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Wilms tumour

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The >90% rates of overall survival for children with Wilms tumour are remarkable and have been achieved at the same time as reducing treatment for most patients. However, beneath this headline figure, 20% of patients still relapse after first-line therapy and up to 25% of survivors report severe late effects. The aim of the SIOP-RTSG is to improve outcomes and to reduce acute and late treatment toxic effects in all children.
December 6, 2016: Nature Reviews. Urology
Irmgard U Haussmann, Zsuzsanna Bodi, Eugenio Sanchez-Moran, Nigel P Mongan, Nathan Archer, Rupert G Fray, Matthias Soller
N(6)-methyladenosine (m(6)A) is the most common internal modification of eukaryotic messenger RNA (mRNA) and is decoded by YTH domain proteins. The mammalian mRNA m(6)A methylosome is a complex of nuclear proteins that includes METTL3 (methyltransferase-like 3), METTL14, WTAP (Wilms tumour 1-associated protein) and KIAA1429. Drosophila has corresponding homologues named Ime4 and KAR4 (Inducer of meiosis 4 and Karyogamy protein 4), and Female-lethal (2)d (Fl(2)d) and Virilizer (Vir). In Drosophila, fl(2)d and vir are required for sex-dependent regulation of alternative splicing of the sex determination factor Sex lethal (Sxl)...
November 30, 2016: Nature
Ronica H Nanda, Bahig M Shehata, Nasim Khoshnam, Megan Durham, Sungjin Kim, Wasim Selwanes, Zhengjia Chen, Chao Zhang, Natia Esiashvili
It is unclear if lymph node sampling in Wilms tumour, though critical for staging purposes, affects survival outcomes. The value of lymph node sampling in patients treated with neoadjuvant chemotherapy (NAC) is even more uncertain. We reviewed our institutional data to determine the impact of lymph node sampling on survival, as well as its role in the context of NAC. A total of 185 patients with Wilms tumour treated at our institution were included in this analysis. The number of nodes sampled (≤7, or >7), lymph node status (unknown, negative, or positive), pathological stage, and use of neoadjuvant chemotherapy were analysed for survival outcomes...
November 29, 2016: Pathology
Kathrine Olaussen Eriksen, Navroop Singh Johal, Imran Mushtaq
Minimally invasive surgery (MIS) in the management of malignant and benign renal tumours in children is gradually becoming more common. Experience is limited and restricted to case reports, retrospective chart reviews and a few cohort studies. There are currently no randomized controlled trials or controlled clinical trials comparing the laparoscopic and open surgical approach for the management of renal tumours in children. MIS may offer the same oncologic outcome in malignant renal tumours whilst providing the advantages associated with MIS in correctly selected cases...
October 2016: Translational pediatrics
Rapinder Gupta
No abstract text is available yet for this article.
February 2016: Pathology
Joëlle Marivel, Anabel Kearney, Anna Nelson, Silvia Ling
No abstract text is available yet for this article.
February 2016: Pathology
Surbhi Goyal, Kiran Mishra, Urvee Sarkar, Satendra Sharma, Anita Kumari
BACKGROUND & OBJECTIVES: Renal tumours constitute about 7 per cent of all neoplasms in children. It is important to differentiate Wilms' tumour (commonest tumour) from non-Wilms' tumours. The aim of this study was to evaluate the immunoexpression and diagnostic role of Wilms' tumour-1 protein (WT1) in paediatric renal tumours. METHODS: A total of 53 cases of renal tumours in children (below 18 yr) who underwent total nephrectomy were included in this retrospective study...
May 2016: Indian Journal of Medical Research
Katarzyna Taran, Tomasz FrÄ Czek, Anita Sikora-Szubert, Anna Sitkiewicz, Wojciech Må Ynarski, Józef Kobos, Piotr Paneth
The paper describes a novel approach to investigating Wilms' tumour (nephroblastoma) biology at the atomic level. Isotope Ratio Mass Spectrometry (IRMS) was used to directly assess the isotope ratios of nitrogen and carbon in 84 Wilms' tumour tissue samples from 28 cases representing the histological spectrum of nephroblastoma. Marked differences in nitrogen and carbon isotope ratios were found between nephroblastoma histological types and along the course of cancer disease, with a breakout in isotope ratio of the examined elements in tumour tissue found between stages 2 and 3...
October 8, 2016: Oncotarget
Jacob Freedman, Piotr Harbut
This is a case report of microwave energy being used to ablate an inoperable metastasis of a Wilms' tumour in a 6-year-old boy using state-of-the-art navigated computed tomography targeting and high-frequency jet ventilation to reduce organ displacement and the potential risk of procedure-related pneumothorax. After the ablation, the young boy had high-dose chemotherapy followed by an autologous stem cell transplantation with rapid reduction of three recurrent right-sided lung metastases.
May 2016: Case Reports in Oncology
C E Dunlop, B M Brady, M McLaughlin, E E Telfer, J White, F Cowie, S Zahra, W H B Wallace, R A Anderson
With the improvement of long-term cancer survival rates, growing numbers of female survivors are suffering from treatment-related premature ovarian insufficiency (POI). Although pre-treatment embryo and oocyte storage are effective fertility preservation strategies, they are not possible for pre-pubertal girls or women who cannot delay treatment. In these cases, the only available treatment option is ovarian cortex cryopreservation and subsequent re-implantation. A 32-year-old woman had ovarian cortex cryopreserved 10 years previously before commencing high-dose chemotherapy and undergoing a haematopoietic stem cell transplant for recurrent adult Wilms tumour, which resulted in POI...
September 17, 2016: Journal of Assisted Reproduction and Genetics
Emma Fosbury, Elwira Szychot, Olga Slater, Mary Mathias, Keith Sibson
Wilms tumour (WT) is the commonest primary malignant renal tumour of childhood. Acquired von Willebrand syndrome (avWS) is a well-described paraneoplastic phenomenon, but it is uncommon and may not be detected until clinically significant bleeding is encountered during interventional procedures. Previous studies on small cohorts of patients have determined an incidence of between 4 and 8%. We have performed a retrospective study on cases of WT presenting over an 11.5-year period to a paediatric haematology/oncology unit in a tertiary referral centre to review the incidence of avWS, bleeding phenotype, management, and response to treatment of the primary pathology...
September 12, 2016: Pediatric Blood & Cancer
Kei Kushitani, Vishwa Jeet Amatya, Yasuko Okada, Yuya Katayama, Amany Sayed Mawas, Yoshihiro Miyata, Morihito Okada, Kouki Inai, Takumi Kishimoto, Yukio Takeshima
AIMS: The aim of this study was to clarify the usefulness of immunohistochemistry in the differential diagnosis of epithelioid mesothelioma with solid growth pattern (solid epithelioid mesothelioma; SEM) and poorly differentiated squamous cell carcinoma (PDSCC), and to confirm a specific type of antibody panel. Additionally, we aimed to clarify the pitfalls of immunohistochemical analyses. METHODS AND RESULTS: Formalin-fixed paraffin-embedded specimens from 36 cases of SEM and 38 cases of PDSCC were immunohistochemically examined for calretinin, podoplanin (D2-40), Wilms' tumour gene product (WT1), cytokeratin (CK) 5/6, p40, p63, carcinoembryonic antigen (CEA), epithelial-related antigen (MOC31), claudin-4, thyroid transcription factor (TTF-1), and napsin A...
September 2, 2016: Histopathology
Yi-Xun Liu
Follicular development and differentiation are sequential events which are tightly regulated by endocrine hormones, intraovarian regulators and cell-cell interactions. Balanced cell proliferation and apoptosis play an important role in the selection of dominant follicle. Primordial germ cell migration and homing within the gonadal ridge requires regulation by integrated signals, such as the oocyte-secreted polypeptide growth factors, the growth and differentiation factor 9, the bone morphogenetic proteins, stem cell factor (SCF), basic fibroblast growth factor (bFGF), the transcription factor Wilms' tumour 1 (Wt1), and involves the contact of primordial germ cells with extra-cellular matrix proteins and cellular substrates and attraction by the developing gonads...
August 25, 2016: Sheng Li Xue Bao: [Acta Physiologica Sinica]
Nicola Sgaramella, Eva Lindell Jonsson, Linda Boldrup, Luigi Califano, Philip J Coates, Gianpaolo Tartaro, Lorenzo Lo Muzio, Robin Fåhraeus, Giuseppe Colella, Giovanni Dell'Aversana Orabona, Lotta Loljung, Mario Santagata, Riccardo Rossiello, Torben Wilms, Karin Danielsson, Göran Laurell, Karin Nylander
More than 30% of patients with squamous cell carcinoma (SCC) of the mobile tongue have clinically undetectable lymph node metastasis. Tumour cells can spread as single cells or collectively. A protein known to play a role in both processes is podoplanin, which is expressed in endothelial cells not only in lymph vessels but also in some aggressive tumours with high invasive and metastatic potential. Here we studied samples from 129 patients with primary SCC of the tongue for expression of podoplanin using immunohistochemistry...
January 2016: Journal of Pathology. Clinical Research
Mark Powis
No abstract text is available yet for this article.
November 2016: Pediatric Blood & Cancer
Tasnim Chagtai, Christina Zill, Linda Dainese, Jenny Wegert, Suvi Savola, Sergey Popov, William Mifsud, Gordan Vujanić, Neil Sebire, Yves Le Bouc, Peter F Ambros, Leo Kager, Maureen J O'Sullivan, Annick Blaise, Christophe Bergeron, Linda Holmquist Mengelbier, David Gisselsson, Marcel Kool, Godelieve A M Tytgat, Marry M van den Heuvel-Eibrink, Norbert Graf, Harm van Tinteren, Aurore Coulomb, Manfred Gessler, Richard Dafydd Williams, Kathy Pritchard-Jones
PURPOSE: Wilms tumor (WT) is the most common pediatric renal tumor. Treatment planning under International Society of Paediatric Oncology (SIOP) protocols is based on staging and histologic assessment of response to preoperative chemotherapy. Despite high overall survival (OS), many relapses occur in patients without specific risk factors, and many successfully treated patients are exposed to treatments with significant risks of late effects. To investigate whether molecular biomarkers could improve risk stratification, we assessed 1q status and other potential copy number biomarkers in a large WT series...
September 10, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Jose Aneiros-Fernandez, Juan Antonio Retamero, Husein Husein-ElAhmed, Victor Carriel, Ricardo Ruiz Villaverde, Francisco O'Valle, Jose Aneiros-Cachaza
BACKGROUND: Smoothelin is a specific marker for smooth muscle cells with contractile capacity which has not been widely studied in glomus lesions. In the same way, the expression for Wilms tumor 1 (WT1) has only been studied occasionally in the endothelial cells of glomovenous malformations and in the glomus cells of glomus tumours. OBJECTIVE: We studied the significance of immunohistochemical expression of smoothelin and WT1 in 25 glomus lesions. METHODS: We assessed 9 cases of solid glomus tumors (SGT), 8 cases of glomus tumors with vascular ectasia (VEGT), 2 cases of glomangiomyomas (GMM) and 6 cases of glomuvenous malformation (GM)...
May 17, 2016: Histology and Histopathology
Amit Sharma, Irfan Shaikh, Radhyeshyam Chaudhri, Mukund Andankar, Hemant Pathak
No abstract text is available yet for this article.
March 2016: Journal of Clinical and Diagnostic Research: JCDR
Luciana Mazzei, Walter Manucha
The study of kidney development at the cellular and molecular levels remains an active area of nephrology research. The functional integrity of the kidney depends on normal development as well as on physiological cell turnover. Apoptosis induction is essential for these mechanisms. A route to cell death revealed in the past decade shows that heat shock proteins (HSPs) and their cofactors are responsible for regulating the apoptotic pathway. Specifically, heat shock protein 70 (Hsp70), the most ubiquitous and highly conserved HSP, helps proteins adopt native conformation or regain function after misfolding...
April 8, 2016: Journal of Nephrology
Mallie Abraham, Mathai Samuel, Mohan Mathew
No abstract text is available yet for this article.
March 2016: Indian Journal of Anaesthesia
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