Low-flow vascular malformations (LFVMs) are congenital vascular anomalies with heterogeneous clinical behavior and variable treatment response. This retrospective study described the clinical characteristics, treatment patterns, and modality-specific outcomes of children aged 0-18 years with LFVM evaluated at Hacettepe University between January 1999 and June 2020. Lesions were classified as capillary, venous, lymphatic, or combined LFVMs, and demographic, clinical, imaging, treatment, follow-up, and adverse event data were reviewed. Radiologic/clinical and symptomatic responses were assessed for sirolimus, image-guided sclerotherapy, and surgery. Among the patients, lesions were combined in 94 (40.5%), lymphatic in 80 (34.5%), and venous in 58 (25.0%). The mean age at diagnosis was 54.6 ± 55.2 months, the mean diagnostic delay was 37.6 ± 48.1 months, and the head and neck was the most frequent primary location (44.4%). Mean follow-up was 50.1 ± 42.3 months. Sirolimus was administered to 118 patients and achieved radiologic/clinical response in 55.1% and symptomatic improvement in 75.4%, with the highest symptomatic improvement observed in lymphatic malformations (84.2%). After sirolimus discontinuation, 19 patients experienced radiologic/clinical progression or symptom worsening. Sclerotherapy (n = 74) achieved radiologic/clinical and symptomatic response rates of 67.6% and 79.7%, respectively, whereas surgery (n = 54) achieved rates of 79.6% and 61.1%, respectively. Progressive disease at the last follow-up was rare (1.3%). Overall, multimodal therapy provided substantial disease control in this long-term pediatric LFVM cohort. Sirolimus primarily improved symptoms, particularly in lymphatic and combined lesions, while sclerotherapy and surgery were effective when selected according to lesion phenotype and treatment goals.

Abstract Background Infantile myofibromatosis (IM) is a rare benign myofibroblastic neoplasm of infancy that usually involves the skin, bone, muscle, and soft tissue and rarely visceral organs. Questions/purposes: The aim of this study is to describe solitary IM involving bone or soft tissue in the extremities across different ages and sexes and to explore whether larger tumor size and beta-catenin (β-catenin) positivity were more frequently observed in recurrent cases. Methods This multi-center study included 15 patients diagnosed and treated with IM between January 2004 and December 2019. All patients were diagnosed with incisional biopsy before definitive surgery to rule out the risk of sarcoma. Data including age, sex, duration of complaints, time to diagnosis, size, tumor histology, surgery type, complications, recurrence, functional outcomes and follow-up were recorded. β-catenin immunoreactivity was assessed in all patients, and the findings were analyzed descriptively because of the small sample size. Results The mean age of the patients was 8.3 ± 6.4 years with male predominance. Recurrence was observed in five patients. Tumors larger than 5 cm and β-catenin positivity were more frequently observed in recurrent cases. Neurovascular involvement, documented on preoperative imaging and/or intraoperative findings, was seen in both recurrent and non-recurrent cases. Given the very small cohort, these observations are presented descriptively. Conclusion Soft tissue IM is difficult to diagnose and can be misdiagnosed as malignancy. Therefore, biopsy before definitive surgery remains crucial. In this series, recurrence was observed in a subset of solitary extremity IM cases, and recurrent cases more often showed larger tumor size and β-catenin positivity. However; Further large-scale, prospective studies are needed to draw more reliable conclusions on this subject. Level of evidence Level III, retrospective study. Clinical trial number Not applicable.

Objective: Mitotane is an effective treatment for advanced adrenocortical carcinoma (ACC). Given the limited pediatric data available, this study aims to evaluate the associations between mitotane dosage, plasma drug levels, and anthropometric measurements, as well as their potential impact on dosage requirements to optimize therapeutic outcomes in pediatric patients with ACC (pACC). Design and methods: A retrospective, international, multicenter study was conducted on pediatric ACC patients treated with mitotane across 18 centers. Mitotane serum levels were obtained from the Lysosafe Online® database or directly from the centers. Data from the cohort with plasma levels within the target range (≥14 mg/L; n = 319) were analyzed and compared to those with levels outside this range (n = 320). Results: Fifty pediatric patients (60% female) diagnosed between 2004 and 2023 were included, with a median follow-up of 34.5 months and a 10-year overall survival of 33 months. The median age at diagnosis was 8.6 years, with most tumors (84%) hormone-secreting. Among 49 patients undergoing surgery, 31 (62%) achieved R0 resection. The median treatment duration was 18 months, with a median mitotane dose of 87 mg/kg/day in patients within the target plasma level range, showing no significant difference from those outside the range. However, BMI was significantly associated with doses of plasma levels in target range (P = 0.001), as underweight (105.4 mg/kg/day) and healthy weight patients (98.4 mg/kg/day) required higher doses than overweight/obese patients (44.4 mg/kg/day). No significant differences in daily dose levels (mg/kg/day and mg/m2/day) were observed based on body weight. Conclusion: This study supports estimating mitotane dosages in pediatric ACC, emphasizing the need for close monitoring and frequent follow-ups at specialized centers due to individualized dosing and a narrow therapeutic window.

PURPOSEDisplaced individuals face significant health care access challenges, particularly for noncommunicable diseases. For these individuals, cancer control remains a severely neglected aspect of health care. This scoping review aims to synthesize and evaluate the current state of knowledge on equity within the cancer care continuum for refugee and asylum seeker populations worldwide.METHODSA systematic search of PubMed and Embase was conducted for peer-reviewed articles published between 2000 and 2024, focusing on refugee and asylum seeker populations and cancer. Eligible studies addressed at least one stage of the cancer care continuum (prevention, detection, diagnosis, treatment, end-of-life care or survivorship) and included perspectives or outcomes of patients, caregivers, or providers. Data were extracted and mapped by study setting, population, cancer continuum stage, and type of barriers or interventions identified.RESULTSOf 372 screened articles, 111 met inclusion criteria. The majority of research focused on cancer prevention, detection, and diagnosis. Common themes included low cancer awareness, reduced screening uptake, delayed diagnoses, treatment interruptions, and poor survival outcomes. Financial, social, and systemic barriers such as cost, stigma, language barriers, and inconsistent policies were frequently reported. Effective interventions included culturally tailored education, refugee-specific health clinics, use of interpreters, sex-concordant providers, and community engagement.CONCLUSIONRefugees and asylum seekers face persistent and multifaceted inequities in cancer care, shaped by both individual and systemic factors. Although some high-performing models and interventions exist, large gaps remain in research and service delivery. The findings emphasize the urgent need for an integrated strategy that incorporates the comprehensive cancer care needs of refugees and asylum seekers into national and international health policies.

Primary immunodeficiency disease (PID)/Inborn Errors of Immunity (IEI) with T-cell dysfunction is well-known for susceptibility to opportunistic/viral infections. Epstein–Barr virus-positive smooth muscle tumor (EBV-SMT) is a rare entity primarily seen in the setting of immunodeficiency, such as transplantation, HIV/AIDS, and IEI. This study aimed to characterize patients’ clinical/immunologic/genetic features with EBV-SMT and assess their association with IEI. We reviewed the medical records of patients with EBV-SMT in an outpatient immunology clinic and analyzed a total of 33 patients, including 24 identified from the literature. The study included nine patients (male/ female = 6/3). The median ages at the onset of symptoms, clinical diagnosis of PID, genetic diagnosis, and EBV-SMT diagnosis were 12 months (1–84 months), 6 years (2–15 years), 10 years (6–18 years), and 10 years (4–18 years), respectively. The parental consanguinity ratio was 7/9(78

Rhabdomyosarcoma constitutes 3% to 4% of childhood cancers, with nearly half seen in the head and neck location. We aimed to investigate the clinical features and treatment outcomes of 65 children diagnosed and treated for head and neck rhabdomyosarcoma (RMS) between 2004 and 2018. The median age was 5.8 years with a 37:28 M/F ratio. The primary location was parameningeal in 49.2%, orbital in 35.4%, and other nonparameningeal in 15.4% patients. The most common histopathologic subtype was the embryonal subtype (73.8%). The chemotherapy regimens of CDCV (cisplatin, doxorubicin, cyclophosphamide, vincristine); VAC/VAdrC (vincristine, actinomycin-D, cyclophosphamide/vincristine, doxorubicin, cyclophosphamide); PIAV (ifosfamide, cisplatin, vincristine, doxorubicin); and VDC/IE (vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide) were used depending on the years of diagnosis. The tumor location, risk grouping, and stage were found as the significant prognostic factors. The 5-year event-free survival (EFS) rate for all patients 41.2% and the overall survival (OS) rate was 59.3%. The 5-year OS rates were 85.2% and 80% in the orbital and other nonparameningeal RMS, respectively, it was 34.2% in the parameningeal RMS patients ( P =0.01). The patients with advanced stage, parameningeal disease have poor prognosis. New treatment approaches should be investigated to improve the outcomes in these groups.

Background. Non-Hodgkin lymphoma of the larynx in children is a rare condition. Diagnosis is difficult as its symptoms are usually attributed to respiratory tract infections and pubertal voice changes. Case Presentations. We report two children diagnosed with laryngeal B-cell lymphoma based on imaging and histopathological findings. We also review other pediatric cases of laryngeal lymphoma documented in the literature, detailing tumor locations, lymphoma types, stages, etiological factors, and treatment regimens of these patients. Conclusion. Diagnosis of laryngeal lymphoma is challenging. Although certain imaging features can be suggestive of the disease, a definitive diagnosis requires histopathological examination. Surgery is not required for the treatment, and chemotherapy is the main treatment approach. Early diagnosis is important.

Background. Febrile neutropenia is a common cause of hospital admissions among pediatric cancer patients. To optimize personalized approaches for hospitalization and antibiotic treatment, risk stratification has been proposed. This study aimed to explore the impact of clinical and laboratory parameters on risk stratification for patient discharge. Methods. This prospective study included pediatric lymphoma and solid tumor patients who were hospitalized due to febrile neutropenia between June 2018 and June 2019. Patient characteristics, primary oncological diagnosis and disease status, comorbid conditions, time elapsed after the last course of chemotherapy, use of granulocyte-colony stimulating factor (G-CSF) prophylaxis, presence of port catheter, infection type, fever values/duration, physical examination findings, and duration of neutropenia were collected. Laboratory investigations including complete blood counts, acute phase reactants at the onset of the episode, culture results were also recorded. Results. The study examined 142 febrile neutropenic episodes from 88 consecutive patients. The median age of the study group was 6.8 years, with 19.3% of cases being lymphoma and 80.7% having solid tumors. The median hospital stay was 7 days. Factors associated with longer hospitalization periods included a lymphoma diagnosis, presence of comorbid conditions, bone marrow involvement, and febrile neutropenic period during hospitalization. Patients presenting with fever ≥ 39 °C at admission, poor general appearance, hypotension, prolonged capillary filling time, and severe infection signs had longer hospital stays. In febrile neutropenic episodes, absolute monocyte count ≤ 100 cells/mm3, platelet count ≤ 50,000/mm3, and prolonged neutropenia delayed discharge time. Patients with microbiologically defined infections, especially those with positive catheter cultures, also had delayed discharge. Conclusion. The diagnosis of lymphoma, poor general condition at admission, presence of microbiologically defined infection, thrombocytopenia, delayed recovery of absolute neutrophil counts, and prolonged fever duration were significant factors in determining the treatment duration and predicting discharge time.

Objective: Childhood cancer treatment disrupts vaccination schedules and weakens or eliminates vaccine-induced immunity. In addition, post-treatment vaccine responses vary. This study aimed to assess post-treatment serum antibody levels and vaccine responses in children. Methods: Pediatric patients treated at Hacettepe University between years 2015 and 2020, achieved remission after chemotherapy for lymphoma and solid tumors were included. Post-treatment vaccination status, serum antibody levels for hepatitis A (HAV), hepatitis B (HBV), varicella-zoster (VZV), measles-mumps-rubella (MMR), and changes in vaccine responses were retrospectively analyzed. Results: The study included 533 patients. Post-treatment seronegativity rates were: measles (83.5%), HAV (64%), rubella (60.1%), HBV (48.5%), VZV (43.3%), and mumps (28%). Post-treatment antibody loss was observed for measles (47.1%), HAV (31.9%), HBV (31.4%), mumps (28.6%), VZV (21.7%), and rubella (11.4%). Seropositivity after 1 vaccine dose was seen with HAV (83.6%), rubella (82.9%), HBV (81.4%), VZV (63.5%), mumps (45.4%), and measles (33.3%). Seropositivity after 2 vaccine doses was achieved with HAV (98.8%), VZV (84.6%), rubella (80%), HBV (80%), measles (32.2%), and mumps (36.2%). Conclusion: Post-treatment serological vaccine responses in children were lower than anticipated despite multiple doses. Given the potential need for periodic serological assessments and booster vaccinations, long-term follow-ups are planned.

AIM:To evaluate the oncological outcomes and the prognostic factors for children with ependymoma who receive radiotherapy (RT) ± chemotherapy after surgery. MATERIAL AND METHODS:The medical records of 71 children with ependymoma who received RT between 2001 and 2022 were retrospectively evaluated. Survival outcomes and prognostic factors were analyzed using log-rank and cox-regression tests. SPSS v24.0 was utilized for statistical analyses. RESULTS:Gross total resection (GTR) was achieved in 37 (52%) patients. Craniospinal fluid (CSF) seeding was observed in 8 (11%) patients at the time of diagnosis. The median RT dose was 54 Gy (42-60 Gy). The median time from surgery to the first RT was 2.4 months (1-109 months). The median follow-up time was 65.9 months (2.5-242.8 months), and 5-y overall survival, progression-free survival (PFS), and local recurrence-free survival (LRFS) were 74%, 39%, and 46%, respectively. Recurrence was observed in 41 (58%) patients. Among patients who initiated treatment with chemotherapy, 5-y PFS and LRFS were higher in patients who received RT at the time of diagnosis than those who received RT at the progression (23% vs. 0%, p < 0.001 and 39% vs 0%, p < 0.001). In multivariate analysis, increased time from surgery to radiotherapy was found to be a poor prognostic factor for PFS. CONCLUSION:Young age, less than GTR, large residual tumor volume, initiation of treatment with chemotherapy after surgery, and increased time from surgery to radiotherapy may deteriorate survival. RT should not be delayed until progression, even in young patients receiving chemotherapy.