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1
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Rahiman EA, Bhat V, Kumar N, Mathai SS. Simpson-Golabi-Behmel syndrome type 1 in a neonate with central hepatoblastoma. BMJ Case Rep 2025; 18:e262331. [PMID: 39880477 PMCID: PMC11780580 DOI: 10.1136/bcr-2024-262331] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/13/2024] [Accepted: 01/23/2025] [Indexed: 01/31/2025] Open
Abstract
We report a neonate evaluated for hepatomegaly during hospitalisation and was diagnosed to have hepatoblastoma, an uncommon childhood malignancy. The presence of dysmorphism, macrosomia and congenital heart defect led to the suspicion of congenital overgrowth conditions. The genetic evaluation revealed a pathogenic variant, conclusive of Simpson-Golabi-Behmel syndrome type 1 (SGBS1). The child was treated with neoadjuvant chemotherapy followed by central hepatectomy and is currently in remission. We discuss the predisposing syndromes for hepatoblastoma, as the neonatal onset of malignancy in the setting of dysmorphism and congenital anomalies led us to the molecular diagnosis. Children with multisystem involvement require interdisciplinary care, which is detailed. The role of neoadjuvant chemotherapy in facilitating extensive liver resection and the use of technological advancement like intraoperative imaging that can aid definitive resection is discussed. Long-term follow-up for surveillance of primary cancers is crucial in the comprehensive care of cancer-predisposing conditions, the recommendations of which are listed.
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MESH Headings
- Humans
- Hepatoblastoma/therapy
- Hepatoblastoma/diagnosis
- Hepatoblastoma/complications
- Hepatoblastoma/genetics
- Liver Neoplasms/therapy
- Liver Neoplasms/diagnosis
- Liver Neoplasms/complications
- Infant, Newborn
- Gigantism/diagnosis
- Gigantism/genetics
- Gigantism/complications
- Heart Defects, Congenital/diagnosis
- Heart Defects, Congenital/genetics
- Heart Defects, Congenital/complications
- Genetic Diseases, X-Linked/diagnosis
- Genetic Diseases, X-Linked/genetics
- Genetic Diseases, X-Linked/complications
- Intellectual Disability/diagnosis
- Intellectual Disability/genetics
- Intellectual Disability/complications
- Arrhythmias, Cardiac/diagnosis
- Arrhythmias, Cardiac/genetics
- Arrhythmias, Cardiac/complications
- Hepatectomy
- Male
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Affiliation(s)
- Emine A Rahiman
- Pediatric Oncology, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India
| | - Vasudev Bhat
- Pediatric Oncology, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India
| | - Naveena Kumar
- Surgical Oncology, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India
| | - Sheila Samanta Mathai
- Division of Neonatology, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India
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2
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Fuchs J, Rabaux-Eygasier L, Ruping F, Kessler M, Günther P, Hoffmann K, Czigany Z, Michalski C, Hery G, Mehrabi A, Branchereau S. Reappraisal of liver resection as an alternative to transplantation in locally advanced hepatoblastoma: A systematic review and analysis of pooled individual patient data. Pediatr Blood Cancer 2024; 71:e31339. [PMID: 39334537 DOI: 10.1002/pbc.31339] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/22/2024] [Revised: 08/08/2024] [Accepted: 09/06/2024] [Indexed: 09/30/2024]
Abstract
BACKGROUND There is ongoing debate regarding liver transplantation (LT) versus liver resection (LR) for locally advanced hepatoblastoma. However, comparative studies are lacking. Consequently, a significant evidence gap persists, hindering the establishment of consensus guidelines. This study aimed to compare LT and LR for locally advanced hepatoblastoma, using predefined inclusion criteria to ensure comparable intervention groups. METHODS According to current Children's Oncology Group (COG) and SIOPEL (European Childhood Liver Tumour Study Group) recommendations, hepatoblastoma that requires LT evaluation was defined as either PRETEXT (PRE-Treatment EXTent of tumor) IV F+, POST-TEXT (POST-Treatment EXTent of tumor) IV, POST-TEXT P+, and/or POST-TEXT V+. A systematic literature search (Medline/Web-of-Science/Embase) was performed. Only patients who met the aforementioned criteria were included. Patient data were extracted individually and pooled. RESULTS A total of 189 patients with locally advanced hepatoblastoma from 55 studies met the specified criteria, with 111 undergoing LT and 78 LR. There were no significant differences between the two groups in age, alpha-fetoprotein (AFP), and PRETEXT stages. Local recurrence was more common after LR (14% vs. 3% in LT, p = .008), while distant recurrence was more often observed after LT (16% vs. 5% in LR, p = .035). Overall survival (OS) and event-free survival (EFS) did not differ significantly between LT and LR (5-year OS: LT = 75.3% [95% confidence interval: 66.5-85.2], LR = 87.6% [80.4-95.6], p = .140; 5-year EFS: LT = 68.5% [59.3-79.1], LR = 71.1% [60.7-83.3], p = .700). CONCLUSION Real-life data revealed that a considerable number of patients with locally advanced hepatoblastoma underwent LR. This analysis suggests that outcomes are similar and favorable for both approaches. LR can therefore be considered an effective alternative to LT in selected cases even in locally advanced hepatoblastoma.
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Affiliation(s)
- Juri Fuchs
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
- Department of Pediatric Surgery, Hôpital Kremlin-Bicêtre, APHP, University of Paris-Saclay, Paris, France
| | - Lucas Rabaux-Eygasier
- Department of Pediatric Surgery, Hôpital Kremlin-Bicêtre, APHP, University of Paris-Saclay, Paris, France
| | - Fabian Ruping
- Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Markus Kessler
- Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Patrick Günther
- Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | | | - Zoltan Czigany
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Christoph Michalski
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Geraldine Hery
- Department of Pediatric Surgery, Hôpital Kremlin-Bicêtre, APHP, University of Paris-Saclay, Paris, France
| | - Arianeb Mehrabi
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Sophie Branchereau
- Department of Pediatric Surgery, Hôpital Kremlin-Bicêtre, APHP, University of Paris-Saclay, Paris, France
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Akhaladze DG, Rabaev GS, Tverdov IV, Merkulov NN, Uskova NG, Talypov SR, Krivonosov AA, Grachev NS. Central Liver Segments Resections vs Extended Hepatectomies in Children: Single-Center Experience. J Pediatr Surg 2024; 59:161927. [PMID: 39368854 DOI: 10.1016/j.jpedsurg.2024.161927] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/13/2024] [Revised: 08/26/2024] [Accepted: 09/07/2024] [Indexed: 10/07/2024]
Abstract
BACKGROUND Central liver segments resection (CLSR) still is not widely used in pediatric surgery due to its technical difficulty, whereas this procedure is widely spread as a parenchyma sparing approach of centrally located liver tumors in adults. The aim of this study is to analyze the outcomes of CLSR in comparison with extended hepatectomy (EH) in children with different liver tumors. METHODS A single-center retrospective analysis of patients who received CLSR (n = 14) and EH (n = 44) from June 2017 to December 2023 was applied. Patient's characteristics, preoperative, intra- and postoperative data were compared between 2 groups. RESULTS Preoperative CT-volumetry showed that future liver remnant volume was higher in CLSR group compared to EH (FLR-V; (54 ± 29 (40-91) % vs 40 ± 12 (17-73) %, p = 0.016). The intraoperative blood loss (200 [90-1150] (20-3000) ml vs 100 [30-275] (10-9000) ml, p = 0.088) and transfusion volume (310 [85-590] (0-1860) ml vs 150 [0-310] (0-4770) ml, p = 0.484) were similar in both groups, while operation time was longer in CLSR group (420 [320-595] (145-785) min vs 280 [203-390] (125-710) min), p = 0.011). There was no difference in biliary leakage (3 (21.4 %) vs 12 (27.3 %); p = 0.479), other complications (4 (28.6 %) vs 5 (11.4 %), p = 0.198) and complications ≥ IIIb by Clavien-Dindo (2 (14.3 %) vs 8 (18.2 %), p = 0.385) postoperatively. CONCLUSION CLSRs allow to preserve more healthy liver parenchyma compared to EH with similar intraoperative and postoperative outcomes. «Extended mesohepatectomy» allows to achieve R0 resection when central liver tumor extends on the left lateral and/or right posterior section. TYPE OF STUDY Retrospective Comparative Study (Level of Evidence III).
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Affiliation(s)
- Dmitry G Akhaladze
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology Ministry of Health of Russian Federation, 117997, Moscow, 1 Samory Mashela Str., Russian Federation
| | - Gavriil S Rabaev
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology Ministry of Health of Russian Federation, 117997, Moscow, 1 Samory Mashela Str., Russian Federation; University Medical Centre Corporate Fund, National Research Center for Maternal and Child Health, 010000, Astana, 32 Turan Str., Kazakhstan.
| | - Ivan V Tverdov
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology Ministry of Health of Russian Federation, 117997, Moscow, 1 Samory Mashela Str., Russian Federation
| | - Nikolay N Merkulov
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology Ministry of Health of Russian Federation, 117997, Moscow, 1 Samory Mashela Str., Russian Federation
| | - Natalia G Uskova
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology Ministry of Health of Russian Federation, 117997, Moscow, 1 Samory Mashela Str., Russian Federation
| | - Sergey R Talypov
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology Ministry of Health of Russian Federation, 117997, Moscow, 1 Samory Mashela Str., Russian Federation
| | - Anatoliy A Krivonosov
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology Ministry of Health of Russian Federation, 117997, Moscow, 1 Samory Mashela Str., Russian Federation
| | - Nikolay S Grachev
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology Ministry of Health of Russian Federation, 117997, Moscow, 1 Samory Mashela Str., Russian Federation
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4
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Gigola F, Morini F, Libro G, Morabito A, Grimaldi C. Congenital hepatoblastoma: Expanding knowledge, improving outcomes. Pediatr Blood Cancer 2024; 71:e31132. [PMID: 38952263 DOI: 10.1002/pbc.31132] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/11/2023] [Revised: 04/13/2024] [Accepted: 05/19/2024] [Indexed: 07/03/2024]
Abstract
Hepatoblastoma (HB) is a rare liver tumour, and its congenital counterpart (CHB) is even less frequent. CHB has a clinically challenging management and a generally perceived worse outcome. This study aims to review the literature on CHB to better define presentation, diagnosis, available treatments and management options. The analysis of outcomes suggests that a significant portion of mortality is unrelated to the malignant nature of the tumour. Key factors influencing overall outcomes were identified: mortality linked to the 'mass effect' during both the prenatal (22%) and perinatal (32%) stages, as well as 'oncological' mortality encompassing tumour and/or treatment-related factors (46%). Overall, after birth, CHB does not seem to confer a worse oncological prognosis per se, and should be managed similarly to older children, if patients are stable enough to undergo proper staging and treatment. A deeper knowledge and better outcomes would come from a large, homogeneous, collection of data possibly allowing a global protocol, focusing on a comprehensive management of CHB.
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Affiliation(s)
- Francesca Gigola
- School of Pediatric Surgery, University of Florence, Florence, Italy
- Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy
| | - Francesco Morini
- Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy
- Department of Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy
| | - Giorgia Libro
- School of Pediatric Surgery, University of Florence, Florence, Italy
- Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy
| | - Antonino Morabito
- Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy
- Department of Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy
| | - Chiara Grimaldi
- Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy
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Xiu WL, Liu J, Zhang JL, Wang JM, Wang XF, Wang FF, Mi J, Hao XW, Xia N, Dong Q. Computer-assisted three-dimensional individualized extreme liver resection for hepatoblastoma in proximity to the major liver vasculature. World J Gastrointest Surg 2024; 16:1066-1077. [PMID: 38690040 PMCID: PMC11056661 DOI: 10.4240/wjgs.v16.i4.1066] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2023] [Revised: 01/29/2024] [Accepted: 03/15/2024] [Indexed: 04/22/2024] Open
Abstract
BACKGROUND The management of hepatoblastoma (HB) becomes challenging when the tumor remains in close proximity to the major liver vasculature (PMV) even after a full course of neoadjuvant chemotherapy (NAC). In such cases, extreme liver resection can be considered a potential option. AIM To explore whether computer-assisted three-dimensional individualized extreme liver resection is safe and feasible for children with HB who still have PMV after a full course of NAC. METHODS We retrospectively collected data from children with HB who underwent surgical resection at our center from June 2013 to June 2023. We then analyzed the detailed clinical and three-dimensional characteristics of children with HB who still had PMV after a full course of NAC. RESULTS Sixty-seven children diagnosed with HB underwent surgical resection. The age at diagnosis was 21.4 ± 18.8 months, and 40 boys and 27 girls were included. Fifty-nine (88.1%) patients had a single tumor, 39 (58.2%) of which was located in the right lobe of the liver. A total of 47 patients (70.1%) had PRE-TEXT III or IV. Thirty-nine patients (58.2%) underwent delayed resection. After a full course of NAC, 16 patients still had close PMV (within 1 cm in two patients, touching in 11 patients, compressing in four patients, and showing tumor thrombus in three patients). There were 6 patients of tumors in the middle lobe of the liver, and four of those patients exhibited liver anatomy variations. These 16 children underwent extreme liver resection after comprehensive preoperative evaluation. Intraoperative procedures were performed according to the preoperative plan, and the operations were successfully performed. Currently, the 3-year event-free survival of 67 children with HB is 88%. Among the 16 children who underwent extreme liver resection, three experienced recurrence, and one died due to multiple metastases. CONCLUSION Extreme liver resection for HB that is still in close PMV after a full course of NAC is both safe and feasible. This approach not only reduces the necessity for liver transplantation but also results in a favorable prognosis. Individualized three-dimensional surgical planning is beneficial for accurate and complete resection of HB, particularly for assessing vascular involvement, remnant liver volume and anatomical variations.
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Affiliation(s)
- Wen-Li Xiu
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Jie Liu
- Department of Pediatric Surgery, Yijishan Hospital of Wannan Medical College, Wuhu 241000, Anhui Province, China
| | - Jing-Li Zhang
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Jing-Miao Wang
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Xue-Feng Wang
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Fei-Fei Wang
- Shandong Provincial Key Laboratory of Digital Medicine and Computer-assisted Surgery, Shandong College Collaborative Innovation Center of Digital Medicine Clinical Treatment and Nutrition Health, Qingdao University, Qingdao 266003, Shandong Province, China
| | - Jie Mi
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Xi-Wei Hao
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Nan Xia
- Shandong Provincial Key Laboratory of Digital Medicine and Computer-assisted Surgery, Shandong College Collaborative Innovation Center of Digital Medicine Clinical Treatment and Nutrition Health, Qingdao University, Qingdao 266003, Shandong Province, China
| | - Qian Dong
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
- Shandong Provincial Key Laboratory of Digital Medicine and Computer-assisted Surgery, Shandong College Collaborative Innovation Center of Digital Medicine Clinical Treatment and Nutrition Health, Qingdao University, Qingdao 266003, Shandong Province, China
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Gong XH, Feng MX, Zhuang ZG, Yan YQ, Wang L, Ren HN, Zhu Y, Song Y, Qian LJ, Xu JR, Zhou Y, Xia Q. Pediatric Hepatoblastoma After Neoadjuvant Chemotherapy: Diagnostic Performance of MR in Staging POSTTEXT and Vascular Involvement. J Magn Reson Imaging 2024; 59:1034-1042. [PMID: 37272790 DOI: 10.1002/jmri.28848] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2023] [Revised: 05/22/2023] [Accepted: 05/22/2023] [Indexed: 06/06/2023] Open
Abstract
BACKGROUND The assessment of resectability after neoadjuvant chemotherapy of hepatoblastoma is dependent on Post-Treatment EXTENT of Disease (POSTTEXT) staging and its annotation factors P (portal venous involvement) and V (hepatic venous/inferior vena cava [IVC] involvement), but MR performance in assessing them remains unclear. PURPOSE To assess the diagnostic performance of contrast-enhanced MR imaging for preoperative POSTTEXT staging and diagnosing vascular involvement in terms of annotation factors P and V in pediatric hepatoblastoma following neoadjuvant chemotherapy. STUDY TYPE Retrospective. SUBJECTS Thirty-five consecutive patients (17 males, median age, 24 months; age range, 6-98 months) with proven hepatoblastoma underwent preoperative MR imaging following neoadjuvant chemotherapy. FIELD STRENGTH/SEQUENCE 3.0 T; T2-weighted imaging (T2WI), T2WI with fat suppression, diffusion weighted imaging, radial stack-of-the-star/Cartesian 3D Dixon T1-weighted gradient echo imaging. ASSESSMENT Three radiologists independently assessed the POSTTEXT stages and annotation factors P and V based on the 2017 PRE/POSTTEXT system. The sensitivities and specificities were calculated for 1) diagnosing each POSTTEXT stage; 2) discrimination of stages III and IV (advanced) from those stages I and II (non-advanced) hepatoblastomas; and 3) annotation factors P and V. The combination of pathologic findings and surgical records served as the reference standard. STATISTICAL TESTS Sensitivity, specificity, Fleiss kappa test. RESULTS The sensitivity and specificity ranges for discriminating advanced from non-advanced hepatoblastomas were 73.3%-80.0% and 80.0%-90.0%, respectively. For annotation factor P, they were 66.7%-100.0% and 90.6%, respectively. For factor V, they were 75.0% and 67.7%-83.9%, respectively. There was excellent, substantial, and moderate agreement on POSTTEXT staging (Fleiss kappa = 0.82), factors P (Fleiss kappa = 0.64), and factors V (Fleiss kappa = 0.60), respectively. DATA CONCLUSION MR POSTTEXT provides reliable discrimination between advanced and non-advanced tumors, and MR has moderate to excellent specificity at identifying portal venous and hepatic venous/IVC involvement. EVIDENCE LEVEL 3 TECHNICAL EFFICACY: Stage 3.
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Affiliation(s)
- Xu Hua Gong
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Ming Xuan Feng
- Liver Surgery, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Zhi Guo Zhuang
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Yun Qi Yan
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Li Wang
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Hai Nan Ren
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Yi Zhu
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Yang Song
- MR Scientific Marketing, Siemens Healthineers Co Ltd, Shanghai, China
| | - Li Jun Qian
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Jian Rong Xu
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Yan Zhou
- Radiology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
| | - Qiang Xia
- Liver Surgery, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
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Abstract
Hepatoblastoma (HB) remains the most common paediatric liver tumour and survival in children with hepatoblastoma has improved considerably since the advent of sequential surgical regimens of chemotherapy based on platinum-based chemotherapeutic agents in the 1980s. With the advent of modern diagnostic imaging and pathology techniques, new preoperative chemotherapy regimens and the maturation of surgical techniques, new diagnostic and treatment options for patients with hepatoblastoma have emerged and international collaborations are investigating the latest diagnostic approaches, chemotherapy drug combinations and surgical strategies. Diagnosis of hepatoblastoma relies on imaging studies (such as ultrasound, computed tomography, and magnetic resonance imaging), alpha-fetoprotein (AFP) levels, and histological confirmation through biopsy. The standard treatment approach involves a multimodal strategy with neoadjuvant chemotherapy followed by surgical resection. In cases where complete resection is not feasible or tumors exhibit invasive characteristics, liver transplantation is considered. The management of metastatic and recurrent hepatoblastoma poses significant challenges, and ongoing research focuses on developing targeted therapies and exploring the potential of immunotherapy. Further studies are necessary to gain a better understanding of the etiology of hepatoblastoma, develop prevention strategies, and personalize treatment approaches. We aim to review the current status of diagnosis and treatment of hepatoblastoma.
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Affiliation(s)
- Yinbiao Cao
- Faculty of Hepato-Pancreato-Biliary Surgery, Chinese PLA General Hospital, Beijing, China
- The First Medical Center of the Chinese PLA General Hospital, Beijing, China
| | - Shurui Wu
- The First Medical Center of the Chinese PLA General Hospital, Beijing, China
| | - Haowen Tang
- Faculty of Hepato-Pancreato-Biliary Surgery, Chinese PLA General Hospital, Beijing, China
- The First Medical Center of the Chinese PLA General Hospital, Beijing, China
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Zheng C, Ye S, Liu W, Diao M, Li L. Prognostic value of systemic inflammation response index in hepatoblastoma patients receiving preoperative neoadjuvant chemotherapy. Front Oncol 2023; 13:1276175. [PMID: 37901310 PMCID: PMC10613067 DOI: 10.3389/fonc.2023.1276175] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2023] [Accepted: 10/02/2023] [Indexed: 10/31/2023] Open
Abstract
Introduction Inflammation is closely associated with tumor development and patient prognosis. The objective of this study is to assess the prognostic value of the preoperative inflammatory indexes in pediatric hepatoblastoma patients who receive neoadjuvant chemotherapy. Methods A retrospective analysis was performed on clinical and pathological data of 199 hepatoblastoma patients who underwent hepatectomy with preoperative neoadjuvant chemotherapy from January 2015 to June 2020. The receiver operating characteristic curve was used to evaluate the prognostic value of neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), systemic immune-inflammation index (SII), and systemic inflammation response index (SIRI) in predicting OS and EFS. Patients were grouped based on optimal cutoff values of preoperative inflammatory indexes. Survival rates were calculated using the Kaplan-Meier method, and survival outcomes were compared between groups using the log-rank test. Univariate and multivariate Cox proportional hazards regression models were used to identify independent prognostic factors, and a nomogram was constructed using R software to predict the probability of OS. Results The receiver operating characteristic curve showed prognostic value for OS, not EFS, in preoperative inflammatory indexes. Patients were categorized into low/high groups: SII ≤ 266.70/higher, NLR ≤ 1.24/higher, PLR ≤ 85.25/higher, and SIRI ≤ 0.72/higher. High NLR, PLR, SII, and SIRI groups had significantly lower 5-year OS than their low counterparts (all p-value < 0.05). The Cox analysis identified four independent prognostic factors: SIRI (HR=2.997, 95% CI: 1.119-8.031), microvascular invasion (HR=2.556, 95% CI: 1.14-5.73), the post-treatment extent of disease (POSTTEXT) staging (IV vs. I: HR=244.204, 95% CI:11.306-5274.556), and alpha-fetoprotein (>100 ng/ml: HR=0.11, 95% CI: 0.032-0.381) for hepatoblastoma patients with neoadjuvant chemotherapy. High SIRI group had more patients with adverse NLR, SII, and POSTTEXT III (all p-value < 0.05). Independent prognostic factors led to an OS nomogram with a concordance index of 0.85 (95% CI: 0.78-0.91, p-value = 1.43e-27) and the calibration curve showed a good fit between the prediction curve and the true curve. Conclusions SIRI is an independent prognostic factor of hepatoblastoma patients receiving neoadjuvant chemotherapy. The OS nomogram based on SIRI, POSTTEXT staging, MiVI, and AFP can be used to assess the prognosis of those patients.
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Affiliation(s)
- Chen Zheng
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment, Chinese Academy of Medical Sciences, Beijing, China
| | - Shiru Ye
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
| | - Wei Liu
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment, Chinese Academy of Medical Sciences, Beijing, China
| | - Mei Diao
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment, Chinese Academy of Medical Sciences, Beijing, China
| | - Long Li
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment, Chinese Academy of Medical Sciences, Beijing, China
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9
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Honda M, Uchida K, Irie T, Hirukawa K, Kadohisa M, Shimata K, Isono K, Shimojima N, Sugawara Y, Hibi T. Recent advances in surgical strategies and liver transplantation for hepatoblastoma. Cancer Med 2023; 12:3909-3918. [PMID: 36394165 PMCID: PMC9972171 DOI: 10.1002/cam4.5300] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2022] [Revised: 08/22/2022] [Accepted: 09/15/2022] [Indexed: 11/18/2022] Open
Abstract
Hepatoblastoma (HB) is the most common malignant liver tumor in children. Although the development of treatment strategies with advances in chemotherapy has greatly improved the prognosis of HB, surgical resection and liver transplantation still play a vital role in the treatment of HB. In recent years, technological innovations have led to the development of new surgical approaches for HB. In this review, we describe the latest research on the surgical management of HB, including new imaging technologies, minimally invasive approaches, and the application of associating liver partition portal vein ligation for staged hepatectomy. We also discuss the current role of liver transplantation, use of ante-situm or ex-situ liver resection with auto-transplantation, and management of metastatic HB.
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Affiliation(s)
- Masaki Honda
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Koushi Uchida
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Tomoaki Irie
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Kazuya Hirukawa
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Masashi Kadohisa
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Keita Shimata
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Kaori Isono
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Naoki Shimojima
- Department of Surgery, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan
| | - Yasuhiko Sugawara
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Taizo Hibi
- Department of Pediatric Surgery and Transplantation, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
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10
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Wu X, Wang J, Duan Y, Liu Y, Liu Y, Chen X, Xia N, Dong Q. Surgical resection of pediatric PRETEXT III and IV hepatoblastoma: A retrospective study investigating the need for preoperative chemotherapy. Front Pediatr 2022; 10:878095. [PMID: 36533225 PMCID: PMC9751315 DOI: 10.3389/fped.2022.878095] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/17/2022] [Accepted: 11/18/2022] [Indexed: 12/03/2022] Open
Abstract
OBJECTIVE This study analyzed the feasibility of upfront surgical resection for pediatric PRETEXT III and IV hepatoblastoma (HB). SUMMARY BACKGROUND DATA Neoadjuvant chemotherapy is recommended for patients with PRETEXT III and IV HB to obtain a chance of curative surgery. However, chemotherapy can cause toxic side effects and adverse outcomes, and the PRETEXT staging system may overstage the patients. Therefore, whether preoperative chemotherapy is necessary for HB patients remains unclear. METHODS The clinical data of 37 children who underwent surgical resection for PRETEXT III and IV HB at our hospital were obtained retrospectively. Patients were divided into the neoadjuvant chemotherapy group (NCG; n = 19) and the routine surgery group (RSG; n = 18). Clinicopathologic characteristics, treatment regimens, and outcomes were compared between the groups. RESULTS The RSG had a lower incidence of portal vein involvement than the NCG (p < 0.002). The estimated 3-year event-free survival rates were similar (RSG: 89 ± 0.7% and NCG: 79 ± 0.9%, p = 0.3923). The RSG underwent fewer courses of chemotherapy than the NCG (five vs. six; p < 0.001). Furthermore, the RSG had lower incidences of febrile neutropenia, myelosuppression, and gastrointestinal reactions (all p < 0.05). The severity of surgery-related complications did not differ significantly. CONCLUSION Upfront surgical resection in children with PRETEXT III and IV HB is safe and feasible, and reduces the total number of courses and side effects of chemotherapy. The degree of vascular involvement is the most important consideration when evaluating resectability during diagnosis.
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Affiliation(s)
- Xiongwei Wu
- Department of Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
- Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Jianyong Wang
- Department of Pediatrics, Yantai Yuhuangding Hospital, Shandong, China
| | - Yuhe Duan
- Department of Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Yusheng Liu
- Department of Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Yao Liu
- Department of Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Xin Chen
- Department of Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Nan Xia
- Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Qian Dong
- Department of Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
- Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
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11
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Fuchs J, Murtha-Lemekhova A, Rabaux-Eygasier L, Kessler M, Ruping F, Günther P, Hoffmann K. Evidence on Indications and Techniques to Increase the Future Liver Remnant in Children Undergoing Extended Hepatectomy: A Systematic Review and Meta-Analysis of Individual Patient Data. Front Pediatr 2022; 10:915642. [PMID: 35712634 PMCID: PMC9197416 DOI: 10.3389/fped.2022.915642] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2022] [Accepted: 05/10/2022] [Indexed: 11/16/2022] Open
Abstract
BACKGROUND Techniques to increase the future liver remnant (FLR) have fundamentally changed the indications and criteria of resectability in adult liver surgery. In pediatric patients however, these procedures have rarely been applied and the potential benefit or harm as well as suited indications are unclear. METHODS A systematic literature search of MEDLINE, Web of Science, and CENTRAL was conducted. Based on a PRISMA-compliant, predefined methodology, all studies reporting pediatric patients (< 18y) undergoing liver resection with either associating liver partition and portal vein ligation for stages hepatectomy (ALPPS) or preoperative portal vein embolization or ligation (PVE/PVL) were included. Baseline data, periinterventional morbidity, increase of FLR and outcomes were analyzed. RESULTS 15 studies reporting on 21 pediatric patients with a mean age of 4 years and 7 months (range 1.8 months - 17 years) were included. 12 ALPPS procedures, 8 PVE and 1 PVL were performed. The applied criteria for performing ALPPS or PVE were heterogenous and thresholds for minimally acceptable FLR varied. Mean FLR [% of total liver volume] before the intervention was 23.6% (range 15.0 - 39.3%) in the ALPPS group and 31.4% (range 21.5 - 56.0%) in the PVE group. Mean increase of FLR before stage 2 resection was 69.4% (range 19.0 - 103.8%) for ALPPS and 62.8% (range 25.0 - 108.0%) after PVE. No postoperative death occurred, one early intrahepatic recurrence after an ALPPS procedure was reported. Overall postoperative morbidity was 23.8%. CONCLUSION Validated criteria for minimal FLR in pediatric liver resection are lacking and so are clear indications for ALPPS or PVE. In special cases, ALPPS and PVE can be valuable techniques to achieve complete resection of pediatric liver tumors. However, more data are needed, and future studies should focus on a definition and validation of posthepatectomy liver failure as well as the minimally needed FLR in pediatric patients undergoing extended hepatectomy. SYSTEMATIC REVIEW REGISTRATION [www.clinicaltrials.gov], identifier [PROSPERO 2021 CRD42021274848].
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Affiliation(s)
- Juri Fuchs
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany.,Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University of Heidelberg, Heidelberg, Germany
| | - Anastasia Murtha-Lemekhova
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany.,Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University of Heidelberg, Heidelberg, Germany
| | - Lucas Rabaux-Eygasier
- Department of Pediatric Surgery, Hôpital Kremlin Bicêtre, Assistance Publique, Hôpitaux de Paris, Paris, France
| | - Markus Kessler
- Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University of Heidelberg, Heidelberg, Germany.,Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Fabian Ruping
- Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University of Heidelberg, Heidelberg, Germany.,Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Patrick Günther
- Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University of Heidelberg, Heidelberg, Germany.,Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Katrin Hoffmann
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany.,Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University of Heidelberg, Heidelberg, Germany
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12
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Yang X, Wang H, Dong B, Hu B, Hao X, Chen X, Zhao J, Dong Q, Zhu C. Standard Liver Volume-Predicting Formulae Derived From Normal Liver Volume in Children Under 18 Years of Age. Front Pediatr 2021; 9:629645. [PMID: 33681103 PMCID: PMC7933551 DOI: 10.3389/fped.2021.629645] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2020] [Accepted: 02/01/2021] [Indexed: 12/17/2022] Open
Abstract
Background: Standard liver volume (SLV) is important in risk assessment for major hepatectomy. We aimed to investigate the growth patterns of normal liver volume with age and body weight (BW) and summarize formulae for calculating SLV in children. Methods: Overall, 792 Chinese children (<18 years of age) with normal liver were enrolled. Liver volumes were measured using computed tomography. Correlations between liver volume and BW, body height (BH), and body surface area (BSA) were analyzed. New SLV formulae were selected from different regression models; they were assessed by multicentral validations and were compared. Results: The growth patterns of liver volume with age (1 day-18 years) and BW (2-78 kg) were summarized. The volume grows from a median of 139 ml (111.5-153.6 in newborn) to 1180.5 ml (1043-1303.1 at 16-18 years). Liver volume was significantly correlated with BW (r = 0.95, P < 0.001), BH (r = 0.92, P < 0.001), and BSA (r = 0.96, P < 0.001). The effect of sex on liver volume increases with BW, and BW of 20 kg was identified as the optimal cutoff value. The recommended SLV formulae were BW≤20 kg: SLV = 707.12 × BSA 1.09; BW>20 kg, males: SLV = 691.90 × BSA 1.06; females: SLV = 663.19 × BSA 1.04. Conclusions: We summarized the growth patterns of liver volume and provided formulae predicting SLV in Chinese children, which is useful in assessing the safety of major hepatectomies.
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Affiliation(s)
- Xintian Yang
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
- Medical College of Qingdao University, Qingdao University, Qingdao, China
| | - Han Wang
- Medical College of Qingdao University, Qingdao University, Qingdao, China
| | - Bingzi Dong
- Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Bin Hu
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Xiwei Hao
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Xin Chen
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Jing Zhao
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Qian Dong
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
- Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
- Shandong College Collaborative Innovation Center of Digital Medicine Clinical Treatment and Nutrition Health, Qingdao University, Qingdao, China
| | - Chengzhan Zhu
- Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
- Department of Hepatobiliary and Pancreatic Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
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13
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He W, Zhang J, Liu B, Liu X, Liu G, Xie L, He J, Wei M, Li K, Ma J, Dong R, Ma D, Dong K, Ye M. S119N Mutation of the E3 Ubiquitin Ligase SPOP Suppresses SLC7A1 Degradation to Regulate Hepatoblastoma Progression. MOLECULAR THERAPY-ONCOLYTICS 2020; 19:149-162. [PMID: 33209975 PMCID: PMC7644817 DOI: 10.1016/j.omto.2020.09.008] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 07/01/2020] [Accepted: 09/30/2020] [Indexed: 02/07/2023]
Abstract
A previous study on hepatoblastoma revealed novel mutations and cancer genes in the Wnt pathway and ubiquitin ligase complex, including the tumor suppressor speckle-type BTB/POZ (SPOP). Moreover, the SPOP gene affected cell growth, and its S119N mutation was identified as a loss-of-function mutation in hepatoblastoma. This study aimed to explore more functions and the potential mechanism of SPOP and its S119N mutation. The in vitro effects of SPOP on cell proliferation, invasion, apoptosis, and in vivo tumor growth were investigated by western blot analysis, Cell Counting Kit-8, colony formation assay, flow cytometry, and xenograft animal experiments. The substrate of SPOP was discovered by a protein quantification assay and quantitative ubiquitination modification assay. The present study further proved that SPOP functioned as an anti-oncogene through the phosphatidylinositol 3-kinase/Akt signaling pathway to affect various malignant biological behaviors of hepatoblastoma both in vitro and in vivo. Furthermore, experimental results also suggested that solute carrier family 7 member 1 (SLC7A1) might be a substrate of SPOP and influence cell phenotype by regulating arginine metabolism. In conclusion, these findings demonstrated the function of SPOP and revealed a potential substrate related to hepatoblastoma tumorigenesis, which might thus provide a novel therapeutic target for hepatoblastoma.
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Affiliation(s)
- Weijing He
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Jingjing Zhang
- Department of Medical Imaging, Nanjing Hospital of Chinese Medicine Affiliated to Nanjing University of Chinese Medicine, Nanjing 210001, China
| | - Baihui Liu
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Xiangqi Liu
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Gongbao Liu
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Lulu Xie
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Jiajun He
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Meng Wei
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Kai Li
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Jing Ma
- ENT Institute, Department of Facial Plastic and Reconstructive Surgery, Eye and ENT Hospital, Fudan University, Shanghai 200031, China
| | - Rui Dong
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
| | - Duan Ma
- Key Laboratory of Metabolism and Molecular Medicine, Ministry of Education, Department of Biochemistry and Molecular Biology, Institute of Biomedical Sciences, Collaborative Innovation Center of Genetics and Development, School of Basic Medical Sciences, Fudan University, Shanghai 200032, China
- Shanghai Key Laboratory of Birth Defect, Children’s Hospital of Fudan University, Shanghai 201102, China
- Corresponding author Duan Ma, Key Laboratory of Metabolism and Molecular Medicine, Ministry of Education, Department of Biochemistry and Molecular Biology, Institute of Biomedical Sciences, Collaborative Innovation Center of Genetics and Development, School of Basic Medical Sciences, Fudan University, Shanghai 200032, China.
| | - Kuiran Dong
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
- Corresponding author Kuiran Dong, Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China.
| | - Mujie Ye
- Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China
- Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China
- Corresponding author Mujie Ye, Department of Pediatric Surgery, Children’s Hospital of Fudan University, Shanghai 201102, China.
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14
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Shen G, Wu L, Zhao J, Wei B, Zhou X, Zhuo X, Dong Q. Imaging and Pathology Study of the Chemotherapy Regression Area of Hepatoblastoma - A Prospective Single-Center Study. Fetal Pediatr Pathol 2020; 39:307-316. [PMID: 31448676 DOI: 10.1080/15513815.2019.1652375] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/13/2019] [Revised: 07/18/2019] [Accepted: 07/29/2019] [Indexed: 12/22/2022]
Abstract
Objective: The objective of this study is to determine the frequency of no residual cancer tissue in the chemotherapy regression area (CRA) of hepatoblastoma after preoperative chemotherapy and to measure the distance between the tumor capsule and the residual cancer nests. Methods: All the tissues in the CRAs of the resected specimens were excised. HE staining and immunohistochemical staining were performed to determine the frequency of residual cancer tissue in the CRA, and the distances between the residual cancer nests and the tumor capsule were measured. Results: A total of 30 patients were included in the study. The tumor volume decreased after chemotherapy by an average of 619 ml. Of the 30 patients, the CRAs of 18 still had residual cancer nests. The longest distance between the residual cancer nest and tumor capsule was 11.2 mm. Conclusions: After chemotherapy, 60% of patients still had residual cancer nests in CRAs, the furthest distance was 11.2 mm.
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Affiliation(s)
- Gang Shen
- Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
- Weifang People's Hospital, Pediatric Surgery, Weifang, China
| | - Linlin Wu
- Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Jie Zhao
- Pathology Group of Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Bin Wei
- Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, Qingdao, China
| | - Xianjun Zhou
- Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Xiaoying Zhuo
- Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - Qian Dong
- Pediatric Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
- Shandong Key Laboratory of Digital Medicine and Computer Assisted Surgery, Qingdao, China
- Shandong College Collaborative Innovation Center of Digital Medicine in Clinical Treatment and Nutrition Health, Qingdao, China
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15
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Filin AV, Kim EF, Charchyan ER, Metelin AV, Dymova OV, Kachanov DY, Moiseenko RA, Khovrin VV, Kryzhanovskaya EY, Kazakova OV, Ryabov AB, Bazarov DV. [Liver transplantation for hepatoblastoma in children]. Khirurgiia (Mosk) 2020:5-13. [PMID: 33210501 DOI: 10.17116/hirurgia20201115] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/11/2023]
Abstract
OBJECTIVE To improve the outcomes in children with hepatoblastoma. MATERIAL AND METHODS There were 160 children with focal liver lesions who underwent surgery at the department of liver transplantation in 2008-2019. Patients with malignant tumors made up 77% (n=123). Hepatoblastoma (HB) prevailed (86%, n=106). Liver transplantation was performed in 19 (18%) patients with HB. Median follow-up after transplantation was 24.3 months by December 2019. Follow-up period did not exceed 4 years in more than 2/3 of patients. RESULTS Overall and disease-free 10-year survival was 87.1% and 82.7%, respectively. Similar values were observed after resections (91.1% and 86.6%). At the same time, actuarial 4-year survival after liver transplantation for HB was 68%. CONCLUSION Improvement of treatment outcomes may be achieved through multidisciplinary interaction ensuring timely drug therapy and liver transplantation.
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Affiliation(s)
- A V Filin
- Petrovsky National Research Centre of Surgery, Moscow, Russia
| | - E F Kim
- Moscow City Hospital of Oncology No. 62, Moscow, Russia
| | - E R Charchyan
- Petrovsky National Research Centre of Surgery, Moscow, Russia
| | - A V Metelin
- Petrovsky National Research Centre of Surgery, Moscow, Russia
| | - O V Dymova
- Petrovsky National Research Centre of Surgery, Moscow, Russia
| | - D Yu Kachanov
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia
| | - R A Moiseenko
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia
| | - V V Khovrin
- Petrovsky National Research Centre of Surgery, Moscow, Russia
| | | | - O V Kazakova
- Petrovsky National Research Centre of Surgery, Moscow, Russia
| | - A B Ryabov
- Herzen Moscow Oncology Research Center, Moscow, Russia
| | - D V Bazarov
- Petrovsky National Research Centre of Surgery, Moscow, Russia
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16
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Surgical Management of Hepatoblastoma and Recent Advances. Cancers (Basel) 2019; 11:cancers11121944. [PMID: 31817219 PMCID: PMC6966548 DOI: 10.3390/cancers11121944] [Citation(s) in RCA: 41] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2019] [Revised: 11/18/2019] [Accepted: 11/29/2019] [Indexed: 12/29/2022] Open
Abstract
Hepatoblastoma is the most common childhood liver malignancy. The management of hepatoblastoma requires multidisciplinary efforts. The five-year overall survival is approximately 80% in developed countries. Surgery remains the mainstay of treatment for hepatoblastoma, and meticulous techniques must be employed to ensure safe and effective local control surgeries. Additionally, there have been several advances from both pediatric and adult literature in the way liver tumor surgery is performed. In this review, we highlight important aspects of liver surgery for hepatoblastoma, the management of metastatic disease, and the most current technical advances in performing these procedures in a safe and effective manner.
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Abstract
Advanced stage hepatoblastoma, including both locally advanced primary tumors as well as metastatic disease, poses unique clinical challenges. Despite substantial advances in chemotherapeutics, surgical extirpation remains the mainstay of cure for this tumor. Locally advanced tumors that involve multiple hepatic lobes and/or invade significant vascular structures can be managed either by complex hepatic resections or liver transplantation. We review the indications, roles, and outcomes of these surgical approaches as well as those for the resection of pulmonary metastases.
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Lezama-Del Valle P, Krauel L, LaQuaglia MP. Error traps and culture of safety in pediatric surgical oncology. Semin Pediatr Surg 2019; 28:164-171. [PMID: 31171152 DOI: 10.1053/j.sempedsurg.2019.04.014] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/01/2023]
Abstract
This article reviews technical issues to improve surgical safety and avoid surgical errors in pediatric surgical oncology, particularly in the three most common extracranial solid tumors: neuroblastoma, hepatoblastoma and Wilms tumor. The use of adjuvant chemotherapy - when indicated - the use of tumor specific classifications, adequate surgical planning, that may include the use of 3D printable models, improved surgical instruments and technology, and following surgical guidelines, would result in avoiding error, increased safety, and therefore in improved surgical outcomes.
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Affiliation(s)
- Pablo Lezama-Del Valle
- Surgical Oncology Service, Department of General Surgery, Hospital Infantil de México Federico Gómez, Mexico City, Mexico.
| | - Lucas Krauel
- Pediatric Surgical Oncology Unit, Department of Pediatric Surgery, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain
| | - Michael P LaQuaglia
- Pediatric Surgical Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
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