wilms' tumour

Anaplasia in Wilms tumor is recognized as the most important prognostically unfavorable histological feature. It is subtyped as focal anaplastic Wilms tumor (FAWT) and diffuse anaplastic Wilms tumor (DAWT). Outcomes of patients with DAWT remain poor in patients with stage III and IV tumors. Important issues relevant to anaplasia in Wilms tumor, including prevalence, treatment, outcomes, biomarkers, anaplasia, and chemotherapy, and the concept of tumor aggressiveness, are reviewed and discussed here. We also consider the differences in clinical approaches to anaplasia in Wilms tumor between the two major renal tumor clinical research groups: the International Society of Paediatric Oncology (SIOP) Renal Tumour Study Group and the Children's Oncology Group (COG) Renal Tumor Group...

BACKGROUND: Wilms' tumour is the most prevalent abdominal malignancy in children. This study focused on the mechanism of the miR-590-3p /Dickkopf 1 ( DKK1 ) axis in Wilms' tumour. METHODS: The mRNA levels of miR-590-3p and DKK1 in 49 pairs of Wilms' tumour pathological specimens and normal tissues were determined using real-time quantitative polymerase chain reaction (RT-qPCR). Wilms' tumour cells' invasion ability and proliferative ability were assessed using a Transwell assay and Cell Counting Kit-8 (CCK-8) assay, respectively...

INTRODUCTION: Diffuse intrinsic pontine glioma (DIPG) and paediatric high-grade glioma (pHGG) are aggressive glial tumours, for which conventional treatment modalities fall short. Dendritic cell (DC)-based immunotherapy is being investigated as a promising and safe adjuvant therapy. The Wilms' tumour protein (WT1) is a potent target for this type of antigen-specific immunotherapy and is overexpressed in DIPG and pHGG. Based on this, we designed a non-randomised phase I/II trial, assessing the feasibility and safety of WT1 mRNA-loaded DC (WT1/DC) immunotherapy in combination with conventional treatment in pHGG and DIPG...

AIM: This study reports the bilateral association of Peters' anomaly and congenital aniridia in monozygotic twins subsequently diagnosed with Wilms tumour (WAGR syndrome). METHODS: Two monozygotic female twins were referred at age 2 months with bilateral corneal opacity. A diagnosis of Peters' anomaly associated to aniridia was made in both eyes of both twins. Physical examination and ultrasonography were carried out at 12 months of age to explore the possibility of WAGR-related anomalies, specifically Wilms tumour...

Non-invasive differentiation of paediatric kidney tumours is particularly important in the SIOP-RTSG protocols, which recommend pre-operative chemotherapy without histological confirmation. The identification of clinical and tumour-related parameters may enhance diagnostic accuracy. Age, metastases, and tumour volume (TV) were retrospectively analysed in 3306 patients enrolled in SIOP/GPOH 9, 93-01, and 2001 including Wilms tumour (WT), congenital mesoblastic nephroma (CMN), clear cell sarcoma (CCSK), malignant rhabdoid tumour of the kidney (MRTK), and renal cell carcinoma (RCC)...

Wilms tumour (nephroblastoma) is a renal embryonal tumour that is frequently caused by constitutional variants in a small range of cancer predisposition genes. TRIM28 has recently been identified as one such gene. Previously, observational data strongly suggested a parent of origin effect, whereby Wilms tumour only occurred following maternal inheritance of a pathogenic genetic variant. However, here we report a child with bilateral Wilms tumour who had inherited a pathogenic TRIM28 variant from their father...

Specialist facilities for children are still unavailable in some Sub-Saharan African contexts. It is the case of pediatric urology, whose recent advances are still largely unshared. Prenatal diagnosis of urinary abnormalities (CAKUT) is largely unknown. Early recognition and referral of Undescended testis (UDT), Hypospadia, bladder exstrophy epispadias complex, ambiguous genitalia, stone disease, and tumours are uncommon in rural areas. Missed diagnosis is not uncommon and delayed management is associated with poor outcomes...

BACKGROUND: An international expert panel recently recommended 15 'non-stage prognostic indicators' (NSPIs) across eight childhood cancers, classified as essential or additional, for collection in population-based cancer registries. We aimed to describe the incidence distribution and survival of each of these NSPIs. PROCEDURES: Cases were extracted from the Australian Childhood Cancer Registry. The study cohort (n = 4187) comprised all children aged under 15 years diagnosed with an eligible cancer between 2010 and 2018, with follow-up until 31 December 2020...

INTRODUCTION: Renal artery embolization has been used in a palliative fashion for symptomatic relief of hematuria or flank pain in unresectable renal cell carcinoma in adults. There is limited data on the use of embolization for actively bleeding and unresectable tumors in the oncological pediatric population. CASE DESCRIPTION: A previously healthy 5-year-old boy with no significant past medical or surgical history presented to the clinic with gradually worsening abdominal distension associated with occasional abdominal pain, gross hematuria, and lethargy for four months...

BACKGROUND: Metastatic tumors account for 80% of all lung tumors in children. Wilms tumour and osteosarcoma are the most tumors of childhood that produce lung metastases. The aim of the current study is to assess the prognostic factors of pulmonary metastatectomy in pediatric solid tumours as age, number, size, site,laterality, resectability of pulmonary nodules, and number of Thoracotomies. Calculate overall survival among patients who underwent pulmonary metastatectomy. METHODS: It is a retrospective study including all pediatric patients with metastatic solid tumors to lungs treated at pediatric oncology department, National Cancer Institute, Cairo University from 2008 to 2014...

Characterisation of histological, immunohistochemical and molecular prognostic and predictive biomarkers has contributed significantly to precision medicine and better outcomes in the management of paediatric solid tumours. Prognostic biomarkers allow predictions to be made regarding a tumour's aggressiveness and clinical course, whereas predictive biomarkers help determine responses to a specific treatment. This review summarises prognostic biomarkers currently used in the more common paediatric solid tumours, with a brief commentary on the most relevant less common predictive biomarkers...

Nephroblastoma, colloquially known as Wilms' tumour (WT), is the predominant malignant renal neoplasm arising in the paediatric population. Modern therapeutic approaches for WT incorporate a synergistic combination of surgical intervention, radiotherapy, and chemotherapy, which substantially ameliorate the overall patient survival rate. Despite this, the optimal sequence of chemotherapy and surgical intervention remains a matter of contention, with each strategy presenting its own strengths and weaknesses that could influence clinical decision-making...

BACKGROUND: Completely necrotic Wilms tumor (CN-WT) following preoperative chemotherapy has been regarded as low-risk WT since the International Society of Paediatric Oncology (SIOP) 93-01 study, and patients have been treated with reduced postoperative therapy. The aim of the study was to evaluate whether the omission of adjuvant chemotherapy in patients with localized CN-WT stage I and radiotherapy in stage III was safe. PATIENTS AND METHODS: The retrospective observational study of outcomes of patients diagnosed with localized CN-WT on central pathology review and treated according to the SIOP 93-01 and SIOP-WT-2001 protocols (1993-2022)...

BACKGROUND: Wilms' tumor (WT) is the most common paediatric renal tumor and is one of the most treatment-responsive solid tumours. Survival from Wilms tumour (WT) in sub-Saharan Africa remains dismal as a result of late presentation, treatment abandonment and infrastructure deficit. The purpose of this study was to analyze the clinical outcome of children with Wilms tumour managed in a Nigerian referral centre over a 15-year period. METHODS: This is a retrospective study of children with WT (nephroblastoma) who were treated at our institution between January 2006 and December 2020...

A carcinosarcoma is a rare form of cancer characterised by the presence of both carcinomatous and sarcomatous components. Here, we present our experience with an extremely rare case of an uterine carcinosarcoma with immature teratoid-like differentiation. The patient was a woman in her 60s. She was referred for the evaluation of a uterine tumour. She underwent total abdominal hysterectomy with bilateral adnexectomy and received postoperative treatment with paclitaxel and carboplatin. On microscopic examination, the tumour had a heterogeneous appearance with a combination of carcinomatous and sarcomatous elements, and teratoid features...

AIMS: Ovarian Wilms tumour (WT)/nephroblastoma is an extremely rare neoplasm that has been reported to occur in pure form or as a component of a teratomatous neoplasm. We hypothesized that teratoma-associated and pure ovarian WT may represent different tumour types with diverging molecular backgrounds. To test this hypothesis, we comprehensively characterized a series of five tumours originally diagnosed as ovarian WT. METHODS AND RESULTS: The five cases comprised three teratoma-associated (two mature and one immature) and two pure WTs...

BACKGROUND: Hypospadias is a common genital malformation among boys. Studies indicate that hypospadias is associated with a higher risk of testicular cancer. Other forms of urological cancer may be linked to hypospadias via a mutual aetiology, hormonal dysfunction, or hypospadias complications, but this has not yet been studied. OBJECTIVE: To investigate the association between hypospadias and testicular cancer and the risk of other urological cancers among individuals born with hypospadias...

OBJECTIVE: To summarize and evaluate the outcomes of laparoscopic radical nephrectomy (LRN) and compare its safety and efficacy with open radical nephrectomy (ORN) in paediatric renal tumours (RT) and Wilms' tumour (WT). SUMMARY BACKGROUND DATA: ORN is the gold standard treatment for paediatric RT, consisting predominantly of WT. LRN is gaining popularity but remains controversial in paediatric surgical oncology. METHODS: Systematic search was performed for all eligible studies on LRN and comparative studies between LRN and ORN in paediatric RT and WT...

Late relapses of Wilms tumour are extremely uncommon but still represent possible events. Even more rarely Wilms tumours present as extrarenal neoplasms, for which it could be difficult to diagnose and treat them promptly.We present a unique case of late recurrence of Wilms tumour 16 years after the primary diagnosis, with location in the gynaecological system. The relapse presented as a vaginal mass, and it gradually raised up to involve the majority of pelvic organs. We accurately studied the tumour extension, even realising a 3D preoperative reconstruction, and we managed to evaluate the patient with a multidisciplinary team involving general surgeons, urologists, gynaecologists and plastic surgeons...

Patients with Wilms tumor (WT) in general have excellent survival, but the prognosis of patients belonging to the subgroup of WT with diffuse anaplasia (DA) is poor due to frequent resistance to chemotherapy. We hypothesized that DA WT cells might undergo changes, such as acquiring a persistent tolerance to DNA damage and copy number aberrations (CNAs), which could eventually lead to their resistance to chemotherapy treatment. Tissue sections from chemotherapy-treated DA WTs (n = 12) were compared with chemotherapy-treated nonanaplastic WTs (n = 15) in a tissue microarray system, enabling analysis of 769 tumor regions...