Objective:To analyse the application of indocyanine green (ICG) fluorescence contrast technique for resection of hepatoblastoma in children and to study its value in the application.Methods:The data of 13 children with pediatric hepatoblastoma who underwent open liver resection by using the ICG real-time image guided system were collected from June 2020 to October 2021 at the Children's Hospital of Nanjing Medical University. There were 10 males and 3 females. Their ages ranged from 5 days to 63 months, with a median of 22 months. The characteristics of the ICG imaging, surgical excision and postoperative pathology were analysed.Results:The tumors showed bright fluorescence in 13 patients. The border between the tumor and normal liver tissues was clear in 12 patients, and there was no difference between the background fluorescence of the liver and fluorescence of the tumor in one patient. Regular hepatic resection was performed in 6 patients and irregular hepatic resection in 7 patients under ICG fluorescence navigation. In one patient, the left half of the liver was resected, and there was no fluorescence on the transected surface of the liver remnant. The surface of the liver remnant was scattered with fluorescence showing multiple tumors, which were then locally enucleated. Twelve specimens which were fluorescently visualized were bisected with 8 specimens showing partial fluorescence visualization of the nodules within the tumor + annular visualization at the margins, and 4 patients showing annular fluorescence visualization at the tumor margins only. In 12 children, histopathological examination of the tissues at the site with circumferential imaging showed distorted, vacuolated and densely arranged hepatocytes resembling pseudo-envelope changes due to compression, but there were no tumor cells.Conclusion:The ICG fluorescence contrast technique was very effective for resection of childhood hepatoblastoma, and the ring fluorescence contrast of the tumor edge indicated the border for tumor resection. The ICG contrast plays an important role in navigating tumor resection, especially in patients with multiple tumor nodules.

Objective:To investigate the application value of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis and treatment of pancreaticobiliary maljunction (PBM) in children.Methods:The clinical data of 77 PBM children who underwent ERCP in General Surgery Department of Children's Hospital affiliated to Nanjing Medical University between January 2018 and December 2021 were retrospectively evaluated. Clinical characteristics, classification and post-operative nursing interventions were summarized, and vital signs, changes of biochemical markers and the occurrence of postoperative complications were compared and recorded.Results:77 patients were classified according to Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM), including 34 patients with type A, 18 patients with type B, 21 patients with type C, and 4 patients with type D. There were 68 patients with congenital bile duct dilation and 9 patients without congenital bile duct dialtion. 92 ERCP procedures were performed under general anesthesia, and 91 cases were successful with a success rate of 98.91%. Among these cases, including 7 cases of endoscopic sphincterotomy, 28 cases of endoscopic balloon dilation of the nipple, 22 cases of endoscopic probe dilation, 22 cases of endoscopic stone removal by balloon or basket, 35 cases of endoscopic retrograde biliary stent drainage, 4 cases of endoscopic pancreatic duct drainage, 18 cases of endoscopic nasobiliary drainage, 2 cases of endoscopic nasobiliary drainage, and 14 cases of biliary stent removal. In 77 children with PBM, body temperature, FLACC score, and laboratory-related biochemical indexes including direct bilirubin, serum amylase, ALT, AST and CGT decreased significantly after ERCP, and all the differences were statistically significant (all P value <0.001). The incidence of postoperative complications was 15.38%(14/91), including hyperamylasemia in 9 cases (9.89%) and abdominal pain in 5 cases (5.49%). Conclusions:ERCP is safe and effective in the treatment of abnormal confluence of pancreatic duct in children.

Objective:To investigate the clinical characteristics and prognostic factors of extracranial malignant rhabdoid tumors (eMRTs) in children.Methods:In this retrospective case series study, a retrospective analysis was conducted on clinical data of 21 eMRT patients admitted to Children′s Hospital of Nanjing Medical University from April 2018 to January 2023 and followed up until October 30, 2023.Patients were grouped according to their gender, age, tumor origin site, clinical staging, initial lactate dehydrogenase (LDH) level, extent of tumor resection, chemotherapy regimen, and radiotherapy.The Kaplan-Meier method was used to calculate the 2-year progression-free survival rate (PFS) and overall survival rate (OS) of the patients, and the Cox regression model was used to analyze the prognostic factors.Results:Among the 21 patients with eMRTs, there were 7 males and 14 females, with the age of onset of 24 (3-138) months.Immunohistochemistry showed that all tumor tissues of the patients did not secrete integrase interactor 1 (INI-1).Among them, 13 cases originated from the kidney, and 8 cases originated from extrarenal non-central sites.At the time of diagnosis, there were 4 cases in clinical stages Ⅰ-Ⅱ, 17 cases in stage Ⅲ-Ⅳ.Thirteen patients underwent complete tumor resection surgery, 7 underwent partial resection, and 1 only underwent biopsy.Among the 13 cases of renal rhabdoid tumors, 8 cases were treated with the AVDC (Epirubicin, Vincristine, Actinomycin D, Cyclophosphamide)/ICE (Ifosfamide, Carboplatin, Etoposide) regimen, and 5 cases were treated with the protocol for nephroblastoma; among the 8 cases of extrarenal non-central rhabdoid tumors, 5 cases were treated with the AVDC/ICE regimen, and 3 cases were treated with the commonly used protocol for soft tissue sarcoma.Thirteen patients received radiotherapy.One patient received consolidation therapy with autologous stem cell transplantation following chemotherapy and radiotherapy.As of October 2023, there were 14 survivors and 7 deaths.The overall 2-year PFS and OS were 56%(95% CI: 35.7%-88.5%) and 62%(95% CI: 43.2%-89.4%), respectively.Among the patients who received the AVDC/ICE alternating chemotherapy regimen, the 2-year PFS and OS were 73%(95% CI: 47.0%-100.0%) and 79% (95% CI: 56.4%-100.0%), respectively.Univariate Cox regression analysis showed that complete tumor resection, the AVDC/ICE alternating chemotherapy, and radiotherapy were associated with a better prognosis in children (all P≤0.05).Multivariate Cox regression analysis showed that whether to receive radiotherapy was an independent risk factor affecting the overall survival in children. Conclusions:eMRTs are more common in infants and young children, with high malignancy and invasiveness.There is currently no standard treatment.Complete tumor resection combined with the AVDC/ICE alternating chemotherapy and radiotherapy may improve the prognosis of children with eMRTs.