Cases Of Hepatoblastoma Research Articles

Development of Novel Indole-Based Covalent Inhibitors of TEAD as Potential Antiliver Cancer Agents.

Abnormal activation of the YAP transcriptional signaling pathway drives proliferation in many hepatocellular carcinoma (HCC) and hepatoblastoma (HB) cases. Current treatment options often face resistance and toxicity, highlighting the need for alternative therapies. This article reports the discovery of a hit compound C-3 from docking-based virtual screening targeting TEAD lipid binding pocket, which inhibited TEAD-mediated transcription. Optimization led to the identification of a potent and covalent inhibitor CV-4-26 that exhibited great antitumor activity in HCC and HB cell lines in vitro, xenografted human HCC, and murine HB in vivo. These outcomes signify the potential of a highly promising therapeutic candidate for addressing a subset of HCC and HB cancers. In the cases of current treatment challenges due to high upregulation of YAP-TEAD activity, these findings offer a targeted alternative for more effective interventions against liver cancer.

A rare case report: multiple intrahepatic masses in a pediatric patient with citrin deficiency

Deficiency of citrin, the liver-type aspartate-glutamate carrier, arises from biallelic mutations of the gene SLC25A13. Although citrin deficiency (CD) is associated with higher risk of hepatocellular carcinoma (HCC) in adult patients, this association remains inconclusive in pediatric cases. The patient in this paper had been diagnosed to have CD by SLC25A13 analysis at the age 10 months, and then in response to dietary therapy, her prolonged jaundice and marked hepatosplenomegaly resolved gradually. However, she was referred to the hospital once again due to recurrent abdominal distention for 2 weeks at her age 4 years and 9 months, when prominently enlarged liver and spleen were palpated, along with a strikingly elevated serum alpha-fetoprotein (AFP) level of 27605 ng/mL as well as a large mass in the right liver lobe and a suspected tumor thrombus within the portal vein on enhanced computed tomography. After 4 rounds of adjuvant chemotherapy, right hepatic lobectomy and portal venous embolectomy were performed at her age 5 years and 3 months, and metastatic hepatoblastoma was confirmed by histopathological analysis. Afterwards, the patient underwent 5 additional cycles of chemotherapy and her condition remained stable for 7 months after surgery. Unfortunately, hepatoblastoma recurred in the left lobe at the age 5 years and 10 months, which progressed rapidly into liver failure, and led to death at the age 6 years and 1 month. As far as we know, this is the the first case of hepatoblastoma in a patient with CD, raising the possibility of an association between these two conditions.

Abstract 3614: T cell receptor-engineered T cell therapy using TCR gene derived from an HLA-A2 hepatoblastoma patient who was completely cured by administration of glypican-3 peptide vaccine

Abstract Glypican-3 (GPC3) is highly expressed in many of hepatocellular carcinoma, hepatoblastoma, ovarian clear cell carcinoma, and squamous cell lung carcinoma, but not expressed in almost all normal tissues, making it an excellent target for cancer immunotherapy. We identified each HLA-A24 and -A2-restricted GPC3-derived peptide and had conducted various clinical trials administering these peptides. All five cases of refractory hepatoblastoma, who had repeated relapses and remissions, are surviving for more than 9 years without recurrence by the only administering these peptide vaccines. We established many kinds of peptide-specific CTL clones with high avidity from PBMC of one of the female HLA-A*02:01-positive refractory hepatoblastoma patient treated with GPC3-derived peptide. Through collaborating research with Takara Bio Inc., which has siTCR technology and extensive experience in developing TCR-T cell therapies including NY-ESO-1 TCR-T, we identified most effective two kinds of TCR by creating a viral vector, preparing TCR-expressing T cells, and evaluating them in various in vitro/in vivo experiments. These TCR-gene engineered T cell can kill cancer cells presenting glypican-3-derived peptides, so this TCR-T therapy has proven to be a promising treatment for patients with cancers that are HLA-A2 positive and express GPC3. The development of TCR-T has been overshadowed by the overwhelming increase in the number of CAR-Ts developed in recent years, but while CAR-Ts have struggled in solid cancers, there have been some reports of successful cases in TCR-Ts and remains a promising treatment. Although GPC3 is a membrane protein, it is not necessarily expressed on the cell membrane and is often expressed within the cell, so we believe that TCR-T is more effective than CAR-T, which targets GPC3. TCR-T cell therapy targeting GPC3 has not yet been implemented anywhere in the world, and we hope this TCR-T therapy as an effective treatment for hepatocellular carcinoma, an intractable cancer with a 10-year survival rate of only 15%, refractory childhood hepatoblastoma, ovarian clear cell carcinoma and lung squamous cell carcinoma that are difficult to respond to anticancer drugs. This development was based on an unmet medical need, and HLA-A2 is found not only in 40% of Japanese, but also in many Caucasians, making it versatile and possible for international expansion. Citation Format: Tsuyoshi Terada, Kazunobu Ohnuki, Manami Shimomura, Toshihiro Suzuki, Yasunori Amaishi, Kaho Takeichi, Sachiko Okamoto, Norihiro Fujinami, Kazumasa Takenouchi, Nobuo Tsukamoto, Kayoko Shoda, Toshiaki Yoshikawa, Tetsuya Nakatsura. T cell receptor-engineered T cell therapy using TCR gene derived from an HLA-A2 hepatoblastoma patient who was completely cured by administration of glypican-3 peptide vaccine [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 3614.

Computed tomography imaging phenotypes of hepatoblastoma identified from radiomics signatures are associated with the efficacy of neoadjuvant chemotherapy

BackgroundThough neoadjuvant chemotherapy has been widely used in the treatment of hepatoblastoma, there still lacks an effective way to predict its effect.ObjectiveTo characterize hepatoblastoma based on radiomics image features and identify radiomics-based lesion phenotypes by unsupervised machine learning, intended to build a classifier to predict the response to neoadjuvant chemotherapy.Materials and methodsIn this retrospective study, we segmented the arterial phase images of 137 cases of pediatric hepatoblastoma and extracted the radiomics features using PyRadiomics. Then unsupervised k-means clustering was applied to cluster the tumors, whose result was verified by t-distributed stochastic neighbor embedding (t-SNE). The least absolute shrinkage and selection operator (LASSO) regression was used for feature selection, and the clusters were visually analyzed by radiologists. The correlations between the clusters, clinical and pathological parameters, and qualitative radiological features were analyzed.ResultsHepatoblastoma was clustered into three phenotypes (homogenous type, heterogenous type, and nodulated type) based on radiomics features. The clustering results had a high correlation with response to neoadjuvant chemotherapy (P=0.02). The epithelial ratio and cystic components in radiological features were also associated with the clusters (P=0.029 and 0.008, respectively).ConclusionsThis radiomics-based cluster system may have the potential to facilitate the precise treatment of hepatoblastoma. In addition, this study further demonstrated the feasibility of using unsupervised machine learning in a disease without a proper imaging classification system.Graphical abstract

Feeding artery characteristics and enhancement patterns of hepatoblastoma patients treated with transarterial chemoembolization (TACE): Digital subtraction angiography evaluation.

Hepatoblastoma is one of the most common primary malignant liver tumors in children. The incidence of hepatoblastoma has been increasing, reaching 1.2 per million children now. The transarterial chemoembolization (TACE) procedure is one of the most practical and effective treatment options besides surgery. Digital subtraction angiography (DSA) is performed as the first step of the TACE procedure. The aim of this study was to provide information about the feeding arteries and enhancement pattern of the hepatoblastoma that was assessed by DSA before the TACE procedure. A retrospective study was conducted among hepatoblastoma cases that had undergone DSA on the TACE procedure to obtain information on the vascularity of the tumor. A total of 26 hepatoblastoma cases who had DSA examination as a part of their first TACE procedure were included, consisting of 15 boys and 11 girls, aged between 1-15 years. All cases were stage III and IV according to the Pre-Treatment Extent of Tumor (PRETEXT) staging classification. All hepatoblastoma cases had multiple feeding arteries, most of which were branches of the right hepatic artery. The largest diameter of the feeding artery was 1.82 mm, and the smallest was 0.63 mm. Most cases (84.62%) had strong contrast absorption, and spread evenly, either at the edges or in the center of the tumor. DSA is believed to be an accurate procedure to provide a detailed description of the feeding artery; enhancement patterns of hepatoblastoma were influenced by an adequate TACE.

ANESTHETIC MANAGEMENT OF LIVER RESECTION IN PEDIATRIC HEPATOBLASTOMA PATIENTS

Background – Children who present for liver resection are a challenge to anesthesiologists. They require adequate preoperative assessment and planning to improve their postoperative outcomes. A balanced technique providing organ protective anesthesia and hemodynamic stability ensures optimal surgical results. Anaesthesia for hep Result: atic resection has seen great changes with improvements in surgical technique and a better understanding of the underlying physiology. Good communication and interdisciplinary strategization between the pediatric oncologist, surgeon and the anesthesiologist also facilitate a good outcome. A retrospective review was performed on 20 cases of pediatric hepatoblastoma planned for partial hepatectomy in a pediatric tertiary care center in South India, over a 3-year period. Conclusion: In this study, general anesthesia with epidural analgesia and goal directed uid management with monitoring of central venous pressure and mean blood pressure during liver resection was found to be acceptable for pediatric liver resection without any signicant complications

Giant pedunculated hepatoblastoma mimicking neuroblastoma in a 4-month-old infant: a case report

BackgroundHepatoblastoma is the most common primary malignancy of hepatic origin in children, with an estimated incidence of 0.5–1.5 per million children. Hepatoblastoma classically has an intraparenchymal location, and pedunculated hepatoblastoma is a relatively rare entity. Accurate diagnosis can be challenging due to its extrahepatic location and possibly its thin peduncle, which is not easily identified in imaging.Case presentationHere, we report a case of asymptomatic giant palpable hepatoblastoma in the LUQ of a four-month-old male infant, initially suspected of neuroblastoma based on abdominal ultrasound findings. The final diagnosis of giant pedunculated hepatoblastoma was made based on the abdominal CT scan and the diagnosis was confirmed by percutaneous biopsy. Due to the size of the tumor, complete removal of the tumor was not initially possible. Therefore, the patient was treated with several courses of chemotherapy. The tumor was shrunk and then completely removed. The patient was treated, and no complications were found in the 6-month follow-up.ConclusionPedunculated hepatoblastoma is rare but should be considered as a possibility in the case of a perihepatic mass in a pediatric patient that can be confused with other upper abdominal masses such as an adrenal mass. Therefore, in such cases, we must look for the vascular pedicle in the imaging and keep the AFP check in mind.

Гепатобластома у детей раннего возраста

Hepatoblastoma is a common primary malignant tumor in children aged 0 to 4 years. In the structure of liver neoplasms, the pathology occupies 80% and has been steadily growing over the past 30 years with an annual increase of 4–5%. Risk factors for the development of hepatoblastoma include an increase in the survival rate of deeply premature babies, oxidative tissue damage associated with intensive therapy of low birth weight premature babies, and IVF. The disease has no specific complaints and characteristic clinical manifestations. The initial manifestations of hepatoblastoma are often hidden under a variety of mask diseases. It is accompanied by the absence of «oncological alertness» of local pediatricians, which determines the late diagnosis of the liver malignant neoplasm. The article presents an analysis of hepatoblastoma cases in young children over a ten-year period. The main initial clinical manifestations of the disease in early children are indicated. The effectiveness of modern methods of diagnosis and therapy of the liver malignant neoplasms in children is shown. A clinical example of successful treatment of this disease is given.

A rare case of non-treated fetal–type hepatoblastoma for five years stayed without metastases in a young boy

Hepatoblastoma is the most common malignant neoplasm of the liver in infancy. In this paper, we present a rare case of non-treated hepatoblastoma in an 18-month-old baby male who presented with an asymptomatic abdominal mass associated with elevated Alpha-fetoprotein. Contrast-enhanced CT revealed a solid mass located in the left lobe of the liver, measuring about 6 × 6 cm, and not enhancing the contrast. US-guided Tru-cut biopsy was performed which showed pure epithelial fetal-type hepatoblastoma. The parents refused neoadjuvant chemotherapy and the connection was disconnected with them because of the ongoing crisis in Syria. After five years, the patient returned with the same clinical picture. CT scan was repeated which revealed the same tumor without metastases. The decision was made to do a left hepatectomy after another confirmation with a surgical biopsy. Histopathological examination showed a multifocal pure epithelial-fetal well-differentiated type of hepatoblastoma with a favorable grade. Moreover, surgical margins and liver capsule appeared free, and the tumor was considered in Stage 1 accordingly. To this date, the boy was followed up with serial US and Alpha-fetoprotein (AFP)levels without signs of recurrence. Hepatoblastoma with pure fetal-type histology can be treated with complete surgical excision without neoadjuvant or adjuvant chemotherapy if the tumor was resected completely because this type of neoplasm is slow growing and has a good prognosis.

Pattern of Different Immunohistochemical Stains in Various Types of Hepatoblastoma

Objective: To assess the utility of four immunohistochemical stains in the categorization of hepatoblastoma.
 Study Design: Retrospective longitudinal study.
 Place and Duration of Study: Histopathology Department, Shaukat Khanum Memorial Cancer Hospital, Peshawar Pakistan, from 2016 to 2018.
 Methodology: We retrospectively reviewed 50 cases of hepatoblastoma from 2016-2018, from our hospital database and studied the pattern of four immunohistochemical stains (Beta-catenin, Glutamine synthetase, Heppar 1 and Cyclin D1) in various subtypes.
 Results: Ten out of fifty cases were mixed epithelial and mesenchymal hepatoblastomas, and forty were pure epithelial. In the pure epithelial category, the fetal subtype showed Heppar 1 and Cyclin D1 positivity in twenty-eight cases, while Glutamine synthetase and Beta-catenin were positive in all the thirty cases. One case of embryonal subtype displayed negativity for Heppar 1; the rest of the immunohistochemical stains were positive. Ten tumours exhibiting mixed epithelial and mesenchymal morphology showed positivity for all the four immunohistochemical stains in the epithelial component. However, Heppar 1 and cyclin D1 were negative in the mesenchymal component in all the ten cases. Five cases (50%) showed Glutamine synthetase and Beta-catenin positivity in the mesenchymal component.
 Conclusion: All the four immunohistochemical stains, especially Beta-catenin and Glutamine synthetase, were efficient diagnostic tool, especially for tumours with complex or vague morphological features.

Клинический случай: гепатобластома печени в нетипичном для гепатобластомы возрасте

Hepatoblastoma is a rare malignant tumor. One case of hepatoblastoma per 1 million of children under the age of 15 is diagnosed annually in the Republic of Kazakhstan (RK), which corresponds to 5–6 cases per year. In 90% of cases the tumor is diagnosed in children under the age of 5 and has two peaks of incidence: the first peak at birth or within the first month of life, and the second peak between the ages of 16 and 18 months. The cases involving patients older than 5 years are extremely rare. The paper reports the case of female patient, who was diagnosed with hepatoblastoma in the age not typical for malignant neoplasms of this type. The review of hepatoblastoma epidemiology, possible risk factors, clinical manifestations, diagnosis and treatment methods is provided in this issue.

Role of Diffusion Weighted MRI (DW-MR) in Detection of Satellite Lesions Not Detected with Multiphase CT Scans in Hepatoblastoma and Its Implications for Management.

To evaluate the role of diffusion-weighted magnetic resonance imaging (DW-MR) in hepatoblastoma as compared to multiphase contrast enhanced computed tomography (CECT) scans for detection of satellite lesions. In this prospective study onnew cases of hepatoblastoma, multiphase CECT scans and DW-MR were performed before initiation of chemotherapy. Results of interpretation were compared for detection of satellite lesions. PRETEXT grouping and risk categorization was done according to SIOPEL based on CECT scan. Nine boys and 1 girl with hepatoblastoma, with median age of 11.5 mo were included. All patients were stratified as high-risk group with 2 (20%) in PRETEXT II and 8 (80%) in PRETEXT III. In 2 of 10 (20%) patients, additional satellite lesions were detected on DW-MR, which upgraded their stage. One of the two patients had one satellite lesion identified on CECT, while additional seven satellite lesions were identified on DW-MR imaging. For the other patient, CECT showed no satellite lesion while DW-MRdetected six satellite lesions. MRI has become the gold standard investigation for evaluation of hepatoblastoma. DW-MR, which is a contrast free technique, is a better tool for assessment of satellite lesions which are usually missed on CECT. Thiswould help in proper staging, planning of management and prognostication.

The utility of core-needle tumor biopsy for pediatric patients.

Core-needle biopsy (CNB) is used less frequently for the diagnosis of tumors in pediatric patients. In this report, the utility and safety of CNB for pediatric patients are described. The medical records of patients who underwent CNB at the Department of Pediatric Surgery, Kyushu University Hospital from April 2020 to November 2021 were retrospectively reviewed. A 14 G or 16 G BARDMISSION Disposable Needle Instrument was used. For the diagnosis of neuroblastoma, a 14 G needle was selected; for the diagnosis of other tumors a 16 G needle was selected. During the above period 17 CNBs were performed in 17 patients, and the median patient age was 8 years (range, 15 days-19 years). The pathological diagnoses of the tumors were as follows: neuroblastoma, n = 6; lymphoma, n = 3; hepatoblastoma, n = 2; and others, n = 6. The quantity and quality of all tumor samples obtained by CNB was sufficient to make a diagnosis. The postoperative course after CNB was uneventful in most cases, with the exception of one case of hepatoblastoma (pseudoaneurysm). Core-needle biopsy is useful for pediatric patients. Sufficient tumor specimens were able to be obtained in all cases, irrespective of the type of tumor, and an accurate diagnosis could be made.

Hepatoblastoma in Alagille Syndrome

Background and Aim: Alagille syndrome (AGS) is a rare multisystem genetic disorder of autosomal dominant inheritance classically characterized by intrahepatic bile duct paucity in conjunction with various combinations of extrahepatic manifestations and characteristic facial features. Apart from various hepatic manifestations these patients are also at risk of developing a wide variety of liver tumours including nodular hyperplasia of the liver, adenomas and hepatocellular carcinomas. Although there are reports of numerous cases of hepatocellular carcinoma among children with AGS but there is a dearth of cases of hepatoblastoma in young children.

Sialoblastoma and Hepatoblastoma in an Infant: A Case Report and Review of the Literature

: Sialoblastoma is a rare salivary gland tumor, diagnosed in the neonatal period or during infancy. Hepatoblastoma accounts for less than 1% of all pediatric malignancies. The co-occurrence of these two tumors is extremely uncommon. Here, we report a case of concurrent sialoblastoma and hepatoblastoma in an infant.

Computed tomography-guided marking using a dye-staining method for preoperative localization of tiny pulmonary lesions in children.

It is important to confirm the existence of pulmonary metastases in pediatric patients with malignancies. Therefore, we aimed to investigate if computed tomography CT-guided marking is a feasible and safe method for the identification and resection of tiny pulmonary lesions in pediatric cancer patients. We retrospectively reviewed the medical records of pediatric cancer patients who underwent CT-guided marking procedures in our institutions between Jan 2011 and Apr 2020. After 2015, these procedures were combined with an indocyanine green (ICG) navigation-guided surgery for hepatoblastoma cases. We targeted a total of 22 nodules in 12 patients. Of these, marking was successful in 18 (81.8%) nodules, 10 of which contained viable malignant cells. Complications caused by the marking procedures included mild pneumothorax and mild atelectasis in two patients, respectively. Of the eight resected nodules in patients with hepatoblastoma, four were ICG-positive and contained viable malignant cells. Two additional ICG-positive nodules, which were unidentified before surgery, were observed intraoperatively. CT-guided marking is a feasible and safe method that can be used to identify and resect tiny pulmonary lesions in pediatric cancer patients. An ICG navigation-guided surgery is useful when combined with CT-guided marking, particularly in hepatoblastoma cases.

A case of hepatoblastoma that was difficult to distinguish from hepatocellular carcinoma

A case of hepatoblastoma that was difficult to distinguish from hepatocellular carcinoma

Therapeutic effect and clinical cost of multi-disciplinary team model of hepatoblastoma in children

Objective To summarize and analyze the treatment process, long-term efficacy and clinical economics of children's hepatoblastoma (HB) in multi-disciplinary team (MDT) mode, so as to provide basis for the rational choice of diagnosis and treatment. Methods From January 2014 to February 2019, 13 cases of hepatoblastoma in children who completed the whole treatment course in the Pediatric Hematology Tumor Ward of Peking University First Hospital were collected and analyzed, and were followed up until June 30, 2020. There were 9 males and 4 females who were diagnosed and treated according to the MDT process in the hospital. The median age was 16 months (2-54 months), 69.23% (9/13) were under 2 years old. The characteristics, diagnosis and treatment process and treatment effect of the cases were summarized, and the cost of clinical treatment was analyzed. Results According to the pretreatment extent of disease(PRETEXT), there were 1, 9 and 3 children with stages Ⅱ, Ⅲ and Ⅳ. 76.92% (10/13) of the patients had the largest tumor diameter > 10 cm. All the patients received preoperative neoadjuvant chemotherapy (8 patients received 4 cycles of chemotherapy, and 6 patients changed the chemotherapy plan), surgical treatment and postoperative chemotherapy, the tumor volume decreased by more than 50% (64%-95%) in 12 cases, except 1 case with no significant increase of alpha fetal protein and multiple lesions.The median length of stay was 87 days (68-214 days), the median cost of stay was 200 000 yuan (115 000-500 000 yuan), the median length of stay was 7 days (5-17 days), the median cost of stay was 20 000 yuan (15 000-60 000 yuan), and the incidence of postoperative complications was 7.69% (1/13). All the patients were followed up for 16-69 months. All the patients survived. Conclusion Under the MDT mode, the treatment is seamless connection, the long-term prognosis of children with HB is good, and the total hospitalization cost and time are within the acceptable range. Standard preoperative neoadjuvant chemotherapy can significantly reduce the tumor, improve the resection rate, reduce postoperative complications, reduce the total individual expenditure, shorten the total hospital stay, and further improve the long-term disease-free survival rate.

Yes-associated protein-1 may serve as a diagnostic marker and therapeutic target for residual/recurrent hepatocellular carcinoma post-transarterial chemoembolization

Background and aimThe transcriptional co-activator Yes-associated protein-1 (YAP1) has been implicated as an oncogene and is overexpressed in different kinds of human cancers, especially hepatocellular carcinoma (HCC). However, the role of YAP1 has not been reported in residual/recurrent HCC after transarterial chemoembolization (TACE). Our aim is to determine whether YAP1 is overexpressed in the residual/recurrent HCC after TACE. MethodsA total of 105 tumor tissues from 71 patients including 30 cases of primary HCC without prior treatment, 35 cases of residual/recurrent HCC post TACE, and 6 cases of hepatoblastoma were included in the immunohistochemical study. YAP1 immunoreactivity was blindly scored as 0, 1+, 2+ or 3+ in density and percentages of positive cells. ResultsAbout 33.3% (10/30) of primary HCC without prior treatment showed 2+ of YAP1 immunoreactivity. While 82.8% (29/35) of residual/recurrent HCCs after TACE treatment displayed 2–3+ of YAP1 immunoreactivity, which was significantly higher compared to primary HCC without prior treatment (P = 0.0002). YAP1 immunoreactivity was moderately to strongly positive (2–3+) in 100% of the hepatoblastoma, particularly in the embryonal components (3+ in 100% cases). ConclusionsYAP1 is significantly upregulated in the residual/recurrent HCCs post TACE treatment, suggesting that YAP1 may serve as a sensitive diagnostic marker and a treatment target for residual/recurrent HCC post TACE.

Hepatoblastoma With Rubinstein-Taybi Syndrome: A Rare Association

Abstract Introduction/Objective Hepatoblastoma is a malignant neoplasm of liver, exclusively seen in pediatric population. It has been known to occur with various syndromes like Li-fraumeni, Beckwith-Wiedemann, and Simpson-Golabi- Behmel among others. Small undifferentiated cells and macro-trabecular pattern with increased mitotic activity and AFP>100 are associated with unfavorable prognosis. Rubinstein-Taybi syndrome is a rare genetic disease. Even though there has been intensive research on the genetic and epigenetic function of the CREBBP and EP300 genes in animal models, the etiology of this rare and devastating syndrome is largely unknown. Hepatoblastoma with Rubinstein-Taybi syndrome is an extremely rare occurrence. Methods Here we present an incidental case of Hepatoblastoma with Rubinstein-Taybi syndrome Results A 17-month-old Caucasian female with Rubinstein-Taybi syndrome, was found to have an incidental 1.1 cm nodule in the right lobe of liver. Her AFP was elevated (244.9ng/ml). Hepatic resection revealed a well-circumscribed tan white mass, measuring 3.5 cm in the greatest dimension. On microscopy, uniform round to cuboidal cells resembling fetal hepatocytes, with finely granular cytoplasm, small round nuclei, and indistinct nucleoli were seen. The cells were arranged in solid sheets and scant trabecular arrangement separated by narrow sinusoids. The tumor displayed predominantly fetal histology, with rare embryonal and small undifferentiated cell nests. The diagnosis of Hepatoblastoma was made. The patient underwent chemotherapy and was doing well at 5-years follow-up. Conclusion Extensive screening of patients in recent times has revealed mutations in CREBBP and EP300 in only around 50% of cases. The genetic and epigenetic associations of the syndrome in the remaining half of cases remains to be identified. Hepatoblastoma with Rubinstein-Taybi Syndrome is an extremely rare occurrence and might shed some light on other genes that could be involved in this syndrome. Further studies to review the mutation spectrum to understand the causative molecular mechanisms are deemed essential.