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1.
BMC Pediatr ; 24(1): 243, 2024 Apr 05.
Artículo en Inglés | MEDLINE | ID: mdl-38580968

RESUMEN

Cholangiocarcinoma in patients with Choledochal cysts is rare in childhood; however, it seriously affects the prognosis of the disease. The key to addressing this situation lies in completely removing the extrahepatic cyst. We herein present a case report of a 3-year-old boy with cholangiocarcinoma associated with a choledochal cyst (CDC). Preoperative 3D simulation, based on CT data, played an important role in the treatment of this patient.


Asunto(s)
Neoplasias de los Conductos Biliares , Colangiocarcinoma , Quiste del Colédoco , Masculino , Humanos , Preescolar , Quiste del Colédoco/complicaciones , Quiste del Colédoco/diagnóstico por imagen , Quiste del Colédoco/cirugía , Colangiocarcinoma/complicaciones , Colangiocarcinoma/diagnóstico por imagen , Colangiocarcinoma/patología , Conductos Biliares Intrahepáticos/diagnóstico por imagen , Conductos Biliares Intrahepáticos/patología , Neoplasias de los Conductos Biliares/complicaciones , Neoplasias de los Conductos Biliares/diagnóstico por imagen , Neoplasias de los Conductos Biliares/patología
2.
BMC Surg ; 24(1): 236, 2024 Aug 21.
Artículo en Inglés | MEDLINE | ID: mdl-39169378

RESUMEN

BACKGROUD: The study objective was to compare three-dimensional and two-dimensional imaging using computer-assisted systems (CASs) in clinical guidance for preoperative surgical planning for middle hepatic tumors in children. METHODS: A retrospective analysis was performed on 23 children who underwent surgery for middle hepatic tumors in our hospital from January 2016 to June 2022. The surgical resection plan was formulated by the operator team using two-dimensional CT images before the operation. Then, the same qualified surgeons conducted an in-depth analysis and formulated the surgical resection scheme for the same pediatric patient using three-dimensional imaging of the middle hepatic tumor. The feasibility of the two schemes was compared and analyzed. RESULT: All the tumors were successfully removed according to the preoperative method developed using three-dimensional imaging. The postoperative short-term follow-up revealed that all patients were doing well. Preoperative plans were revised in 9 cases after evaluating the three-dimensional images due to the disparity between the original plans and the three-dimensional relationship between the tumor and blood vessels, vascular variation, and the volume of remnant liver. CONCLUSIONS: Three-dimensional imaging with a computer-assisted surgery system is superior to two-dimensional imaging in the preoperative planning of pediatric hepatoblastoma.


Asunto(s)
Hepatectomía , Imagenología Tridimensional , Neoplasias Hepáticas , Cirugía Asistida por Computador , Tomografía Computarizada por Rayos X , Humanos , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/diagnóstico por imagen , Estudios Retrospectivos , Femenino , Masculino , Cirugía Asistida por Computador/métodos , Niño , Tomografía Computarizada por Rayos X/métodos , Preescolar , Hepatectomía/métodos , Cuidados Preoperatorios/métodos , Lactante , Adolescente , Estudios de Factibilidad
3.
Crit Rev Eukaryot Gene Expr ; 32(8): 1-8, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36017911

RESUMEN

Long noncoding RNA (lncRNA) PCAT29 has been characterized as a tumor suppressor in several types of cancer, although its involvement in neuroblastoma (NB) is unknown. In this study, we analyzed the role of PCAT29 in NB. In paired NB and nontumor tissues from 56 patients with NB, microRNA (miR)-21 and PCAT29 expression was determined with reverse transcription quantitative PCR. Correlation between miR-21 and PCAT29 was evaluated with linear regression. The interaction between miR-21 and PCAT29 was predicted by the IntaRNA 2.0 program. In NB cells, miR-21 and PCAT29 were overexpressed to explore their relationship. In NB cell proliferation, the roles of miR-21 and PCAT29 were analyzed with propidium iodide staining and Ki67 staining assays. The results showed that PCAT29 was downregulated and miR-21 was upregulated in NB. MiR-21 was inversely correlated with PCAT29. RNA-RNA interaction prediction revealed that miR-21 might target PCAT29. MiR-21 overexpression reduced PCAT29 expression and increased NB cell proliferation, whereas PCAT29 overexpression inhibited NB cell proliferation. PCAT29 overexpression promoted NB cell apoptosis, while miR-21 overexpression inhibited NB cell apoptosis and attenuated PCAT29 overexpression-mediated NB cell apoptosis. In conclusion, MiR-21 may target PCAT29 to promote cell apoptosis in NB.


Asunto(s)
MicroARNs , Neuroblastoma , ARN Largo no Codificante , Línea Celular Tumoral , Movimiento Celular/genética , Proliferación Celular/genética , Regulación Neoplásica de la Expresión Génica/genética , Humanos , MicroARNs/genética , MicroARNs/metabolismo , Neuroblastoma/genética , Neuroblastoma/metabolismo , Neuroblastoma/patología , ARN Largo no Codificante/genética
4.
BMC Pediatr ; 22(1): 9, 2022 01 03.
Artículo en Inglés | MEDLINE | ID: mdl-34980023

RESUMEN

BACKGROUND: Necrotizing enterocolitis (NEC) is a gastrointestinal disease that tends to occur in premature infants. Some features may be associated with an increased probability that preterm infants with NEC will require surgical treatment. This study aimed to identify the factors that increased the probability of surgical treatment in infants with NEC. METHODS: We retrospectively analyzed the data of premature infants with NEC who were hospitalized at The Affiliated Hospital of Qingdao University from April 2011 to April 2021. According to the treatments received, these patients were divided into medical NEC group and surgical NEC group. The perinatal characteristics, clinical manifestations, and laboratory values before the onset of NEC were subjected to univariate and multivariate analyses. RESULTS: A total of 623 preterm infants with NEC (> Bell's stage I) were included in this study, including 350 (56%) who received surgical treatment and 273 (44%) who received conservative medical treatment. Multivariate analysis showed that lower gestational age (P = 0.001, odds ratio (OR) (95% CI) = 0.91[0.86-0.96]), early occurrence of NEC (P = 0.003, OR (95% CI) = 0.86 [0.77-0.95]), hemodynamically significant patent ductus arteriosus (P = 0.003, OR (95% CI) = 7.50 [2.03-28.47]), and low serum bicarbonate (P = 0.043, OR (95% CI) = 0.863 [0.749-0.995]) were associated with an increased probability of surgical treatment in preterm infants with NEC. CONCLUSIONS: Our findings were applied to identify potential predictors for surgical treatment in preterm infants with NEC, which may facilitate early decisive management.


Asunto(s)
Conducto Arterioso Permeable , Enterocolitis Necrotizante , Enfermedades del Prematuro , Conducto Arterioso Permeable/tratamiento farmacológico , Enterocolitis Necrotizante/epidemiología , Enterocolitis Necrotizante/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , Enfermedades del Prematuro/cirugía , Embarazo , Estudios Retrospectivos
5.
Fetal Pediatr Pathol ; 41(3): 371-380, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32969743

RESUMEN

Objective: We investigated three-dimensional (3 D) reconstruction for the assessment of the tumor margin microstructure of hepatoblastoma (HB). Methods: Eleven surgical resections of childhood hepatoblastomas obtained between September 2018 and December 2019 were formalin-fixed, paraffin-embedded, serially sectioned at 4 µm, stained with hematoxylin and eosin (every 19th and 20th section stained with alpha-fetoprotein and glypican 3), and the digital images of all sections were acquired at 100× followed by image registration using the B-spline based method with modified residual complexity. Reconstruction was performed using 3 D Slicer software. Results: The reconstructed orthogonal 3 D images clearly presented the internal microstructure of the tumor margin. The rendered 3 D image could be rotated at any angle. Conclusions: Microstructure 3 D reconstruction is feasible for observing the pathological structure of the HB tumor margin.


Asunto(s)
Hepatoblastoma , Neoplasias Hepáticas , Humanos , Imagenología Tridimensional/métodos
6.
J Surg Res ; 259: 458-464, 2021 03.
Artículo en Inglés | MEDLINE | ID: mdl-33059908

RESUMEN

BACKGROUND: Perioperative complications are common during the surgical treatment of pediatric retroperitoneal teratoma (RPT). Some clinical and radiographic features could be associated with perioperative complications. This study was designed to identify the factors associated with such complications. MATERIALS AND METHODS: We retrospectively analyzed the clinical data of RPT patients who underwent surgical treatment at the Department of Pediatric Surgery of The Affiliated Hospital of Qingdao University between January 2008 and January 2020, including demographics, imaging data, intraoperative findings, perioperative complications, pathological data, and outcomes. RESULTS: A total of 91 patients were included in this study, including 30 boys and 61 girls. Of these, 71 patients (78%) were 1 y old or younger. Thirty-eight patients (41%) had perioperative complications (44 intraoperative and 7 postoperative). Preoperative imaging studies showed that the tumor distorted adjacent arteries, veins, and organs in all patients. More veins and organs were displaced and distorted by the tumor in patients who had perioperative complications. Multivariate analysis showed that the number of organs compressed and distorted by the tumor was significantly related to perioperative complications (odds ratio 1.69, 95% confidence interval 1.19-2.41). CONCLUSIONS: Surgical treatment of RPT is complex and challenging. As majority are benign, a complete excision is usually curative. The number of organs compressed and distorted by the tumor is positively related to perioperative complications.


Asunto(s)
Complicaciones Intraoperatorias/epidemiología , Complicaciones Posoperatorias/epidemiología , Neoplasias Retroperitoneales/cirugía , Teratoma/cirugía , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Complicaciones Intraoperatorias/etiología , Complicaciones Intraoperatorias/prevención & control , Masculino , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/prevención & control , Neoplasias Retroperitoneales/irrigación sanguínea , Neoplasias Retroperitoneales/diagnóstico , Neoplasias Retroperitoneales/patología , Espacio Retroperitoneal/diagnóstico por imagen , Espacio Retroperitoneal/cirugía , Estudios Retrospectivos , Factores de Riesgo , Teratoma/irrigación sanguínea , Teratoma/diagnóstico , Teratoma/patología , Resultado del Tratamiento
7.
Pediatr Surg Int ; 37(11): 1575-1583, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34309718

RESUMEN

PURPOSE: To explore the treatment of pediatric hepatic hemangioma and the role of the Hisense computer-assisted surgery (Hisense CAS) system in diagnosis and treatment. METHODS: We collected the clinical and follow-up data of all pediatric hepatic hemangioma cases in our pediatric surgery department from March 2008 to March 2021 for retrospective analysis. The Hisense CAS system was used to create three-dimensional (3D) reconstructions based on computed tomography data. RESULTS: There were 71 patients, mainly infants (prenatal to 39 months). There were more males than females (42 vs. 29), and the alpha-fetoprotein level was significantly increased in 8 cases. 3D reconstruction by the Hisense CAS system showed that hepatic artery tracking was helpful for the differential diagnosis of pediatric hepatic hemangioma. Twenty-three children treated with propranolol showed significant differences in the tumour diameter, volume, and tumour-to-liver volume ratio after treatment (all P < 0.05). Compared with early surgical treatment, the curative effect of this approach was obvious. CONCLUSION: As a non-surgical treatment for symptomatic pediatric hepatic hemangioma, propranolol can replace surgical resection to a certain extent and reduce the proportion of children who need surgical intervention. Hisense CAS has advantages in evaluating the tumour volume before and after propranolol treatment. The liver volume and the liver tumour volume percentage provide new perspectives for evaluating the tumour outcome.


Asunto(s)
Hemangioma , Neoplasias Hepáticas , Cirugía Asistida por Computador , Niño , Femenino , Hemangioma/diagnóstico , Hemangioma/cirugía , Humanos , Lactante , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/cirugía , Masculino , Embarazo , Estudios Retrospectivos , Tomografía Computarizada por Rayos X
8.
Pediatr Hematol Oncol ; 38(4): 319-330, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-33667139

RESUMEN

Hepatoblastoma (HB) is the most common liver cancer in children, this study aims at analyzing the prognostic factors affecting the survival rates and summarizing the treatment experience. In this study, we reviewed patients with primary HB under the age of 14 years who underwent complete tumor resection from June 1997 to March 2019. The data of 72 patients were collected. Survival analysis was performed by Kaplan-Meier, multivariate Cox proportional hazards regression and linear mixed model for repeated measures (LMMRM). The 5-year and the 10-year event-free survival (EFS) of all patients were 78.2% and 73%, respectively. Both the 5-year and 10-year overall survival (OS) were 85.7%. Kaplan-Meier survival analysis showed that patients with tumor capsule infiltration (TCI) and patients with surgical margin less than 1 cm may also have a good prognosis. The Cox proportional hazards regression model analysis results were similar to the Kaplan-Meier analysis results. LMMRM analysis showed that there were significant differences in platelet, alpha-fetoprotein, C-reactive protein and hemoglobin values after surgery in the metastasis group (P < 0.05). This study suggests that patients with TCI or narrow surgical margin (<1 cm) may also have a good prognosis, and the risk stratification of HB can be used as the latest grading standard to evaluate the prognosis of patients.


Asunto(s)
Hepatoblastoma/epidemiología , Neoplasias Hepáticas/epidemiología , Preescolar , China/epidemiología , Supervivencia sin Enfermedad , Hepatoblastoma/diagnóstico , Hepatoblastoma/terapia , Humanos , Estimación de Kaplan-Meier , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/terapia , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia
9.
Cell Mol Biol Lett ; 25: 5, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32082390

RESUMEN

BACKGROUND: MicroRNAs (miRNAs or miRs) can participate in the development and progression of neuroblastoma. Many studies have indicated that miR-429 can participate in tumor development. However, the mechanism underlying miR-429-mediated progression of neuroblastoma remains largely unclear. METHODS: Colony formation and apoptosis assays were used to determine the effect of miR-429 on cell proliferation. Its impact on cell migration was determined using the wound-healing and Transwell assays. The target gene of miR-429 was confirmed via western blotting and luciferase reporter assays. A nude mouse xenograft model with miR-429 overexpression was used to assess the effect on tumor growth. RESULTS: Our findings indicate that miR-429 is downregulated in neuroblastoma cell lines. We also found that it can induce apoptosis and inhibit proliferation in cells of those lines. MiR-429 can bind to the 3'-UTR of IKKß mRNA and overexpression of IKKß can reverse cell proliferation, blocking the effect of miR-429. Furthermore, miR-429 overexpression inhibited neuroblastoma growth in our nude mouse xenograft model. CONCLUSION: We provide important insight into miR-429 as a tumor suppressor through interaction with IKKß, which is a catalytic subunit of the IKK complex that activates NF-κB nuclear transport. Our results demonstrate that miR-429 may be a new target for the treatment of neuroblastoma.


Asunto(s)
Movimiento Celular/genética , Proliferación Celular/genética , Genes Supresores de Tumor , MicroARNs/metabolismo , FN-kappa B/metabolismo , Neuroblastoma/metabolismo , Regiones no Traducidas 3' , Animales , Apoptosis/genética , Línea Celular Tumoral , Regulación hacia Abajo , Regulación Neoplásica de la Expresión Génica/genética , Humanos , Quinasa I-kappa B/genética , Quinasa I-kappa B/metabolismo , Ratones , Ratones Endogámicos BALB C , Ratones Desnudos , MicroARNs/genética , Neuroblastoma/genética , Transducción de Señal/genética , Ensayos Antitumor por Modelo de Xenoinjerto
10.
BMC Surg ; 20(1): 244, 2020 Oct 19.
Artículo en Inglés | MEDLINE | ID: mdl-33076895

RESUMEN

BACKGROUND: Pediatric inguinal hernia (PIH) is a common disease in children. Laparoscopic hernia repair (LHR) has developed rapidly in recent years, but there are still different opinions compared with traditional open hernia repair (OHR). The purpose of this study was to compare the advantages and disadvantages of LHR and OHR in the treatment of pediatric inguinal hernia. METHODS: We performed a retrospective review of all children (< 14 years) who underwent repair of inguinal hernia in the pediatric surgery center of the Affiliated Hospital of Qingdao University from January 2015 to December 2015. We collected the medical records of all the children and analyzed the clinical characteristics, operation-related information and follow-up. RESULTS: In the OHR group, 202 cases underwent unilateral inguinal hernia repair, and 43 cases underwent bilateral inguinal hernia repair. In the LHR group, 168 cases underwent unilateral inguinal hernia repair, and 136 cases underwent bilateral inguinal hernia repair. There was a significant difference in the operation time between the two groups, but there were no significant differences in postoperative hospitalization time and incidence of ipsilateral recurrent hernia between the two groups. The incidence rates of metachronous contralateral hernia (MCH) and surgical site infection in LHR group were significantly lower than those in the OHR group. CONCLUSION: Our study shows that compared with OHR, LHR has the advantages of concealed incision, minimal invasiveness, reduced operation time, detection of contralateral patent processus vaginalis, and reduced incidence of MCH. In conclusion, LHR is safe and effective in the treatment of pediatric indirect inguinal hernia.


Asunto(s)
Hernia Inguinal/cirugía , Laparoscopía/métodos , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Niño , Preescolar , China/epidemiología , Femenino , Herniorrafia , Humanos , Masculino , Estudios Retrospectivos , Resultado del Tratamiento
11.
Surg Radiol Anat ; 42(12): 1467-1473, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-32424682

RESUMEN

PURPOSE: To study the variations of the right branch of the hepatic portal vein in children. METHODS: A total of 810 children's abdominal CT images were reconstructed with three-dimensional (3D) simulation software, Variations of the right branch of the hepatic portal vein were analyzed and classified. RESULTS: The most common anatomy (type A) was seen in 355 patients (43.83%). Trifurcation in the right anterior portal vein (type B) variation was seen in 250 cases (30.86%). The right posterior portal vein arched without obvious branching (type C) was seen in 71 cases (8.77%). There were 134 special variants (16.54%) named type D, including 14 cases (1.73%) with the right anterior branch in four sub-branches, 13 cases (1.60%) in one trunk and multiple sub-branches, 92 cases (11.36%) originating from the left trunk of the portal vein, and 15 cases (1.85%) with the VI segment of the portal vein originating from the right anterior branch of the portal vein. CONCLUSION: Variations in the right branch of the hepatic portal vein seems to be very frequent. Recognition of such variations is important in the preoperative evaluation of children with surgery planned, because these variations may have implications for anatomy-guided liver resection and for planning the operative approach.


Asunto(s)
Variación Anatómica , Vena Porta/anatomía & histología , Adolescente , Niño , Preescolar , Femenino , Humanos , Imagenología Tridimensional , Lactante , Masculino , Vena Porta/diagnóstico por imagen , Estudios Retrospectivos , Tomografía Computarizada Espiral
12.
Epidemiol Infect ; 147: e305, 2019 11 26.
Artículo en Inglés | MEDLINE | ID: mdl-31767044

RESUMEN

Epidemiological data for Toxoplasma gondii regarding malignancy have gained increasing attention; however, the information about T. gondii infection among children with malignant lymphoma (ML) in China is unclear. Therefore, 314 children with lymphoma and 314 healthy children, age- and gender-matched, were recruited to estimate the seroprevalence of T. gondii in the participants and identify the risk factors of infection. Blood samples from all participants were collected and examined for T. gondii IgG and IgM antibodies using ELISA. The results showed that the overall seroprevalence of T. gondii antibodies (including IgG and/or IgM) in ML patients and healthy controls was 19.8% and 9.9%, respectively. Contact with the cats, consumption of oysters and history of chemotherapy were estimated to be the risk factors for T. gondii infection in children with lymphoma by multivariable logistic regression analysis, whereas in healthy children, contact with cats and consumption of oysters were the risk factors. Moreover, among various histological types of lymphoma, individuals with NK/T-cell lymphoma, B-small lymphocytic lymphoma, marginal zone B-lymphoma and Hodgkin's lymphoma had a higher seroprevalence than healthy controls (P < 0.05). These findings indicated the high prevalence of T. gondii infection in children with lymphoma, and hence, efforts should be performed to evaluate the effect of the infection further in lymphoma patients.


Asunto(s)
Linfoma/parasitología , Toxoplasmosis/complicaciones , Adolescente , Anticuerpos Antiprotozoarios/sangre , Biomarcadores/sangre , Estudios de Casos y Controles , Niño , Preescolar , China/epidemiología , Femenino , Humanos , Lactante , Recién Nacido , Modelos Logísticos , Masculino , Factores de Riesgo , Estudios Seroepidemiológicos , Toxoplasma/inmunología , Toxoplasmosis/sangre , Toxoplasmosis/diagnóstico , Toxoplasmosis/epidemiología
13.
Pediatr Surg Int ; 32(4): 387-95, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26809670

RESUMEN

INTRODUCTION: Three-dimensional (3D) imaging instead of two-dimensional (2D) computed tomography (CT) for diagnosis and preoperative planning in infants and young children with complex liver tumors is a promising technique for precision hepatectomy. METHODS: This study was a retrospective analysis of 26 infants and young children with giant liver tumors involving the hepatic hilum who underwent precise hepatectomy at the Affiliated Hospital of Qingdao University between February 2012 and January 2015. All patients received upper abdominal contrast-enhanced CT scanning before surgery. 16 patients used Hisense CAS system for 3D reconstruction as the reconstruction group. While ten patients underwent 3D CT reconstruction by the CT Workstation as the control group. The clinical outcomes were analyzed and compared between the two groups. The 3D reconstruction of abdominal organs and blood vessels was generated using the Hisense CAS system. Diagnosis and preoperative planning assisted by the system was used for preoperative and intraoperative decision-making for precise hepatectomy. RESULTS: All patients underwent successful surgery. The 3D models clearly demonstrated the association of liver tumors with the intrahepatic vascular system and provided a preoperative assessment of resectability, assisting surgeons in preoperative procedural planning. Anatomic hepatectomy was successfully completed in the reconstruction group. The mean operation time was shorter in the reconstruction group (137.81 ± 17.51 min) than in the control group (192 ± 34.66 min) (P < 0.01). The mean intraoperative blood loss was lesser in the reconstruction group (21.81 ± 14.05 ml) than in the control group (53.50 ± 21.35 ml) (P < 0.01). The difference was statistically significant. DISCUSSION: 2D CT scan images cannot accurately display the spatial relationship between the tumor and surrounding vasculature. The 3D reconstruction model used in this study gave detailed and accurate anatomical information and allowed for the assessment of tumor resectability and provided a detailed road map for preoperative decision-making and predicted the postoperative liver function. CONCLUSIONS: 3D visualization technology provides preoperative assessment and allows individualized surgical planning. Surgical controllability, accuracy, and safety can be improved in infants and young children undergoing precise hepatectomy for complex liver tumors.


Asunto(s)
Hepatectomía/métodos , Imagenología Tridimensional , Neoplasias Hepáticas/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Hígado , Neoplasias Hepáticas/patología , Neoplasias Hepáticas/cirugía , Masculino , Cuidados Preoperatorios , Estudios Retrospectivos
14.
Heliyon ; 10(4): e26581, 2024 Feb 29.
Artículo en Inglés | MEDLINE | ID: mdl-38420445

RESUMEN

Neuroblastoma is a childhood cancer characterized by the formation of tumors derived from neuroblasts. Identifying the genetic mutations underlying neuroblastoma for genetic counseling and early diagnosis is essential. Thus, this study aimed to screen for pathogenic gene variants within a neuroblastoma family, aiming to contribute to genetic counseling practices. Clinical data was collected from a family affected by neuroblastoma, and peripheral blood DNA samples were obtained from all family members. A combination of whole-exome sequencing and Sanger sequencing was utilized to detect potential gene mutations. Proband 1 and her sister (Proband 2) were diagnosed with neuroblastoma, while their parents and siblings were unaffected. The analysis revealed a novel missense mutation, c.422G > A (p.Arg141Gln), in the PHOX2B gene, which was inherited from the mother. Notably, this mutation represents a previously unreported variant within the PHOX2B gene. Detecting the missense mutation c.422G > A (p.Arg141Gln) in the PHOX2B gene implies its potential pathogenic role within this neuroblastoma family. This finding widens the range of mutations observed in the PHOX2B gene and has important implications for early neuroblastoma diagnosis within this family.

15.
Transl Oncol ; 45: 101967, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38653100

RESUMEN

BACKGROUND: Persistent pancreaticobiliary reflux (PBR) is associated with a high risk of biliary malignancy. This study aimed to evaluate the proportion of PBR in biliary tract diseases and mechanisms by which PBR promoted cholangiocarcinoma progression. METHODS: Overall 227 consecutive patients with primary biliary tract disease participated in this study. The amylase levels in the collected bile were analyzed. The mechanisms underlying the effect of high-amylase bile on bile duct epithelial and cholangiocarcinoma cells progression were analyzed. The source of interleukin-8 (IL-8) and its effects on the biological functions of cholangiocarcinoma cells were investigated. RESULTS: The bile amylase levels in 148 of 227 patients were higher than the upper serum amylase limit of 135 IU/L. PBR was significantly correlated with sex, pyrexia, and serum gamma-glutamyl transferase (GGT) levels in the patient cohort. High-amylase bile-induced DNA damage and genetic differences in the transcript levels of the gallbladder mucosa and facilitated the proliferation and migration of bile duct cancer cells (HUCCT1 and QBC939 cells). The concentration of many cytokines increased in high-amylase bile. IL-8 is secreted primarily by macrophages via the mitogen-activated protein kinase pathway and partially by bile duct epithelial cells. IL-8 promotes the progression of HUCCT1 and QBC939 cells by regulating the expression of epithelial-mesenchymal transition-associated proteins and activating the phosphatidylinositol 3-kinase/nuclear factor kappa-B pathway. CONCLUSIONS: PBR is one of the primary causes of biliary disease. IL-8 secreted by macrophages or bile duct epithelial cells stimulated by high-amylase bile promotes cholangiocarcinoma progression.

16.
Mol Med Rep ; 30(6)2024 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-39364756

RESUMEN

Following the publication of this article, an interested reader drew to the authors' attention that the forward and reverse primer sequences written for GAPDH in Table I on p. 3 were incorrect. Upon requesting an explanation of these errors from the authors, they realized that these sequences had been written incorrectly in the paper: The sequence of the forward primer in Table I should have been written as 5'­CAG GAGGCATTGCTGATGAT­3', and the reverse primer should have been written as 5'­GAAGGCTGGGGCTCATTT­3'. The Editorial Office also requested seeing proof of purchase of the primers used in this study from the authors. The authors are grateful to the Editor of Molecular Medicine Reports for allowing them the opportunity to publish this corrigendum, and all the authors agree with its publication. The authors also regret the inconvenience that these mistakes have caused. [Molecular Medicine Reports 23: 245, 2021; DOI: 10.3892/mmr.2021.11884].

17.
World J Gastrointest Surg ; 16(4): 1066-1077, 2024 Apr 27.
Artículo en Inglés | MEDLINE | ID: mdl-38690040

RESUMEN

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.

18.
World J Gastrointest Surg ; 15(5): 984-991, 2023 May 27.
Artículo en Inglés | MEDLINE | ID: mdl-37342847

RESUMEN

BACKGROUND: Ganglioneuroblastoma (GNB) is a peripheral neuroblastoma (NB) with malignant degree between highly malignant NB and benign ganglioma (GN). Pathology is the gold standard of diagnosis. Although GNB is not uncommon in children, biopsy alone may lead to an inaccurate diagnosis, especially for giant tumors. However, surgical resection may be associated with significant complications. Here, we report a case of computer-assisted surgical resection of a giant GNB in a child and successful rescue of the inferior mesenteric artery. CASE SUMMARY: A 4-year-old girl was admitted to our department for a giant retroperitoneal lesion, which was considered to be an NB by her local hospital. The symptoms of the girl disappeared spontaneously without treatment. On physical examination, a mass of about 10 cm × 7 cm could be palpated in her abdomen. Ultrasonography and contrast-enhanced computed tomography performed in our hospital also showed an NB, and there was a very thick blood vessel inside the tumor. However, aspiration biopsy revealed GN. Surgical resection is the best treatment option for this giant benign tumor. For precise preoperative evaluation, three-dimensional reconstruction was performed. It was clear that the tumor was close to the abdominal aorta. The superior mesenteric vein was pushed forward, and the inferior mesenteric artery passed through the tumor. Because GN generally does not invade blood vessels, we split the tumor with a CUSA knife during the operation and found that there was indeed a straight and intact vascular sheath. Arterial pulsation was observed in the completely exposed inferior mesenteric artery. The pathologists interpreting the tissue finally diagnosed it as a mixed GNB (GNBi), which is more malignant than GN. However, both GN and GNBi usually have a good prognosis. CONCLUSION: This was a case of successful surgical resection of a giant GNB, and aspiration biopsy underestimated the pathological staging of the tumor. Preoperative three-dimensional reconstruction assisted with the radical resection of the tumor and rescue of the inferior mesenteric artery.

19.
Int J Med Robot ; 19(2): e2489, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36471636

RESUMEN

BACKGROUND: Computer-assisted Surgery system (CAS) is an effective medical imaging simulation tool, which is widely used in preoperative planning of surgery. The objective of this study is to investigate the clinical application of CAS in pediatric mediastinal tumor resection. METHODS: This retrospective study investigated 74 children who underwent mediastinal tumor resection between June 2008 and June 2022 at the pediatric surgical center of the Affiliated Hospital of Qingdao University and Qingdao Women and Children's Hospital. Preoperative chest computed tomography imaging was performed on all children. A total of 44 children (the CAS-assisted group) underwent clinical image 3D reconstruction and preoperative simulation using Hisense CAS. The control group consisted of 30 children who underwent a conventional procedure without CAS. The demographic, preoperative, and complication data were analyzed and compared between the two groups. t-test, Mann-Whitney U test, X2 test, or Fisher's exact test were used accordingly in this study during analysis. RESULTS: The median operative duration was 119.00 min in the CAS-assisted group and 140.50 min in the control group. The median intraoperative blood loss of the CAS-assisted group and the control group was 14.00 and 31.00 ml respectively. Relative to the control groups, the CAS-assisted group experienced shorter operative duration time (p = 0.041), and less intraoperative blood loss (p < 0.001). The difference in postoperative drain indwelling between the CAS-assisted group (median:4.00 days) and the control group (median:7.00 days) reached a statistical significance (p = 0.001). And the duration of hospitalization after the operation for the CAS-assisted group (median:7.00 days) was shorter than that for the control group (median:9.00 days) (p = 0.001). No significant difference could be found in the rate of blood transfusion (p = 0.258) and the incidence of postoperative complications (p = 0.719) between the two groups. CONCLUSION: Hisense CAS could effectively assist surgeons to clearly determine the anatomical site of tumors and provide accurate preoperative simulation for surgeons, so as to assist surgeons to specify effective surgical plans for patients.


Asunto(s)
Neoplasias del Mediastino , Cirugía Asistida por Computador , Humanos , Femenino , Niño , Estudios Retrospectivos , Neoplasias del Mediastino/diagnóstico por imagen , Neoplasias del Mediastino/cirugía , Pérdida de Sangre Quirúrgica , Cirugía Asistida por Computador/métodos , Hepatectomía/métodos
20.
Front Oncol ; 12: 745258, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35321432

RESUMEN

Objective: To explore a new model to predict the prognosis of liver cancer based on MRI and CT imaging data. Methods: A retrospective study of 103 patients with histologically proven hepatocellular carcinoma (HCC) was conducted. Patients were randomly divided into training (n = 73) and validation (n = 30) groups. A total of 1,217 radiomics features were extracted from regions of interest on CT and MR images of each patient. Univariate Cox regression, Spearman's correlation analysis, Pearson's correlation analysis, and least absolute shrinkage and selection operator Cox analysis were used for feature selection in the training set, multivariate Cox proportional risk models were established to predict disease-free survival (DFS) and overall survival (OS), and the models were validated using validation cohort data. Multimodal radiomics scores, integrating CT and MRI data, were applied, together with clinical risk factors, to construct nomograms for individualized survival assessment, and calibration curves were used to evaluate model consistency. Harrell's concordance index (C-index) values were calculated to evaluate the prediction performance of the models. Results: The radiomics score established using CT and MR data was an independent predictor of prognosis (DFS and OS) in patients with HCC (p < 0.05). Prediction models illustrated by nomograms for predicting prognosis in liver cancer were established. Integrated CT and MRI and clinical multimodal data had the best predictive performance in the training and validation cohorts for both DFS [(C-index (95% CI): 0.858 (0.811-0.905) and 0.704 (0.563-0.845), respectively)] and OS [C-index (95% CI): 0.893 (0.846-0.940) and 0.738 (0.575-0.901), respectively]. The calibration curve showed that the multimodal radiomics model provides greater clinical benefits. Conclusion: Multimodal (MRI/CT) radiomics models can serve as effective visual tools for predicting prognosis in patients with liver cancer. This approach has great potential to improve treatment decisions when applied for preoperative prediction in patients with HCC.

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