13,277 results match your criteria Wilms Tumor

Novel combined variants of WT1 and TET2 in a refractory and recurrent AML patient.

BMC Med Genomics 2021 Jun 13;14(1):158. Epub 2021 Jun 13.

Department of Hematology, Xuanwu Hospital, Capital Medical University, No. 45 Changchun Street, Beijing, 100053, People's Republic of China.

Background: Somatic mutations in Wilms' tumor 1 (WT1) and tet methylcytosine dioxygenase 2 (TET2) genes were separately perceived as contributors to hematopoietic disorders and usually thought to have a mutually exclusive effect in acute myeloid leukemia (AML). However, we found novel WT1 and TET2 variants persistently co-existed in a refractory and recurrent AML patient with t(9;11)(p21.3;q23. Read More

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Spatio-Phenotypic Tumor Profiling via Single-Cell 3-D Image Analysis.


Cancer Discov 2021 Jun 11. Epub 2021 Jun 11.

Single-cell 3-D imaging was used to spatially and phenotypically profile a pediatric Wilms tumor. Read More

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Age related gene DST represents an independent prognostic factor for MYCN non-amplified neuroblastoma.

BMC Pediatr 2021 Jun 11;21(1):272. Epub 2021 Jun 11.

Medical Research Center, Fujian Maternity and Child Health Hospital, Affiliated Hospital of Fujian Medical University, Fuzhou, Fujian, China.

Background: MYCN amplification and age are two critical prognostic factors of pediatric neuroblastoma. Previously, we had revealed the prognosis of MYCN target genes. However, the prognostic effects of age related genes in neuroblastoma are unclear. Read More

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Staging childhood cancers in Europe: Application of the Toronto stage principles for neuroblastoma and Wilms tumour. The JARC pilot study.

Pediatr Blood Cancer 2021 Jun 11:e29020. Epub 2021 Jun 11.

Developmental Biology and Cancer Programme, UCL Great Ormond Street Institute of Child Health, University College London, London, UK.

Background: The 'Toronto consensus principles and guidelines' (TG) provided paediatric-specific staging system affordable by population-based cancer registries (CRs). Within the European Rare Cancers Joint Action, a pilot study of the application of TG for childhood cancer (CC) was conducted to test the ability of CRs to reconstruct stage, describe stage across countries and assess survival by stage.

Procedure: Twenty-five CRs representing 15 countries contributed data on a representative sample of patients with neuroblastoma (NB) and Wilms tumour (WT) <15 years, diagnosed between 2000 and 2016. Read More

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Synchronous bilateral Wilms' tumor with liver metastasis.

BMC Urol 2021 Jun 10;21(1):91. Epub 2021 Jun 10.

Department of Medicine, School of Medicine, Makerere University College of Health Sciences, Kampala, Uganda.

Background: Wilms' tumor (nephroblastoma) is mostly unilateral; however, bilateral Wilms' tumors are seen in about 5-8% of patients. This can be synchronous or metachronous. It is uncommon to get liver metastasis from bilateral Wilms' tumor. Read More

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Prognostic Significance Of Histopathological Response To Preoperative Chemotherapy In Unilateral Wilms Tumor: An Analysis Of 899 Patients Treated On The Siop Wt 2001 Protocol In The Uk Cclg And Gpoh Studies.

Int J Cancer 2021 Jun 10. Epub 2021 Jun 10.

Department of Hematology and Oncology, University of Saarland, Homburg, Germany.

In the SIOP Wilms tumor (WT) studies preoperative chemotherapy is used as primary treatment, and tumors are classified thereafter by pathologists. Completely necrotic WTs (CN-WTs) are classified as low-risk tumors. The aim of the study was to evaluate whether a subset of regressive type WTs (RT-WTs)(67-99% chemotherapy-induced changes - CIC) showing an exceptionally good response to preoperative chemotherapy had comparably excellent survivals as CN-WTs, and to establish a cut-off point of CIC that could define this subset. Read More

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New mutation in WT1 gene in a boy with an incomplete form of Denys-Drash syndrome: A CARE-compliant case report.

Medicine (Baltimore) 2021 May;100(19):e25864

Children's Republican Clinical Hospital of the Ministry of Health of the Republic of Tatarstan, Kazan, Russian Federation.

Rationale: Pediatric patients with WTl-associated syndromes (including Wilms' tumor-aniridia syndrome and Denys-Drash syndrome), Perlman syndrome, mosaic aneuploidy, and Fanconi anemia with a biallelic breast cancer type 2 susceptibility protein mutation have the highest risk of developing Wilms' tumor.

Patient Concerns And Diagnosis: We describe a patient with bilateral metachronous Wilms' tumor, ambiguous genitalia characterized by 46, XY disorder of sexual development (DSD) with scrotal hypospadias and bilateral abdominal cryptorchidism, but without nephropathy. At the age of 7 months, the child underwent left nephrectomy with left orchiopexy. Read More

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Recent Advances in Pathology: the 2021 Annual Review Issue of The Journal of Pathology.

J Pathol 2021 07;254(4):303-306

RECAMO, Masaryk Memorial Cancer Institute, Brno, Czech Republic.

The 2021 Annual Review Issue of The Journal of Pathology contains 14 invited reviews on current research areas of particular importance in pathology. The subjects included here reflect the broad range of interests covered by the journal, including both basic and applied research fields but always with the aim of improving our understanding of human disease. This year, our reviews encompass the huge impact of the COVID-19 pandemic, the development and application of biomarkers for immune checkpoint inhibitors, recent advances in multiplexing antigen/nucleic acid detection in situ, the use of genomics to aid drug discovery, organoid methodologies in research, the microbiome in cancer, the role of macrophage-stroma interactions in fibrosis, and TGF-β as a driver of fibrosis in multiple pathologies. Read More

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The Emerging Role of Thymopoietin-Antisense RNA 1 as Long Noncoding RNA in the Pathogenesis of Human Cancers.

DNA Cell Biol 2021 Jun 4. Epub 2021 Jun 4.

State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, National Clinical Research Center for Infectious Diseases, Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

Long noncoding RNAs (lncRNAs) play essential roles in the occurrence and development of multiple human cancers. An accumulating body of researches have investigated thymopoietin antisense RNA 1 (TMPO-AS1) as a newly discovered lncRNA, which functions as an oncogenic lncRNA that is upregulated in various human malignancies and associated with poor prognosis. Many studies have detected abnormally high expression levels of TMPO-AS1 in multiple cancers, such as lung cancer, breast cancer, colorectal cancer (CRC), hepatocellular carcinoma, CRC, gastric cancer, ovarian cancer, thyroid cancer, esophageal cancer, Wilms tumor, cervical cancer, retinoblastoma, bladder cancer, osteosarcoma, and prostate cancer. Read More

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Induced dendritic cells co-expressing GM-CSF/IFN-α/tWT1 priming T and B cells and automated manufacturing to boost GvL.

Mol Ther Methods Clin Dev 2021 Jun 9;21:621-641. Epub 2021 Apr 9.

Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, 30625 Hannover, Germany.

Acute myeloid leukemia (AML) patients with minimal residual disease and receiving allogeneic hematopoietic stem cell transplantation (HCT) have poor survival. Adoptive administration of dendritic cells (DCs) presenting the Wilms tumor protein 1 (WT1) leukemia-associated antigen can potentially stimulate T and B cell development to harness the graft-versus-leukemia (GvL) effect after HCT. We established a simple and fast genetic modification of monocytes for simultaneous lentiviral expression of a truncated WT1 antigen (tWT1), granulocyte macrophage-colony-stimulating factor (GM-CSF), and interferon (IFN)-α, promoting their self-differentiation into potent "induced DCs" (iDCtWT1). Read More

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Childhood Clear Cell Sarcoma of Kidney: Incidence and Survival.

Front Pediatr 2021 20;9:675373. Epub 2021 May 20.

Anhui Provincial Cancer Institute, The First Affiliated Hospital of Anhui Medical University, Hefei, China.

This study is to describe current incidence of childhood clear cell sarcoma of kidney (CCSK) and to investigate the present survival of this cancer. Surveillance, Epidemiology, and End Result (SEER) data was used to identify children with CCSK and Wilms tumor (WT) aged 0-19 years in the US. Age-adjusted incidences were estimated over the decades. Read More

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Maintenance of WT1 expression in tumor cells is associated with a good prognosis in malignant glioma patients treated with WT1 peptide vaccine immunotherapy.

Cancer Immunol Immunother 2021 Jun 5. Epub 2021 Jun 5.

Department of Neurosurgery, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kawaramachi-dori, Hirokoji-agaru, Kamigyo-ku, Kyoto, 602-8566, Japan.

We have previously revealed the overexpression of Wilms' tumor gene 1 (WT1) in malignant glioma and developed WT1 peptide vaccine cancer immunotherapy. A phase II clinical trial indicated the clinical efficacy of the WT1 peptide vaccine for recurrent malignant glioma. Here, we aimed to investigate the immunological microenvironment in glioma tissues before and after WT1 peptide vaccine treatment. Read More

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Revealing the spatio-phenotypic patterning of cells in healthy and tumor tissues with mLSR-3D and STAPL-3D.

Nat Biotechnol 2021 Jun 3. Epub 2021 Jun 3.

Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.

Despite advances in three-dimensional (3D) imaging, it remains challenging to profile all the cells within a large 3D tissue, including the morphology and organization of the many cell types present. Here, we introduce eight-color, multispectral, large-scale single-cell resolution 3D (mLSR-3D) imaging and image analysis software for the parallelized, deep learning-based segmentation of large numbers of single cells in tissues, called segmentation analysis by parallelization of 3D datasets (STAPL-3D). Applying the method to pediatric Wilms tumor, we extract molecular, spatial and morphological features of millions of cells and reconstruct the tumor's spatio-phenotypic patterning. Read More

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Impact of the COVID-19 pandemic on paediatric patients with cancer in low-income, middle-income and high-income countries: protocol for a multicentre, international, observational cohort study.

BMJ Open 2021 06 2;11(6):e045679. Epub 2021 Jun 2.

Nuffield Department of Surgical Sciences, University of Oxford, Oxford, UK.

Introduction: Childhood cancers are a leading cause of non-communicable disease deaths for children around the world. The COVID-19 pandemic may have impacted on global children's cancer services, which can have consequences for childhood cancer outcomes. The Global Health Research Group on Children's Non-Communicable Diseases is currently undertaking the first international cohort study to determine the variation in paediatric cancer management during the COVID-19 pandemic, and the short-term to medium-term impacts on childhood cancer outcomes. Read More

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Pediatric Tumors-Mediated Inhibitory Effect on NK Cells: The Case of Neuroblastoma and Wilms' Tumors.

Cancers (Basel) 2021 May 14;13(10). Epub 2021 May 14.

Immunology Research Area, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Natural killer (NK) cells play a key role in the control of cancer development, progression and metastatic dissemination. However, tumor cells develop an array of strategies capable of impairing the activation and function of the immune system, including NK cells. In this context, a major event is represented by the establishment of an immunosuppressive tumor microenvironment (TME) composed of stromal cells, myeloid-derived suppressor cells, tumor-associated macrophages, regulatory T cells and cancer cells themselves. Read More

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Role of anticoagulation in the management of tumor thrombus: A 10-year single-center experience.

Pediatr Blood Cancer 2021 Jun 1:e29173. Epub 2021 Jun 1.

Texas Children's Hospital, Cancer and Hematology Center, Baylor College of Medicine, Houston, Texas, USA.

Background: Children with cancer diagnosis are overall at a higher risk of thrombosis. For a newly diagnosed blood clot, patients are commonly started on anticoagulants to prevent further extension and embolization of the clot. In the rare instance that a pediatric patient has a tumor thrombus, role of anticoagulation is less clear. Read More

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Comparative analysis of the clinical characteristics and outcomes of patients with Wilms tumor in the United Kingdom and Japan.

Pediatr Blood Cancer 2021 May 31:e29143. Epub 2021 May 31.

Developmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK.

Background: Wilms tumor (WT) demonstrates epidemiological differences by world region and ethnicity. To enhance understanding of these differences, we retrospectively analyzed clinical trial data sets from the UK and Japan over a 20-year period.

Procedure: We used data from three consecutive clinical trials in the UK and a single study in Japan that enrolled patients diagnosed during 1996-2015, to compare clinical characteristics and outcomes between countries. Read More

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Novel findings and expansion of phenotype in a mosaic RASopathy caused by somatic KRAS variants.

Am J Med Genet A 2021 May 30. Epub 2021 May 30.

Division of Genetics and Metabolism, Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.

Mosaic KRAS variants and other RASopathy genes cause oculoectodermal, encephalo-cranio-cutaneous lipomatosis, and Schimmelpenning-Feuerstein-Mims syndromes, and a spectrum of vascular malformations, overgrowth and other associated anomalies, the latter of which are only recently being characterized. We describe eight individuals in total (six unreported cases and two previously reported cases) with somatic KRAS variants and variably associated features. Given the findings of somatic overgrowth (in seven individuals) and vascular or lymphatic malformations (in eight individuals), we suggest mosaic RASopathies (mosaic KRAS variants) be considered in the differential diagnosis for individuals presenting with asymmetric overgrowth and lymphatic or vascular anomalies. Read More

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Clonal Cytopenia of Undetermined Significance in a Patient with Congenital Wilms' Tumor 1 and Acquired DNMT3A Gene Mutations.

Intern Med 2021 May 29. Epub 2021 May 29.

Department of Hematology, Sumitomo Hospital, Japan.

Congenital mutations of the Wilms' tumor 1 (WT1) gene can lead to various abnormalities, including renal/gonadal developmental disorders and cardiac malformations. Although there have been many reports of somatic WT1 mutations in patients with acute myeloid leukemia and myelodysplastic syndrome, congenital WT1 mutations have not been reported in hematological disorders. We herein report a patient with early-onset clonal cytopenia of undetermined significance that was associated with a congenital mutation of WT1 and an acquired mutation of DNMT3A (encoding DNA [cytosine-5]-methyltransferase 3A). Read More

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Galinpepimut-S (GPS): an investigational agent for the treatment of acute myeloid leukemia.

Expert Opin Investig Drugs 2021 May 31:1-7. Epub 2021 May 31.

Malignant Hematology Department, H. Lee Moffitt Cancer Center, Tampa, FL, USA.

: Acute myeloid leukemia (AML) is a disorder wherein clonal expansion of undifferentiated myeloid precursors results in compromised hematopoiesis and bone marrow failure. Even though numerous AML patients respond to induction chemotherapy, relapse is common and hence new therapeutic approaches are needed. Wild-type Wilms tumor gene (WT1) is greatly expressed in numerous blood disorders and so this has led to development of galinpepimut-S, a WT1 vaccine as a modality to maintain remission in patients with AML. Read More

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Impact of neoadjuvant chemotherapy on thrombus viability in patients with Wilms tumour and caval extension: systematic review with meta-analysis.

BJS Open 2021 May;5(3)

Alder Hey Children's Hospital NHS Foundation Trust, School of Health and Life Science, University of Liverpool, UK.

Background: Inferior vena cava (IVC) tumour thrombus in children with Wilms tumour is typically managed with neoadjuvant chemotherapy with the intention of achieving thrombus regression in order to minimize the risks associated with complex vascular surgery.

Methods: A systematic review of Medline and Embase databases was undertaken to identify all eligible studies with reference to thrombus viability in Wilms tumour index cases with caval/cardiac extension. A meta-analysis of proportions was utilized for pooled thrombus viability data across studies. Read More

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Feline mesothelioma: case report and review of cytologic, immunocytochemical, histopathologic, and immunohistochemical findings.

J Vet Diagn Invest 2021 May 28:10406387211017489. Epub 2021 May 28.

Pathobiological Sciences, School of Veterinary Medicine, Louisiana State University, Baton Rouge, LA, USA.

Mesotheliomas are uncommon neoplasms that arise from mesothelial cells in either the abdominal or thoracic cavities and are rarely diagnosed in cats. A 10-y-old spayed female domestic shorthair cat was presented to the Louisiana State University oncology service for evaluation of a large amount of abdominal effusion. Abdominal ultrasound identified a large mesenteric mass with numerous ill-defined nodules. Read More

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Long-term follow-up of surgical outcomes for patients with Wilms tumor and neuroblastoma.

Cancer 2021 May 27. Epub 2021 May 27.

Division of General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Background: There are minimal data on long-term surgical outcomes of patients who have undergone resection for Wilms tumor (WT) and neuroblastoma (NB).

Methods: A retrospective review of patients in a long-term survivor clinic between the years 1967 and 2016 in a pediatric tertiary care hospital (>5 years posttreatment) was performed.

Results: Eighty-six survivors of WT and 86 survivors of NB who had ongoing follow-up in the survivors' clinic were identified. Read More

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Non-transplantable cord blood units as a source for adoptive immunotherapy of leukaemia and a paradigm of circular economy in medicine.

Br J Haematol 2021 May 26. Epub 2021 May 26.

Adult Hematology and Stem cell Transplantation Department, King Fahad Specialist Hospital Dammam, Dammam, Saudi Arabia.

Advances in immunotherapy with T cells armed with chimeric antigen receptors (CAR-Ts), opened up new horizons for the treatment of B-cell lymphoid malignancies. However, the lack of appropriate targetable antigens on the malignant myeloid cell deprives patients with refractory acute myeloid leukaemia of effective CAR-T therapies. Although non-engineered T cells targeting multiple leukaemia-associated antigens [i. Read More

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Gene expression-based immune infiltration analyses of renal cancer and their associations with survival outcome.

BMC Cancer 2021 May 24;21(1):595. Epub 2021 May 24.

Department of Pediatric Surgery, Cangzhou Central Hospital, No.16 Xinhua West Road, Cangzhou, 061000, Hebei, China.

Background: Renal cancer is a common malignant tumor with an increasing incidence rate.

Methods: In this study, based on the gene expression profiles, we analyzed the compositions of tumor-infiltrating immune cells (TIICs) in renal cancer and paracancerous samples using CIBERSORT. The proportions of 22 TIICs subsets in 122 paired renal carcinoma and paracancerous samples, and 224 Wilms tumor (WT) samples varied between intragroup and intergroup. Read More

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Estimated clinical benefit of combining highly conformal target volumes with Volumetric-Modulated Arc Therapy (VMAT) versus conventional flank irradiation in pediatric renal tumors.

Clin Transl Radiat Oncol 2021 Jul 3;29:20-26. Epub 2021 May 3.

Princess Máxima Center for Pediatric Oncology, Heidelberglaan 25, 3584 CS, Utrecht, the Netherlands.

Background: For decades, Anterior-Posterior/Posterior-Anterior (AP/PA) photon beams were standard-of-care for flank irradiation in children with renal cancer. Recently, highly conformal flank target volumes were defined correcting for postoperative organ shift and intra-fraction motion.By radiotherapy treatment plan comparison, this study aims to estimate the clinical benefits and potential risks of combining highly conformal target volumes with Volumetric-Modulated Arc Therapy (VMAT) versus conventional target volumes with AP/PA beams for flank irradiation. Read More

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Simpson-Golabi-Behmel syndrome: One family, same mutation, different outcome.

Am J Med Genet A 2021 May 18. Epub 2021 May 18.

Department of Genetics, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Simpson-Golabi-Behmel syndrome (SGBS) is a rare X-linked condition characterized by pre and postnatal overgrowth with visceral and skeletal abnormalities. The syndrome is caused mainly by mutations in the X-linked gene GPC3. Clinical presentation of SGBS in affected males is well defined, but there is a lack of knowledge about affected females, with very few reported cases. Read More

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Isolated- and Beckwith-Wiedemann syndrome related- lateralised overgrowth (hemihypertrophy): Clinical and molecular correlations in 94 individuals.

Clin Genet 2021 May 16. Epub 2021 May 16.

Department of Medical Genetics, University of Cambridge and NIHR Cambridge Biomedical Research Centre, and Cancer Research UK Cambridge Centre, Cambridge Biomedical Campus, Cambridge, UK.

The congenital imprinting disorder, Beckwith-Wiedemann syndrome (BWS) is associated with variable clinical features including hemihypertrophy/lateralised overgrowth (LO) and embryonal tumour predisposition. BWS-associated (epi)genetic alterations occur in a subset of patients with isolated LO (ILO), leading to the concept of BWS spectrum disorder (BWSp). We investigated the relationship between clinical features and molecular diagnostic results in a cohort with LO using the BWSp international consensus group (BWSICG) clinical scoring system. Read More

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Risk factors for post-nephrectomy hypotension in pediatric patients.

Pediatr Nephrol 2021 May 14. Epub 2021 May 14.

Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-8535, Japan.

Background: Although hypotension is a life-threatening complication of nephrectomy in children, risk factors for its development remain unknown. We evaluated the incidence, clinical course, and associated risk factors of pediatric post-nephrectomy hypotension in an observational study.

Methods: This retrospective observational study included the clinical data of children who underwent nephrectomy in our center between 2002 and 2020. Read More

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