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Mesoblastic nephroma

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https://www.readbyqxmd.com/read/29733801/evaluation-of-renal-tumors-in-children
#1
Gülçin Bozlu, Elvan Çağlar Çıtak
OBJECTIVE: Renal tumors are not uncommon in children. In this study, we aimed to evaluate the clinical and pathological features of renal tumors in children. MATERIAL AND METHODS: Between January 2008 and December 2017, the records of children with renal tumors in our institution were retrospectively analyzed. Data collected were composed of demographic and clinical characteristics including gender, age at time of diagnosis, symptoms, laterality of the tumor and pathological evaluation...
May 2018: Turkish Journal of Urology
https://www.readbyqxmd.com/read/29683818/pan-trk-immunohistochemistry-identifies-ntrk-rearrangements-in-pediatric-mesenchymal-tumors
#2
Erin R Rudzinski, Christina M Lockwood, Bradley A Stohr, Sara O Vargas, Rachel Sheridan, Jennifer O Black, Veena Rajaram, Theodore W Laetsch, Jessica L Davis
Activating neurotrophic receptor kinase (NTRK) fusions define certain pediatric mesenchymal tumors, including infantile fibrosarcoma and cellular mesoblastic nephroma. Traditionally, molecular confirmation of these fusions has included either fluorescent in situ hybridization for ETV6 rearrangements or reverse-transcriptase polymerase chain reaction for the classic ETV6-NTRK3 fusion. However, these methods overlook variant NTRK rearrangements, which are increasingly appreciated as recurrent events in a subset of pediatric mesenchymal tumors...
April 20, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29681695/use-of-pretherapy-core-biopsy-in-the-diagnosis-of-pediatric-renal-tumors
#3
Jujju Jacob Kurian, Kenneth R L Nongpiur, Susan Jehangir
Background: Pretreatment core biopsy of pediatric renal tumors has been advocated by United Kingdom Children's Cancer Study Group to circumvent the disadvantage of International Society of Paediatric Oncology protocol, where neoadjuvant chemotherapy initiated without histopathological confirmation can result in over- or under-treatment. Aim: This study aims (a) to assess if pretherapy core biopsy correlates with the nephrectomy biopsy; (b) to assess if neoadjuvant chemotherapy changes Wilms tumor (WT) histology, and (c) to assess the incidence of biopsy site recurrence...
April 2018: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/29286563/etv6-ntrk3-in-congenital-mesoblastic-nephroma-a-report-of-the-siop-gpoh-nephroblastoma-study
#4
Christian Vokuhl, Nasenien Nourkami-Tutdibi, Rhoikos Furtwängler, Manfred Gessler, Norbert Graf, Ivo Leuschner
BACKGROUND: Congenital mesoblastic nephroma (MN) is a rare pediatric renal tumor representing approximately 5% of all pediatric renal tumors. Three different types of MN are distinguished histologically: classical, cellular, and mixed. A frequent genetic alteration is the translocation t(12;15) resulting in a fusion of the ETV6 gene on 12p13 and the NTRK3 gene on 15p15 that occurs almost exclusively in cellular MN. The aim of this study was to determine translocation status of a large cohort of MN with respect to tumor subtype and outcome...
April 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29200690/unusual-presentation-of-metanephric-stromal-tumor-in-a-neonate
#5
Prashant Sadashiv Patil, Abhaya Gupta, Rahul Gupta, Paras L Kothari, Geeta Kekre, Kedar Mudkhedkar, Vishesh Dikshit
Metanephric stromal tumor (MST) of kidney is an under-reported benign stromal specific renal neoplasm with good prognosis. This tumor is to be differentiated from congenital mesoblastic nephroma and clear-cell sarcoma of the kidney. In this case report, we describe the imaging, gross, and microscopic features of MST with ischemic left kidney in a 1-month-old child and discuss treatment with relevant literature.
July 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29099503/recurrent-eml4-ntrk3-fusions-in-infantile-fibrosarcoma-and-congenital-mesoblastic-nephroma-suggest-a-revised-testing-strategy
#6
Alanna J Church, Monica L Calicchio, Valentina Nardi, Alena Skalova, Andre Pinto, Deborah A Dillon, Carmen R Gomez-Fernandez, Namitha Manoj, Josh D Haimes, Joshua A Stahl, Filemon S Dela Cruz, Sarah Tannenbaum-Dvir, Julia L Glade-Bender, Andrew L Kung, Steven G DuBois, Harry P Kozakewich, Katherine A Janeway, Antonio R Perez-Atayde, Marian H Harris
Infantile fibrosarcoma and congenital mesoblastic nephroma are tumors of infancy traditionally associated with the ETV6-NTRK3 gene fusion. However, a number of case reports have identified variant fusions in these tumors. In order to assess the frequency of variant NTRK3 fusions, and in particular whether the recently identified EML4-NTRK3 fusion is recurrent, 63 archival cases of infantile fibrosarcoma, congenital mesoblastic nephroma, mammary analog secretory carcinoma and secretory breast carcinoma (tumor types that are known to carry recurrent ETV6-NTRK3 fusions) were tested with NTRK3 break-apart FISH, EML4-NTRK3 dual fusion FISH, and targeted RNA sequencing...
March 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28856451/recurrent-and-metastatic-congenital-mesoblastic-nephroma-where-does-the-evidence-stand
#7
Susan Jehangir, Jujju J Kurian, Dharshini Selvarajah, Reju J Thomas, Andrew J A Holland
PURPOSE: Fifty years ago, Bolande described Congenital Mesoblastic Nephroma (CMN) as a benign lesion. Unexpected aggressive clinical behaviors prompted a sub-classification based on histology. Recent molecular genetic evidence has identified the aggressive cellular variant to be the renal manifestation of congenital infantile fibrosarcoma. We submit a reappraisal and analysis of the available literature on recurrent and metastatic CMN. METHODS: An electronic search of PubMed, MEDLINE, EMBASE, and Scopus yielded 38 children with local recurrence and/or metastases...
November 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28770142/cellular-congenital-mesoblastic-nephroma-in-a-newborn
#8
Prasanta Kumar Tripathy, Susmita Behera, Hiranya K Mohanty
No abstract text is available yet for this article.
April 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28736178/neonatal-solid-tumors
#9
Aravindan Chandrasekaran
BACKGROUND: Neonatal tumors are different from tumors of the older children and knowledge gained from treating older children can not be extrapolated to neonates. Neonates have immature physiology and their haematopoietic and immune systems are not fully developed and the response to therapy is unpredictable. Hence it is imperative to study these tumors as separate entity. The aim of this study is to analyse this rare set of tumors in terms of their incidence, clinical features and management...
February 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28643124/-solid-pediatric-tumors-a-brief-survey-of-the-rarity-cabinet
#10
B Gürtl-Lackner, D Gisselsson-Nord, G Vujanic
Solid tumors in childhood are extremely rare entities, which are usually treated in specialized centers. Diagnosis and therapy are carried out according to a joint European protocol, whereby the pathological evaluation and therapy are carried out according to international guidelines. For the correct diagnosis and/or therapy of most tumors, analysis of specific genetic changes is mandatory; therefore, tumors have to be adequately sampled for parallel genetic analysis during the pathological work-up. A second opinion reference of the histopathological assessment is part of the international guidelines...
July 2017: Der Pathologe
https://www.readbyqxmd.com/read/28426529/diagnostic-utility-of-pax8-pax2-and-ngfr-immunohistochemical-expression-in-pediatric-renal-tumors
#11
Nicoleta C Arva, Jeffrey Bonadio, Elizabeth J Perlman, Mariana M Cajaiba
Pediatric renal tumors (PRT) with small round blue or spindle cell morphology can be diagnostically challenging and only a limited number of immunohistochemical markers have been documented to help in the diagnosis: paired box (Pax) 2 and nerve growth factor receptor (NGFR) positivity have been demonstrated in Wilms tumor (WT) and clear cell sarcoma of the kidney (CCSK), respectively. However, the immunohistochemical expression of these markers in other PRT remains unknown. This study investigated Pax8, Pax2, and NGFR immunophenotype in a large series of PRT...
April 19, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28303733/bi-parametric-magnetic-resonance-imaging-applied-to-obstetrics
#12
Lucia Manganaro, Valeria Vinci, Antonella Giancotti, Sandro Gerli, Denis A Cozzi, Teresa Pusiol, Michele Scialpi, Luca Roncati
Foetal magnetic resonance imaging (MRI) plays an increasingly important role in the diagnosis of foetal abnormalities. Over the years, we have successfully applied bi-parametric MRI (bp-MRI) to the following obstetric conditions: (1) neurologic vascular diseases; (2) assessment of lung parenchyma maturation; (3) renal pathologies, such as polycystic kidney, suspected renal infarction, unilateral or bilateral renal agenesis; (4) placental pathologies, as twin-twin transfusion syndrome or placenta accreta; (5) benignant and malignant congenital tumours or cysts of the liver, such as haemangioendothelioma, hepatoblastoma or metastatic neuroblastoma, of the kidney (e...
July 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28282819/management-of-mixed-type-congenital-mesoblastic-nephroma-case-series-and-review-of-the-literature
#13
REVIEW
J Daniel, A Ruzic, J Dalland, V Miller, M Hanna
Congenital mesoblastic nephroma (CMN) is the most common renal tumor of infancy; however, it occurs infrequently with an incidence of 1 : 125,000. The cellular and classical variants are the most common subtypes of tumors, with a mixed variant occurring infrequently. We describe two cases of mixed variant CMN, which presented within days of each other differing in their clinical behavior. The first case followed a typical course, previously described in the literature, while the other deviated significantly...
2017: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/28137351/congenital-mesoblastic-nephroma-presenting-with-refractory-hypertension-in-a-premature-neonate-a-case-study
#14
Tracey Robertson-Bell, Desi M Newberry, Amy J Jnah, Stephen D DeMeo
The most common nonencapsulated solid renal tumor in the neonatal period is congenital mesoblastic nephroma. Mesoblastic nephroma is a solid lesion originating within or extending from the renal parenchyma. These tumors proliferate rapidly, typically within 3-6 months after birth. Mesoblastic nephromas are stratified by classification as either classical (benign) or atypical (malignant); masses composed of both benign and malignant cells are also reported. The hallmark clinical manifestation of mesoblastic nephroma is a palpable abdominal mass, which may be accompanied by hypertension, hypercalcemia, hematuria, and polyuria...
January 1, 2017: Neonatal Network: NN
https://www.readbyqxmd.com/read/28124468/congenital-mesoblastic-nephroma-50-years-after-its-recognition-a-narrative-review
#15
REVIEW
S L Gooskens, M E Houwing, G M Vujanic, J S Dome, T Diertens, A Coulomb-l'Herminé, J Godzinski, K Pritchard-Jones, N Graf, M M van den Heuvel-Eibrink
Congenital mesoblastic nephroma (CMN) is a rare pediatric renal tumor with low malignant potential that most commonly occurs early in infancy. Treatment strategies are based on the few published CMN series, while a significant number of CMN patients have been described in case reports. The aim of this narrative review was to create an up-to-date overview of the literature. Complete surgical removal is curative in most cases. The risk of treatment-related mortality (both surgery- and chemotherapy-related) is relatively high in the first weeks of life, indicating that these young patients deserve special attention with respect to timing and type of treatment...
July 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27820132/renal-tumors-in-children-younger-than-12-months-of-age-a-65-year-single-institution-review
#16
Margaret G Lamb, Jennifer H Aldrink, Sarah H O'Brien, Han Yin, Michael A Arnold, Mark A Ranalli
Wilms tumor (WT) is the most prevalent pediatric renal tumor and most commonly occurs between ages 1 and 5 years. Data are lacking on children younger than 12 months with renal tumors. The cancer registry at the authors' institution was queried to identify patients 12 months and younger with renal masses. Demographics, clinical presentation, histopathology, stage, and survival outcomes were reviewed. The most common presenting symptoms included an asymptomatic abdominal mass (73%) and hematuria (9%). Histopathology revealed WT in 73% of patients, mesoblastic nephroma in 20%...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27769870/braf-exon-15-mutations-in-pediatric-renal-stromal-tumors-prevalence-in-metanephric-stromal-tumors
#17
Lily Marsden, Lawrence J Jennings, Samantha Gadd, Min Yu, Elizabeth J Perlman, Mariana M Cajaiba
Metanephric stromal tumors (MSTs) are rare renal stromal tumors that predominantly affect children. They belong to the metanephric family of tumors, along with metanephric adenofibroma and metanephric adenoma. The previous documentation of BRAF exon 15 mutations in 88% of metanephric adenomas and in isolated cases of metanephric adenofibroma prompted us to investigate the prevalence of these mutations in MSTs and in other pediatric renal stromal tumors. In this study, 17 MSTs, 22 congenital mesoblastic nephromas, and 6 ossifying renal tumors of infancy were selected for BRAF exon 15 testing...
February 2017: Human Pathology
https://www.readbyqxmd.com/read/27748279/diagnostic-utility-of-wilms-tumour-1-protein-wt-1-immunostaining-in-paediatric-renal-tumours
#18
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
https://www.readbyqxmd.com/read/27646897/-congenital-mesoblastic-nephroma-a-clinicopathologic-analysis-of-17-cases
#19
H Wang, Y Y Ma, D W Zhang, B Y Wu, C Fang, L Chen
No abstract text is available yet for this article.
September 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/27484189/the-value-of-mitotic-count-and-ki67-proliferation-index-in-congenital-mesoblastic-nephroma
#20
Evsen Apaydin, Yasemin Ozluk, Secil Yuksel, Basak Erginel, Deniz Tugcu, Alaaddin Celik, Isin Kilicaslan
OBJECTIVE: We aimed to define the histopathologic features and proliferative rate of congenital mesoblastic nephroma (CMN) as a risk factor for recurrence. METHODS: Fourteen cases of CMN among 138 registered pediatric renal tumors were retrospectively reviewed. The prognostic impact for mitotic rate and Ki67 index was investigated. RESULTS: There were four (28.6%) classic, six (42.9%) cellular, and four (28.6%) mixed type CMNs, with average Ki-67 counts of 16...
2016: Fetal and Pediatric Pathology
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