Search Results (46)

Retinoblastoma is a success story in pediatric oncology, evolving from life-saving interventions to approaches that preserve eyes and vision while minimizing complications. Initially managed with enucleation and radiotherapy, treatment now emphasizes eye preservation through chemotherapy as the cornerstone therapy. Various chemotherapy delivery methods—including intravenous (IVC), intraarterial (IAC), intravitreal, intracameral, and periocular—offer flexibility in treatment. Studies show nearly 100% eye salvage rates for groups A–C. For advanced cases (groups D and E), IAC has achieved outcomes that were not possible before. Intravitreal injections, when performed safely, may help avoid enucleation and radiotherapy in advanced cases, preserving vision, even in complex scenarios, with vitreous seeding. Each strategy may be tailored to tumor and patient characteristics that may help optimize outcomes. Recent innovations like liquid biopsy, prenatal diagnosis, prognostic biomarkers, and new surgical methods, such as tylectomy and chemoplaque, are paving the way for more personalized care. While advanced extraocular or metastatic retinoblastoma remains challenging, these advancements underscore a shift towards better outcomes and individualized management. The future holds promise for refining treatment strategies to maximize eye and vision preservation while ensuring patient survival. Full article

Background: Malnutrition is increasingly prevalent due to rising life expectancy, oncological cases, and chronic diseases. Early detection is crucial for rehabilitation, complication prevention, and cost reduction. However, nutritional support is often suboptimal. This study aimed to determine malnutrition prevalence and nutritional support status within our hospital. Methods: A point prevalence study was conducted in adult inpatient clinics (excluding pediatrics, infectious diseases, and intensive care) by a 12-member team following ethical approval. NRS-2002 scores, arm/calf circumferences, BMI, and laboratory data (albumin, leukocytes, lymphocytes, neutrophils, hemoglobin, CRP, creatinine) were assessed. Enteral and parenteral nutrition treatments were recorded. Patients with NRS-2002 scores ≥ 3 were classified as at risk of malnutrition. Results: Among 178 patients, 24.7% were at risk of malnutrition. Surgical clinics had a higher malnutrition risk (32.3%) than internal medicine clinics (20.3%). Only 27.1% of at-risk patients received nutritional support (surgical: 19%, internal medicine: 44%). Patients at risk of malnutrition exhibited significantly lower arm circumference (p: 0.000), calf circumference (p: 0.002), lymphocyte counts (p: 0.000), hemoglobin (p: 0.018), albumin (p: 0.001), and BMI (p: 0.038), as well as significantly higher age (p: 0.000) and CRP levels (p: 0.000). Conclusions: Nutritional support remains inadequate despite increased attention to malnutrition. Intensified nutrition education, particularly in surgical inpatient clinics, is needed to improve patient rehabilitation and outcomes. Full article

Background: 5-Aminolevulinic acid (5-ALA) serves as a precursor in the heme biosynthesis pathway, resulting in the selective accumulation of protoporphyrin IX (PpIX) within glioma cells. This property facilitates fluorescence-guided resection (FGR) in high-grade gliomas (HGGs), enhancing surgical precision and oncological results. Nonetheless, its clinical implementation is restricted by factors such as accessibility, cost, and technical limitations. Methods: A systematic review of PubMed literature (2019–2024) was conducted to assess the efficacy of 5-ALA in HGG surgery compared to conventional white light microscopy. Studies focusing on non-neurosurgical applications, pediatric populations, and non-HGG indications were excluded. Results: Nineteen articles met the criteria. Recent studies indicate that 5-ALA-guided resection significantly enhances gross total resection (GTR) rates compared to white light surgery (75.4% vs. 54.3%, p < 0.001). Patients receiving 5-ALA-assisted resection exhibit enhanced progression-free survival (PFS) at 6 months (median 8.1 months compared to 5.4 months, p = 0.002) and overall survival (OS) (median 15.2 months versus 12.3 months, p = 0.008). The necessity for specialized neurosurgical microscopes equipped with blue light filters restricts accessibility, especially in low-resource environments. Recent advancements in fluorescence-enhancing technologies, particularly loupe-based systems, have demonstrated increases in fluorescence intensity by up to tenfold through direct emission. Sodium fluorescein, originally designed for ophthalmological use, has been adapted for enhancing contrast in intracranial tumors; however, its non-specific binding to serum albumin restricts its accuracy in glioma resection. Conclusions: Recent publications demonstrate that 5-ALA fluorescence-guided surgery significantly improves gross total resection rates and survival outcomes in patients with high-grade gliomas. Although it offers clinical advantages, cost and equipment constraints continue to pose substantial obstacles to broad implementation. Additional research is required to enhance fluorescence-guided techniques and increase accessibility in resource-constrained environments. Full article

Background/Objectives: In bilateral nephroblastoma and high-risk neuroblastoma in children with extensive tumor involvement of the renal vessels or pedicle, complete tumor resection with preservation of healthy renal tissue is not feasible with in situ nephron-sparing surgery or vascular replacement. The aim of this study was to present our experience with ante situ tumor resection and renal autotransplantation (RATX) in these children. Methods: A retrospective study of children with bilateral nephroblastoma and high-risk neuroblastoma who underwent tumor resection and RATX at an international referral center for pediatric surgical oncology between 2006 and 2024 was performed. RATX was performed by transection of renal vessels, ante situ mobilization, and perfusion of the kidney with Bretschneider’s solution. Tumor resection was performed on a bloodless kidney under hypothermia. Results: Ante situ tumor resection and RATX were performed at a median age of 36 months (range 13–62) in 4 children with bilateral nephroblastoma and 4 children with high-risk neuroblastoma. Complete tumor resection was achieved in all patients. One patient with neuroblastoma died of sepsis after 14 days. The 7 surviving patients showed no evidence of disease and normal to slightly decreased glomerulofiltration rates at a median follow-up of 20 months (range 3–155). Limitations include the retrospective design, small sample size, and heterogeneity of the study population due to very rare indication. Conclusions: Ante situ tumor resection and RATX is a feasible surgical approach for children with multifocal bilateral nephroblastoma or high-risk neuroblastoma who are ineligible for in situ nephron-sparing surgery or vascular reconstruction. Full article

Neuropathic pain (NP) is a common complication associated with some types of childhood cancer, mainly due to nerve compression, chronic post-surgical pain, chemotherapy, and radiotherapy. NP is usually less responsive to traditional analgesics, and there is generally a lack of evidence on its management in cancer patients, leading to recommendations often based on clinical trials conducted on other forms of non-malignant NP. In pediatric oncology, managing NP is still very challenging for physicians. Different factors contribute to increasing the risk of undertreatment: (a) children may be unable to describe the quality of pain; therefore, the risk for NP to be underestimated or remain unrecognized; (b) specific tools to diagnose NP have not been validated in children; (c) there is a lack of randomized clinical trials involving children, with most evidence being based on case series and case reports; (d) most drugs used for adult patients are not approved for childhood cancers, and drug regulation varies among different countries; (e) recommendations for pediatric pain treatment are still not available. In this paper, a multidisciplinary team will review the current literature regarding children with cancer-related NP to define the best possible diagnostic strategies (e.g., clinical and instrumental tests) and propose a therapeutic care pathway, including both non-pharmacological and pharmacological approaches, which could help pediatricians, oncologists, neurologists, and pain therapists in designing the most effective multidisciplinary approach. Full article

Background: Advancements in artificial intelligence (AI) and machine learning (ML) are set to revolutionize healthcare, particularly in fields like endoscopic surgery that heavily rely on digital imaging. However, to effectively integrate these technologies and drive future innovations, pediatric surgeons need specialized AI/ML skills. This survey evaluated the current level of readiness and educational needs regarding AI/ML among members of the European Society of Pediatric Endoscopic Surgeons (ESPES). Methods: A structured survey was distributed via LimeSurvey to ESPES members via email before and during the 2024 Annual Conference. Responses were collected over four weeks with voluntary, anonymous participation. Quantitative data were analyzed using descriptive statistics. Results: A total of 125 responses were received. Two-thirds (65%) of respondents rated their AI/ML understanding as basic, with only 6% reporting advanced knowledge. Most respondents (86%) had no formal AI/ML training. Some respondents (31%) used AI/ML tools in their practice, mainly for diagnostic imaging, surgical planning, and predictive analytics; 42% of the respondents used these tools weekly. The majority (95%) expressed interest in further AI/ML training, preferring online courses, workshops, and hands-on sessions. Concerns about AI/ML in pediatric surgery were high (85%), especially regarding data bias (98%). Half of respondents (51%) expect AI/ML to play a significant role in advancing robotic surgery, oncology, and minimally invasive techniques. A strong majority (84%) felt that the ESPES should lead AI education in pediatric surgery. Conclusions: This survey presents the ESPES with a unique opportunity to develop a competency map of its membership’s AI/ML skills and develop targeted educational programs, thus positioning the society to take the lead in AI education and the advancement of AI solutions in pediatric endosurgery. Full article

Background and Aim: Ex vivo fluorescence confocal microscopy (FCM) systems are innovative optical imaging tools that create virtual high-resolution histological images without any standard tissue processing, either freezing or fixing in formalin and embedding in paraffin. These systems have opened an era that would revolutionize pathological examination by providing rapid, real-time assessments across various pathology subspecialties, potentially replacing conventional methods that are tissue- and time-consuming. This study aimed to present the first utilization of FCM in pediatric surgical oncology, focusing on assessing the benefits, particularly in facilitating rapid and accurate diagnosis. Methods: This preliminary study comprised five consecutive patients undergoing surgical biopsy for disease characterization and surgical strategy selection. After biopsy, tissue samples were prepared and analyzed using FCM without sectioning. A pathologist who evaluated macroscopic and microscopic images, once obtained remotely, could promptly indicate any interventions that require timeliness. Samples were then evaluated with conventional methods. Results: All five lesions were deemed suitable for evaluation. Preliminary diagnoses utilizing FCM included atypical Spitz nevus (1), Wilm’s tumor (1), lymph node reactive hyperplasia (1), malignant germ cell tumor of the testis (1), and Hodgkin’s lymphoma (1). Final histopathological analyses revealed atypical Spitz nevus (1), Wilm’s tumor (1), hyperplastic lymphadenopathy with a prevalent marginal pattern (1), mixed nonseminomatous malignant germinal neoplasm consisting of embryonal carcinoma (90%) and yolk sac tumor (10%), and Hodgkin’s lymphoma nodular sclerosis variant (1). In the case of diagnosis of atypical Spitz nevus, the widening of the resection margins was performed in the same surgery. In the case of testicular neoplasm, radical orchiectomy was performed. A high level of agreement between FCM evaluation and definitive histological examination was observed for all parameters evaluated. Conclusions: FCM represents a significant advancement in pathological imaging technology, offering potential benefits in enhancing traditional tissue processing methods. This preliminary report marks the first application of FCM in pediatric surgical oncology. Our findings underscore the promising role of FCM as an adjunctive tool in pediatric oncology, facilitating prompt diagnosis and treatment initiation. Full article

Introduction: High-risk neuroblastoma, particularly in the relapse/refractory (R/R) setting, poses unique challenges to obtaining the representative-quality tissue that is mostly required for molecular analysis. This study explores the use of 123I-MIBG radioguided surgery to access complex locations of MIBG-positive neuroblastoma as a tool to overcome the difficulties associated with repeated surgeries in these patients. Methods: This study is a retrospective review of all patients with R/R neuroblastoma and MIBG-uptaking lesions who underwent radioguided surgery between February 2020 and 2023 at SJD Barcelona Children’s Hospital. The Europrobe 3.2 gamma probe was used to identify neuroblastoma tissue in the operating room. Results: Ten patients were identified. Radioguided surgery was useful in all patients. One patient with previous multiple operations developed an entero-cutaneous fistula with posterior full recovery. Mean surgical time was 111.7 min. The gamma probe identified 100% of neuroblastoma lesions which were all completely removed (123I-MIBG-SPECT/CT negative post-surgery). Pathology and molecular studies could be successfully performed in all samples. Conclusions: 123I-MIBG radioguided surgery proved effective in obtaining viable tissue from difficult-to-access sites in high-risk relapsed neuroblastoma. Full article

We analyzed data on pediatric invasive fungal diseases of the central nervous system (CNS-IFDs) reported by five of a total of eight Pediatric Hematology-Oncology Departments in Greece for 16 years (2007–2022). A total of twelve patients (11 boys, median age: 9.5 years, range: 2–16) were reported suffering from CNS-IFDs. The underlying malignancy was acute lymphoblastic leukemia in 9/12 and acute myeloid leukemia, Ewing sarcoma, and rhabdomyosarcoma in one each. Eleven patients presented with CNS-related symptoms (i.e., seizures, headache, cerebral palsy, ataxia, hallucination, seizures, blurred vision, amaurosis). All patients had pathological MRI findings. Multifocal fungal disease was observed in 6/12 patients. Nine proven and three probable CNS-IFD cases were diagnosed. Causative pathogens in proven cases were Aspergillus spp. and Candida albicans (n = 2 each), Mucor spp., Rhizopus arrhizus, Absidia spp., Fusarium oxysporum and Cryptococcus neoformans (n = 1 each). Causative pathogens in probable cases were Aspergillus spp. (n = 2) and Candida spp. (n = 1). All patients received appropriate antifungal therapy (median duration: 69.5 days, range 19–364). Two patients underwent additional surgical treatment. Six patients were admitted to the Intensive Care Unit due to complications. Three patients (25%) died, two due to IFD and one due to an underlying disease. Early recognition and prompt intervention of CNS-IFDs may rescue the patients and improve overall survival. Full article

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms that primarily affect adults, with pediatric cases constituting only 0.5–2.7% of the total. Pediatric GISTs present unique clinical, genetic, and pathological features that distinguish them from adult cases. This literature review aims to elucidate these differences, emphasizing diagnostic and therapeutic challenges. We discuss the resistance of pediatric GISTs to conventional chemotherapy and highlight the importance of surgical intervention, especially in emergency situations involving intra-abdominal bleeding. The review also explores the molecular characteristics of pediatric GISTs, including rare mutations such as quadruple-negative wild-type GIST with an FGF3 gene gain mutation. To illustrate these points, we conclude with a case from our clinic involving a 15-year-old female with multiple CD117-positive gastric GISTs and a quadruple-negative wild-type genetic profile who required urgent surgical intervention following a failed tumor embolization. This case underscores the critical need for early diagnosis and individualized therapeutic strategies combining oncologic and surgical care to improve outcomes in pediatric GIST patients. Full article

Wilms tumor (WT) is the most common kidney tumor in pediatric patients. Intravascular extension of WT above the level of the renal veins is a rare manifestation that complicates surgical management. Patients with intravascular extension are frequently asymptomatic at diagnosis, and tumor thrombus extension is usually diagnosed by imaging. Neoadjuvant chemotherapy is indicated for thrombus extension above the level of the hepatic veins and often leads to thrombus regression, obviating the need for cardiopulmonary bypass in cases of cardiac thrombus at diagnosis. In cases of tumor extension to the retrohepatic cava, neoadjuvant therapy is not strictly indicated, but it may facilitate the regression of tumor thrombi, making resection safer. Hepatic vascular isolation and cardiopulmonary bypass increase the risk of bleeding and other complications when utilized for tumor thrombectomy. Fortunately, WT patients with vena caval with or with intracardiac extension have similar overall and event-free survival when compared to patients with WT without intravascular extension when thrombectomy is successfully performed. Still, patients with metastatic disease at presentation or unfavorable histology suffer relatively poor outcomes. Dedicated pediatric surgical oncology and pediatric cardiothoracic surgery teams, in conjunction with multimodal therapy directed by a multidisciplinary team, are preferred for optimized outcomes in this patient population. Full article

Introduction: Pediatric oncology patients use totally implantable venous access devices (TIVADs) to enable central venous access. Anxiety, pain and/or discomfort are common despite anesthesia. Virtual reality (VR) is a non-pharmacological approach that may reduce pain and anxiety in these patients. We aimed to assess the use of VR for reducing anxiety/pain in patients with TIVADs while facilitating the task of healthcare providers when accessing a TIVAD. Methods: patients 4–18 years old with a TIVAD were prospectively randomized to an intervention group (IG) or a control group (CG). In the IG, VR goggles (Oculus Quest 2, Meta Platforms^®, Menlo Park, CA, USA) were used displaying a relaxing video in the Raja Ampat environment (Ecosphere app from Phoria^®) while the TIVAD was accessed. The CG was managed as per standard of care. Satisfaction and pain were measured by FPS-R and STAIC scales, respectively. Results: this is the report of a prospective, randomized (60 per group)—unblinded—, single institution study of 120 pediatric oncology patients enrolled from January to April 2022. Median ages for IG and CG were 9.22 and 10.52 years, respectively. Satisfaction was higher in the IG (4.80) compared to the CG (3.92), p ≤ 0.0001. Regarding pain, mean FPS-R scores were 1.79 for the CG and 0.83 for the IG. Significantly different scores were found in the 12 to 18 years group, p ≤ 0.05. The healthcare professionals index of satisfaction was high (4.50 mean Likert score) for the IG compared to accessing the TIVAD without VR (3.73 mean Likert score). Conclusion: The use of VR helped reduce pain and/or discomfort in pediatric oncology patients, mainly in the older age group as they can better interact with VR. Healthcare providers were satisfied with the help of VR for TIVAD management. Full article

Cancer poses significant emotional challenges for children and adolescents, despite improvements in survival rates due to new therapies. However, there is growing concern about the long-term effects, including fertility issues. This review examines recent advancements and future directions in fertility preservation within a pediatric population subjected to oncological therapies. Worldwide, there is variability in the availability of fertility preservation methods, influenced by factors like development status and governmental support. The decision to pursue preservation depends on the risk of gonadotoxicity, alongside factors such as diagnosis, treatment, clinical status, and prognosis. Currently, options for preserving fertility in prepubertal boys are limited compared to girls, who increasingly have access to ovarian tissue preservation. Adolescents and adults have more options available, but ethical considerations remain complex and diverse. Full article

Melatonin, an endogenous neurohormone produced by the pineal gland, has received increased interest due to its potential anti-cancer properties. Apart from its well-known role in the sleep–wake cycle, extensive scientific evidence has shown its role in various physiological and pathological processes, such as inflammation. Additionally, melatonin has demonstrated promising potential as an anti-cancer agent as its function includes inhibition of tumorigenesis, induction of apoptosis, and regulation of anti-tumor immune response. Although a precise pathophysiological mechanism is yet to be established, several pathways related to the regulation of cell cycle progression, DNA repair mechanisms, and antioxidant activity have been implicated in the anti-neoplastic potential of melatonin. In the current manuscript, we focus on the potential anti-cancer properties of melatonin and its use in treating and managing pediatric osteosarcoma. This aggressive bone tumor primarily affects children and adolescents and is treated mainly by surgical and radio-oncological interventions, which has improved survival rates among affected individuals. Significant disadvantages to these interventions include disease recurrence, therapy-related toxicity, and severe/debilitating side effects that the patients have to endure, significantly affecting their quality of life. Melatonin has therapeutic effects when used for treating osteosarcoma, attributed to its ability to halt cancer cell proliferation and trigger apoptotic cell death, thereby enhancing chemotherapeutic efficacy. Furthermore, the antioxidative function of melatonin alleviates harmful side effects of chemotherapy-induced oxidative damage, aiding in decreasing therapeutic toxicities. The review concisely explains the many mechanisms by which melatonin targets osteosarcoma, as evidenced by significant results from several in vitro and animal models. Nevertheless, if further explored, human trials remain a challenge that could shed light and support its utility as an adjunctive therapeutic modality for treating osteosarcoma. Full article

Solid tumors of the cervicothoracic junction, the posterior mediastinum, or bilateral dorsal thoracic tumors represent a challenge in pediatric surgical oncology. The aim of this study was to evaluate trap-door thoracotomy and clamshell thoracotomy as surgical approaches. A single-center retrospective study of children with solid tumors in these specific localizations was performed. From 2015 to 2023, 26 children (17 girls; 9 boys) were treated at a median age of 54 months (range 8–229). Tumor resection was performed for neuroblastoma (n = 11); metastatic disease (n = 7); malignant rhabdoid tumor (n = 4); Ewing sarcoma (n = 1); inflammatory myofibroblastic tumor (n = 1); rhabdomyosarcoma (n = 1); and neurofibroma (n = 1). The surgical goal of macroscopic complete excision was achieved in all of the 14 children who underwent trap-door thoracotomy and in 11 of the 12 children who underwent clamshell thoracotomy. There were no major complications. At a median follow-up of 8 months (range 0–60), the disease was under local control or in complete remission in 66.7% of the children. In conclusion, surgical resection of solid tumors of the cervicothoracic junction in children can be performed safely and successfully with trap-door thoracotomy and with clamshell thoracotomy for posterior mediastinal or bilateral dorsal thoracic tumors. Full article