin induced apoptosis on treated cells via alteration of cellular membrane integrity and cleavage of DNA. On the other hand, post-mitochondrial caspase-3 activity was significantly elevated in HepG2 cells treated with goniothalamin after 72 h. These findings suggest that goniothalamin induced apoptosis on HepG2 liver cancer cells via induction of caspase-3 with less sensitivity on the cell line of Chang cells.
    Matched MeSH terms: Hepatoblastoma/enzymology; Hepatoblastoma/pathology*
  4. Al-Qubaisi M, Rozita R, Yeap SK, Omar AR, Ali AM, Alitheen NB
    Molecules, 2011 Apr 06;16(4):2944-59.
    PMID: 21471934 DOI: 10.3390/molecules16042944
    Liver cancer has become one of the major types of cancer with high mortality and liver cancer is not responsive to the current cytotoxic agents used in chemotherapy. The purpose of this study was to examine the in vitro cytotoxicity of goniothalamin on human hepatoblastoma HepG2 cells and normal liver Chang cells. The cytotoxicity of goniothalamin against HepG2 and liver Chang cell was tested using MTT cell viability assay, LDH leakage assay, cell cycle flow cytometry PI analysis, BrdU proliferation ELISA assay and trypan blue dye exclusion assay. Goniothalamin selectively inhibited HepG2 cells [IC₅₀ = 4.6 (±0.23) µM in the MTT assay; IC₅₀ = 5.20 (±0.01) µM for LDH assay at 72 hours], with less sensitivity in Chang cells [IC₅₀ = 35.0 (±0.09) µM for MTT assay; IC₅₀ = 32.5 (±0.04) µM for LDH assay at 72 hours]. In the trypan blue dye exclusion assay, the Viability Indexes were 52 ± 1.73% for HepG2 cells and 62 ± 4.36% for Chang cells at IC₅₀ after 72 hours. Cytotoxicity of goniothalamin was related to inhibition of DNA synthesis, as revealed by the reduction of BrdU incorporation. At 72 hours, the lowest concentration of goniothalamin (2.3 µL) retained 97.6% of normal liver Chang cells proliferation while it reduced HepG2 cell proliferation to 19.8% as compared to control. Besides, goniothalamin caused accumulation of hypodiploid apoptosis and different degree of G2/M arrested as shown in cell cycle analysis by flow cytometry. Goniothalamin selectively killed liver cancer cell through suppression of proliferation and induction of apoptosis. These results suggest that goniothalamin shows potential cytotoxicity against hepatoblastoma HepG2 cells.
    Matched MeSH terms: Hepatoblastoma/pathology*
  5. Lee WS, Grundy R, Milford DV, Taylor CM, de Ville de Goyet J, McKiernan PJ, et al.
    Pediatr Transplant, 2003 Aug;7(4):270-6.
    PMID: 12890004
    Combination of cyclosporine (CsA) and tacrolimus immunosuppression post-liver transplantation (LT) and the chemotherapeutic drugs used to treat hepatoblastoma (HB), are nephrotoxic. We aimed to determine the severity and duration of nephrotoxicity in children following LT for unresectable HB. We reviewed all children undergoing LT for unresectable HB at the Liver Unit, Birmingham Children's Hospital, UK, from 1991 to July 2000. Thirty-six children undergoing LT for biliary atresia, matched for age and sex, were selected as controls to compare pre- and post-LT renal function. Renal function was determined by estimation of glomerular filtration rate (eGFR) derived from plasma creatinine using Schwartz's formula. Twelve children with HB (mean age of diagnosis 33 months) who underwent LT (mean age 47 months) and 36 controls (mean age of LT 34 months) were studied. CsA was the main immunosuppressive drug used in each group. The median eGFR before, and at 3, 6, 12, 24 and 36 months after LT in HB group was significantly lower than controls (93 vs. 152, 66 vs. 79, 62 vs. 86, 66 vs. 87, 64 vs. 94, 53 vs. 90 mL/min/1.73 m2, respectively; 0.01 < p < 0.03). The reductions in the median eGFR of both the HB group and controls before and at 36 months after LT were 49 and 41%, respectively. At 36 months after LT, there was a trend for partial recovery of the eGFR in the controls but not in the HB group. Children who underwent LT for unresectable HB had renal dysfunction before transplantation that persisted for 36 months after LT.
    Matched MeSH terms: Hepatoblastoma/surgery*
  6. Azlin AH, Looi LM, Cheah PL
    Asian Pac J Cancer Prev, 2014;15(9):3959-63.
    PMID: 24935581
    The tumour suppressor genes, p53 and pRb, are known to play important roles in neoplastic transformation. While molecular routes to the uncontrolled growth of hepatocytes, leading to primary liver cancer have generated considerable interest, the roles of p53 and pRb mutations in hepatocellular carcinoma (HCC) and hepatoblastoma (HB) remain to be clarified. We examined the immunohistochemical expression of p53 and pRb gene products in 26 HCC and 9 HB, sampled into tissue microarray blocks. 10 (38%) of 26 HCC showed > 10% tumour nuclear staining for p53 protein, 3 of these also being HbsAg positive. Conversely, none of 9 HB expressed nuclear p53 immunopositivity. Some 24 (92%) HCC and 8 (89%) HB showed loss of pRb nuclear expression. Two of the 26 HCC and one of the 9 HB showed >10% tumour nuclear staining for pRb protein. Our results suggest that p53 does not have an important role in the development of HB but may contribute in HCC. There is also loss of pRb expression in the majority of HCC and HB, supporting loss of pRb gene function in the hepatocarcinogenesis pathway. However, a comparison of the staining profiles of p53 and pRb proteins in HCC and HB did not reveal a consistent pattern to differentiate between the two types of tumours immunohistochemically. Hence the use of p53 and pRB protein expression has no contribution in the situation where there is a diagnostic difficulty in deciding between HCC and HB.
    Matched MeSH terms: Hepatoblastoma/genetics*; Hepatoblastoma/pathology
  7. Norsarwany M, Abdelrahman Z, Rahmah N, Ariffin N, Norsyahida A, Madihah B, et al.
    Trop Biomed, 2012 Sep;29(3):479-88.
    PMID: 23018511
    Strongyloidiasis is an infection caused by the intestinal nematode Strongyloides stercoralis. Infected healthy individuals are usually asymptomatic, however it is potentially fatal in immunocompromised hosts due to its capacity to cause an overwhelming hyperinfection. Strongyloidiasis could be missed during routine screening because of low and intermittent larval output in stool and variable manifestations of the symptoms. We present two cases of strongyloidiasis occurring in children with solid organ malignancies suspected to have the infection based on their clinical conditions and treatment history for cancer. Both patients were diagnosed by molecular and serological tests and were successfully treated. Thus, strongyloidiasis in patients undergoing intensive treatment for malignancies should be suspected, properly investigated and treated accordingly.
    Matched MeSH terms: Hepatoblastoma/complications; Hepatoblastoma/radiography; Hepatoblastoma/surgery
  8. Teh SH, Ong GB
    Med J Malaysia, 2007 Oct;62(4):345-6.
    PMID: 18551945 MyJurnal
    Beckwith-Wiedemann Syndrome (BWS) is associated with early development of embryonal tumours usually in the first four years of life. We describe a patient who presented with a right adrenal cyst in the first month of life and hepatoblastoma in the third month of life. A cavernous haemangioma was subsequently found in the resected tumour.
    Matched MeSH terms: Hepatoblastoma/diagnosis
  9. Abdul Rahman Sazli F, Jubri Z, Abdul Rahman M, Karsani SA, Md Top AG, Wan Ngah WZ
    PMID: 25886747 DOI: 10.1186/s12906-015-0590-y
    To determine the antiproliferative effect of gamma-tocotrienol (GTT) treatment on differential protein expression in HepG2 cells.
    Matched MeSH terms: Hepatoblastoma/drug therapy; Hepatoblastoma/metabolism*
  10. Kaur G, Mutum SS
    Malays J Med Sci, 2001 Jan;8(1):69-72.
    PMID: 22973160
    Hepatoblastoma is the most common primary liver tumour of childhood. This is a case report of a one-year-old boy who presented with a one-month history of progressive abdominal distension and weight loss. He was cachexic, anaemic, had gross hepatomegaly and ascites. He had been born prematurely with a birth weight of 1.23 kg, and his developmental milestones were delayed. Ultrasound and CT scan demonstrated a large solid tumour in the left lobe of the liver with a smaller superficial nodule in the right lobe. Serum alpha fetoprotein was significantly raised. A left lobe hepatectomy and complete excision of the right sided nodule was performed. There was no evidence of metastatic disease. Histopathological examination confirmed hepatoblastoma of the fetal type. The patient developed features of intestinal obstruction a few days after surgery and he succumbed ten days after re-laparotomy. The clinical presentation and investigation results in this case are characteristic. Recent reports have suggested a strong relationship between very low birth weight (< 1500gm)/prematurity and hepatoblastoma as is present in this case. Surgery is the mainstay of therapy in hepatoblastoma. A brief review of the literature on this tumour is presented.
    Matched MeSH terms: Hepatoblastoma
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