Although rare in adults, Wilms tumor is the most common pediatric renal tumor. Treatment typically involves radical nephrectomy followed by adjuvant chemotherapy or radiation, although outcomes differ between children and adults which may be due to challenges in accurately diagnosing these patients. In this article, we present a case report of an adult patient with Jeune syndrome and multiple urologic abnormalities who underwent radical nephrectomy for a large renal mass and was subsequently diagnosed with an epithelial predominant Wilms tumor. Epithelial predominant Wilms tumor may have distinct origins from other Wilms tumor histological subtypes and may incur better outcomes. Herein, we discuss the literature surrounding this rare entity as well as the anticipated treatment course.

BACKGROUND: Precision in surgical documentation is essential to avoid miscommunication and errors in patient care. Synoptic operative reports are more precise than narrative operative reports, however they have not been widely implemented in pediatric surgical oncology. To assess the need for implementation of synoptic operative reports in pediatric surgical oncology, we examined the completeness of narrative operative reports in patients undergoing resection of Wilms tumor.
METHODS: We conducted a retrospective review of narrative operative reports for resection of Wilms tumor at a single pediatric oncology center from January 2022 through July 2023. Primary outcomes were the presence or absence of 11 key operative report components. Inclusion rates were calculated as simple percentages. Unilateral and bilateral operations were considered.
RESULTS: Thirty-five narrative reports for Wilms tumor resection were included. The most consistently documented operative report components were estimated blood loss, indication for surgery, intraoperative complications, and specimen naming (100% documentation rates). Documentation of lymph node sampling was present in 94.3% of reports. The least consistently documented components were assessment of intraoperative tumor spillage, completeness of resection, metastatic disease, and assessment of vascular involvement (each ≤40% documentation rate). All 11 key components were documented in three reports.
CONCLUSIONS: Even at a large tertiary pediatric oncology referral center, narrative operative reports for pediatric Wilms tumor resection were found to be frequently missing important components of surgical documentation. Often, these were omissions of negative findings. Utilization of synoptic operative reports may be able to reduce these gaps.

BACKGROUND: Wilms tumor (WT) survival has been affected by the evolution in clinical and biological prognostic factors. Significant differences in survival rates indicate the need for further efforts to reduce these disparities. This study aims to evaluate the clinicopathological data impact on survival among patients after Wilm\'s diagnosis.
METHODS: The study utilized the SEERStat Database to identify Wilms tumor patients, applying SEERStat software version 8.3.9.2 for data extraction. Selection criteria involved specific codes based on the International Classification of Diseases for Oncology (ICDO-3), excluding cases with unknown SEER stage, incomplete survival data, unknown size, or lymph node status. Statistical analyses, including Kaplan-Meier estimates and Cox regression models, were conducted using R software version 3.5. Standardized mortality ratios (SMR) were computed with SEER*Stat software, and relative and conditional survival analyses were performed to evaluate long-term survival outcomes.
RESULTS: Of 2273 patients diagnosed with Wilms tumor, (1219 patients, 53.6% were females with an average age group of 3-8 years (50.2%). The overall mean survival after five years of diagnosis was 93.6% (2.6-94.7), and the overall mean survival rate was 92.5% (91.3-93.8) after ten years of diagnosis. Renal cancers were identified as the leading cause of death (77.3%), followed by nonrenal cancers (11%) and noncancer causes (11%). Additionally, robust relative survival rates of 98.10%, 92.80%, and 91.3% at one, five, and ten years, respectively, were observed, with corresponding five-year conditional survival rates indicating an increasing likelihood of survival with each additional year post-diagnosis. Univariate Cox regression identified significant prognostic factors: superior CSS for patients below 3 years (cHR 0.48) and poorer CSS for those older than 15 years (cHR 2.72), distant spread (cHR 10.24), regional spread (cHR 3.09), and unknown stage (cHR 4.97). In the multivariate model, age was not a significant predictor, but distant spread (aHR 9.22), regional spread (aHR 2.84), and unknown stage (aHR 4.98) were associated with worse CSS compared to localized tumors.
CONCLUSIONS: This study delving into WT survival dynamics reveals a multifaceted landscape influenced by clinicopathological variables. This comprehensive understanding emphasizes the imperative for ongoing research and personalized interventions to refine survival rates and address nuanced challenges across age, stage, and tumor spread in WT patients.

BACKGROUND: Concomitant Wilms tumor (WT) and autosomal dominant polycystic kidney disease (ADPKD) is exceedingly rare, presenting a diagnostic and technical challenge to pediatric surgical oncologists. The simultaneous workup and management of these disease processes are incompletely described.
METHODS: We performed a retrospective analysis of patients treated at our institution with concomitant diagnoses of WT and ADPKD. We also review the literature on the underlying biology and management principles of these conditions.
RESULTS: We present three diverse cases of concomitant unilateral WT and ADPKD who underwent nephrectomy. One patient had preoperative imaging consistent with ADPKD with confirmatory testing postoperatively, one was found to have contralateral renal cysts intraoperatively with confirmatory imaging post nephrectomy, and one was diagnosed in childhood post nephrectomy. All patients are alive at last follow-up, and the patient with longest follow-up has progressed to end-stage kidney failure requiring transplantation and dialysis in adulthood. All patients underwent germline testing and were found to have no cancer predisposition syndrome or pathogenic or likely pathogenic variants for WT.
CONCLUSIONS: Concomitant inheritance of ADPKD and development of WT are extremely rare, and manifestations of ADPKD may not present until late childhood or adulthood. ADPKD is not a known predisposing condition for WT. When ADPKD diagnosis is made by family history, imaging, and/or genetic testing before WT diagnosis and treatment, the need for extensive preoperative characterization of cystic kidney lesions in children and increased risk of post-nephrectomy kidney failure warrant further discussion of surgical approach and perioperative management strategies.

Wilms tumor (WT), or nephroblastoma, is the predominant renal malignancy in the pediatric population. This narrative review explores the evolution of personalized care strategies for WT, synthesizing critical developments in molecular diagnostics and treatment approaches to enhance patient-specific outcomes. We surveyed recent literature from the last five years, focusing on high-impact research across major databases such as PubMed, Scopus, and Web of Science. Diagnostic advancements, including liquid biopsies and diffusion-weighted MRI, have improved early detection precision. The prognostic significance of genetic markers, particularly WT1 mutations and miRNA profiles, is discussed. Novel predictive tools integrating genetic and clinical data to anticipate disease trajectory and therapy response are explored. Progressive treatment strategies, particularly immunotherapy and targeted agents such as HIF-2α inhibitors and GD2-targeted immunotherapy, are highlighted for their role in personalized treatment protocols, especially for refractory or recurrent WT. This review underscores the necessity for personalized management supported by genetic insights, with improved survival rates for localized disease exceeding 90%. However, knowledge gaps persist in therapies for high-risk patients and strategies to reduce long-term treatment-related morbidity. In conclusion, this narrative review highlights the need for ongoing research, particularly on the long-term outcomes of emerging therapies and integrating multi-omic data to inform clinical decision-making, paving the way for more individualized treatment pathways.

Wilms tumor (WT) is the most common renal malignancy seen in pediatric patients. Although lungs are the most common site of metastasis in Wilms tumor, non-malignant pleural effusion has been infrequently reported. Here, we report a case of an eleven-year-old female who presented with an abdominal mass and progressive breathlessness. On further evaluation, she was found to have a right-sided Wilms tumor with ipsilateral massive pleural effusion. The effusion resolved almost completely after four weeks of chemotherapy. We conclude that patients suffering from Wilms tumor presenting with pleural effusion need not be synonymous with metastatic disease and can have a favorable prognosis.

BACKGROUND: Wilms\' tumor (WT) is the most frequent renal tumor in childhood. Therapeutic management progression has increased survival rates, and as a result, long-term adverse effects.
METHODS: A descriptive retrospective study of a case series from 1977 to 2023 was carried out. The characteristics of the treatments received and the adverse effects listed on medical records were analyzed via phone surveys.
RESULTS: 50 patients (25 boys-25 girls) with a mean age of 3.6 years (3 months-11 years) at diagnosis were included. Most of them (94%) were treated according to the protocol established by the European standards of pediatric oncology, which are characterized by the use of neoadjuvant chemotherapy. In one patient, the American treatment scheme was followed. The most common drugs used were vincristine and actinomycin D (78%). Only 12 patients (28%) received anthracyclines. Unilateral nephrectomy was the most frequent surgical technique (84%). Renal disorders were the most common (46%). However, the occurrence of second neoplasias (9%) and reproductive disorders (8% between boys and girls) had a greater impact on patients\' quality of life. Multiple - cardiac (23%), endocrine (26%), and pulmonary (15%) - disorders associated with the treatments received were reported.
CONCLUSIONS: WT treatment has an impact on health. Adequate and rigorous surgery, close follow-up, and limiting chemotherapy doses and radiation exposure can minimize long-term sequels.
BACKGROUND: El tumor de Wilms (TW) es el tumor renal más frecuente en la infancia. La evolución del manejo terapéutico ha incrementado la tasa de supervivencia y como consecuencia, los efectos secundarios a largo plazo.
METHODS: Realizamos un estudio retrospectivo descriptivo a partir de una serie de casos entre 1977 y 2023. Estudiamos las características de los tratamientos recibidos y los efectos secundarios que constan en su historia clínica y a través de cuestionarios telefónicos.
RESULTS: Localizamos 50 pacientes (25 hombres-25 mujeres) con edad media al diagnóstico de 3,6 años (3 meses-11 años). La mayoría fueron tratados según protocolo vigente de las guías europeas de oncología pediátrica (94%) caracterizadas por el uso de quimioterapia neoadyuvante. En un paciente Se siguió el esquema americano de tratamiento. Los fármacos más utilizados fueron vincristina y actinomicina D (78%); solo 12 pacientes (28%) recibieron antraciclinas. La nefrectomía unilateral fue la técnica quirúrgica más empleada (84%). Las alteraciones renales fueron las más frecuentes (46%). Sin embargo, la aparición de segundas neoplasias (9%) y aquellas alteraciones relacionadas con la reproducción (8% entre hombres y mujeres) suponen un mayor impacto en la calidad de vida de los pacientes. Se describen múltiples alteraciones: cardíacas (23%), endocrinas (26%) o pulmonares (15%) relacionadas con los tratamientos recibidos.
CONCLUSIONS: El tratamiento del TW afecta a la salud general. Una cirugía adecuada y rigurosa, limitar las dosis de quimioterapia, minimizar la exposición a la radiación y un seguimiento estrecho puede minimizar las secuelas a largo plazo.

UNASSIGNED: Angiogenesis is critical for tumor growth and metastasis. Bevacizumab is an antiangiogenic drug used to treat various adult and childhood solid tumors. Its potential efficacy in Wilms tumor (WT) with poor prognosis is not established.
UNASSIGNED: The response to bevacizumab-containing regimens in relapsed or refractory WT was reviewed in available literature. Searches were conducted using PubMed, Scopus, and ClinicalTrials.gov databases. Eight papers were identified, published between 2007 and 2020, including six treatment regimens, predominantly vincristine, irinotecan, and bevacizumab (VIB) ± temozolomide (VITB). Among 16 evaluable patients, there were two complete responses, seven partial responses, five patients achieved stable disease (SD), and two patients had progressive disease. Objective responses (OR) were observed in 56% of all cases. OR or SD was observed in 89% (8/9) patients who received VIB/VITB. Bevacizumab was generally well tolerated. Related toxicities included hypertension, proteinuria, and delayed wound healing.
UNASSIGNED: This review suggests potential effectiveness and good tolerability of bevacizumab in the setting of relapsed/refractory WT when used in combination with other drugs. Such combination therapies may serve as a bridging treatment option to other interventions and more personalized treatment options in the future; however, focused trials are needed to obtain additional evidence.

How disruptions to normal cell differentiation link to tumorigenesis remains incompletely understood. Wilms tumor, an embryonal tumor associated with disrupted organogenesis, often harbors mutations in epigenetic regulators, but their role in kidney development remains unexplored. Here, we show at single-cell resolution that a Wilms tumor-associated mutation in the histone acetylation reader ENL disrupts kidney differentiation in mice by rewiring the gene regulatory landscape. Mutant ENL promotes nephron progenitor commitment while restricting their differentiation by dysregulating transcription factors such as Hox clusters. It also induces abnormal progenitors that lose kidney-associated chromatin identity. Furthermore, mutant ENL alters the transcriptome and chromatin accessibility of stromal progenitors, resulting in hyperactivation of Wnt signaling. The impacts of mutant ENL on both nephron and stroma lineages lead to profound kidney developmental defects and postnatal mortality in mice. Notably, a small molecule inhibiting mutant ENL\'s histone acetylation binding activity largely reverses these defects. This study provides insights into how mutations in epigenetic regulators disrupt kidney development and suggests a potential therapeutic approach.

OBJECTIVE: To present the experience of laparoscopic nephrectomies and kidney resections in children.
METHODS: There were 28 minimally invasive surgeries for renal tumors between July 2015 and March 2023 (92 months). There were 16 (57%) boys and 12 (43%) girls who underwent 22 nephrectomies and 6 kidney resections. The median age of patients was 54 (38; 76.5) months.
RESULTS: In the laparoscopic nephrectomy group, the median surgery time was 135 (108-188) min, blood loss - 10 (3.75-15) ml. Total resection was confirmed in all patients. In the group of minimally invasive kidney resections, these values were 182.5 (157.5; 265) min and 50 (42.5; 117.5) ml, respectively. Histological examination confirmed total resection in all patients. In both groups, none patient developed postoperative complications. Event-free survival was 86.72% with a median follow-up of 82 months, and local recurrence-free survival was 95.8% with a median follow-up of 89.8 months.
CONCLUSIONS: Minimally invasive nephrectomies and resections are safe in children in case of careful patient selection.
UNASSIGNED: Представить опыт лапароскопических нефрэктомий и резекций почек у детей.
UNASSIGNED: За период с июля 2015 по март 2023 г. (92 мес) в отделении онкологии и детской хирургии ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии имени Дмитрия Рогачева» Минздрава России выполнено 28 минимально инвазивных операций по поводу очаговых новообразований почек у детей [мальчиков — 16 (57%), девочек — 12 (43%)], среди которых: 22 нефрэктомии и 6 резекций почки. Медиана возраста больных составила 54 (38; 76,5) мес.
UNASSIGNED: В группе лапароскопических нефрэктомий медиана продолжительности операции составила 135 (108—188) мин, а медиана кровопотери 10 (3,75—15) мл. У всех пациентов подтверждено радикальное удаление опухоли. В группе минимально инвазивных резекций почек медиана продолжительности операции составила 182,5 (157,5; 265) мин, а медиана кровопотери — 50 (42,5; 117,5) мл. При гистологическом исследовании у всех пациентов подтверждена радикальная резекция. В обеих группах в послеоперационном периоде ни у кого из больных не отмечено развития осложнений. Бессобытийная выживаемость составила 86,72%, при медиане наблюдения 82 мес, а выживаемость без локального рецидива — 95,8%, при медиане наблюдения 89,8 мес.
UNASSIGNED: Таким образом, при тщательном отборе пациентов возможно безопасное выполнение как нефрэктомий, так и паренхимосберегающих вмешательств из минимально инвазивного доступа у детей.