Recent scientific data recognize the B7-H3 checkpoint molecule as a potential target for immunotherapy of pediatric solid tumors (PSTs). B7-H3 is highly expressed in extracranial PSTs such as neuroblastoma, rhabdomyosarcoma, nephroblastoma, osteosarcoma, and Ewing sarcoma, whereas its expression is absent or very low in normal tissues and organs. The influence of B7-H3 on the biological behavior of malignant solid neoplasms of childhood is expressed through different molecular mechanisms, including stimulation of immune evasion and tumor invasion, and cell-cycle disruption. It has been shown that B7-H3 knockdown decreased tumor cell proliferation and migration, suppressed tumor growth, and enhanced anti-tumor immune response in some pediatric solid cancers. Antibody-drug conjugates targeting B7-H3 exhibited profound anti-tumor effects against preclinical models of pediatric solid malignancies. Moreover, B7-H3-targeting chimeric antigen receptor (CAR)-T cells demonstrated significant in vivo activity against different xenograft models of neuroblastoma, Ewing sarcoma, and osteosarcoma. Finally, clinical studies demonstrated the potent anti-tumor activity of B7-H3-targeting antibody-radioimmunoconjugates in metastatic neuroblastoma. This review summarizes the established data from various PST-related studies, including in vitro, in vivo, and clinical research, and explains all the benefits and potential obstacles of targeting B7-H3 by novel immunotherapeutic agents designed to treat malignant extracranial solid tumors of childhood.

Background/Aim. Acute appendicitis (AA) is the most frequent emergency and appendectomy is the most frequent abdominal operation in the pediatric surgery. Diagnosis of AA in children is more difficult due to a lack of cooperation and poor clinical history data, leading to significant number of misdiagnostic cases. Our aim was to explore whether neutrophil to lymphocyte ratio (NLR) may be useful in diagnosis and follow-up of AA in children. Methods. Prospective investigation of NLR values in 129 consecutive patients admitted to the Mother and Child Healthcare Institute of Serbia and referred for surgery due to AA was performed. According to the pathohistological findings, patients were divided into 3 groups: normal/early, uncomplicated (phlegmonous) and complicated (gangrenous or/and perforated) AA. Laboratory analysis was done preoperativly and on the 1st and the 3rd postoperative days. Results. Statistically significant differences of NLR values were found in the different time points in total of patients and per groups. Some statistically significant differences of NLR values among histopathological groups were recorded. Investigations of correlation of NLR and other laboratory and clinical parameters showed strong positive correlation between NLR and C-reactive protein postoperatively and between NLR and Pediatric Appendicitis Score (PAS) preoperatively. Strong negative correlation with preoperative symptoms duration (PSD) were also present. Optimal cutoff NLR value between negative and positive appendectomies was 6.14. Conclusion. NLR provides well monitoring of progression of AA in children and, its cutoff values may help in distinguishing the phases of AA. Because of that, NLR should be used in diagnosis of AA in children.

Background: Cleft lip and palate are the most common congenital craniofacial anomalies. There are several classifications of cleft lip and palate that have been proposed over the years; however, only a few have found clinical application. We presented classification of a cleft lip and palate that is comprehensive and uniform, and it can precisely define the vast majority of the clefts. Methods: Five hundred and twenty-one consecutive patients underwent their primary surgical procedure for cleft lip and palate deformities. These intraoperative diagnoses were translated into abbreviated diagnoses based on the classification that we presented. In this paper, we compared the accuracy of preoperative diagnoses with that of intraoperative diagnoses according to the well-known classification of cleft lip and palate that we presented. Results: A total of 343 cleft lip surgeries and 413 palatoplasty surgeries were performed on 521 patients. The isolated cleft palate was the most common type of cleft in our group of patients. Associated anomalies occurred in 16% of patients with clefts of the lip and palate. In our study, 18% of patients had an inaccurate preoperative diagnosis. The intraoperative diagnoses were more accurate compared to preoperative diagnoses. The simplicity and comprehensibility of the proposed classification were shown through the comparison of preoperative, intraoperative, and diagnostic information presented by this classification. Conclusion: The classification of cleft lip and palate that we presented is clear, easy to understand, and can be a successful tool in studying the epidemiology of clefts.

Introduction/Objective: Extrarenal Wilms' tumor (ERWT) comprises 0.5% to 1% of all nephroblastoma cases. The most common locations of ERWT are the retroperitoneum, inguinal, lumbosacral and pelvic region, female genital organs, mediastinum and chest wall, spermatic cord and paratesticular region. ERWT most likely originates from the ectopic nephrogenic rest that undergoes a malignant transformation. The exclusion of primary renal tumor is necessary to establish the diagnosis of ERWT. Patient Review: A 15-month-old male was operated on because of a left-sided inguinal mass that clinically resembled a funicular hydrocele. The surgical exploration of the inguinal canal revealed a solid tumor located in the area of the external inguinal ring, measuring around 2cm in diameter, with no ingvinal hernia present. Complete surgical resection of the mass was performed. The histological structure of the tumor corresponded to non-anaplastic nephroblastoma (Wilms tumor), mixed type. After abdominal CT scan primary renal tumor was excluded and the diagnosis of ERWT was made. Because of microscopic tumor rests on the resection margin, the patient was treated according to the high-risk SIOP Wilms tumor protocol. The patient made a full recovery. During the 10-year follow-up, the boy has remained disease-free. Conclusion: ERWT is mostly diagnosed after the surgical removal of the specimen. The clinical resemblance of the ERWT located in the inguinal region to common benign inguinal conditions in children may lead to the omission of detailed preoperative work-up. Most children with ERWT in the inguinal region have an excellent prognosis, although most of them require adjuvant chemotherapy and/or radiotherapy after surgery.

Cleft lip and palate are the most common congenital craniofacial anomalies. The etiology of this condition is unclear. The overall incidence of cleft lip and palate is 1/700. Prenatal diagnosis of cleft lip by 3D ultrasound is possible in more than 70% of cases, while it is less effective for cleft palate. There are several classifications of cleft lip and palate that were proposed over the years, however, only few have found clinical application, with none of them generally accepted as most effective and understandable. Allori et al. presented classification of a cleft lip and palate which is comprehensive and uniform, and it can precisely define the vast majority of the clefts, thus enabling easier communication between the health workers during their everyday practice. In this paper, we compared the accuracy of preoperative diagnoses compared to intraoperative diagnoses according to Kernahan and Stark classification from 1958, and these diagnoses were converted according to the Allori et al. classification of cleft lip and palate. Classification of cleft lip and palate presented by Allori et al. is clear, easy to understand, and it can be successful tool in studying the epidemiology of clefts.