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https://www.readbyqxmd.com/read/28431179/mirna-regulation-in-gliomas-usual-suspects-in-glial-tumorigenesis-and-evolving-clinical-applications
#1
Heather Ames, Marc K Halushka, Fausto J Rodriguez
In recent years, an increasing role for noncoding small RNAs (miRNA) has been uncovered in carcinogenesis. These oligonucleotides can promote degradation and/or inhibit translation of key mRNAs. Recent studies have also highlighted a possible role for miRNAs in adult and pediatric brain tumors, including high- and low-grade gliomas, medulloblastoma, ependymoma, and neoplasms associated with neurofibromatosis type 1. Gliomas represent the most common category of primary intraparenchymal brain tumors, and, for example, manipulation of signaling pathways, through inhibition of PTEN transcription appears to be an important function of miRNA dysregulation through miR-21, miR-106b, and miR-26a...
April 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28429507/mucinous-hidradenoma-in-a-child-a-case-report-and-review-of-the-literature
#2
Aadil Ahmed, Wendy Kim, Jodi Speiser
Hidradenomas are benign tumors of the sweat glands that are rarely reported in childhood. The presence of mucinous change in hidradenomas, which consists of variable numbers of mucin-rich goblet cells, is occasionally observed in some adult clinical practices. However, it has not been previously reported in the pediatric population. Herein, we present a unique case of a mucinous hidradenoma presenting on the thigh of a 3-year old boy. The clinical presentation and histological features of the case, along with a literature review of published case reports of pediatric hidradenomas are described...
April 21, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28426850/second-primary-malignant-neoplasms-and-survival-in-adolescent-and-young-adult-cancer-survivors
#3
Theresa H M Keegan, Archie Bleyer, Aaron S Rosenberg, Qian Li, Melanie Goldfarb
Importance: Although the increased incidence of second primary malignant neoplasms (SPMs) is a well-known late effect after cancer, few studies have compared survival after an SPM to survival of the same cancer occurring as first primary malignant neoplasm (PM) by age. Objective: To assess the survival impact of SPMs in adolescents and young adults (AYAs) (15-39 years) compared with that of pediatric (<15 years) and older adult (≥40 years) patients with the same SPMs...
April 20, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28426529/diagnostic-utility-of-pax8-pax2-and-ngfr-immunohistochemical-expression-in-pediatric-renal-tumors
#4
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/28412288/adult-t-type-lymphoblastic-lymphoma-treatment-advances-and-prognostic-indicators
#5
REVIEW
Stéphane Lepretre, Carlos Graux, Aurore Touzart, Elizabeth Macintyre, Nicolas Boissel
T-cell lymphoblastic lymphoma (T-LBL) is a rare, aggressive neoplasm of precursor T cells that occurs mostly in adolescents and young adults. In this review, we describe the treatment of adult T-LBL with a focus on recent advances using pediatric-inspired acute lymphoblastic leukemia regimens, which have greatly improved outcome. We also discuss the development of prognostic indicators for T-LBL, especially oncogenetic factors, that can identify patients at higher risk of relapse and should help further extend T-LBL patient survival...
April 12, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28407793/the-diagnosis-and-management-of-the-spitz-nevus-in-the-pediatric-population-a-systematic-review-and-meta-analysis%C3%A2-protocol
#6
Jean Abboud, Michael Stein, Michele Ramien, Claudia Malic
BACKGROUND: Spitz nevi are uncommon melanocytic neoplasms found in children. Historically, the diagnosis and management of these tumors has lacked consensus among oncologists, pathologists, plastic surgeons, and dermatologists. Once interpreted and treated as a "juvenile melanoma", many have argued for the benignancy of such tumors in certain patient age groups, encouraging a conservative approach. The lack of consensus surrounding the diagnosis and perceived malignant potential of these tumors has led physicians to approach them on a case-by-case basis and institutional protocols...
April 13, 2017: Systematic Reviews
https://www.readbyqxmd.com/read/28405541/it-s-rare-so-be-aware-pleuropulmonary-blastoma-mimicking-congenital-pulmonary-airway-malformation
#7
Fayza Haider, Khulood Al Saad, Fatima Al-Hashimi, Hakima Al-Hashimi
Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of infancy and early childhood. The tumor arises in the lung and pleura and is regarded as a pulmonary dysontogenetic or embryonic neoplasm. Four types are defined in literature. Type I PPB is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced with a poor prognosis. We report this case to increase awareness about this entity so that the pediatricians, pediatric surgeons, radiologist, and pathologist recognize it early...
January 2017: Thoracic and Cardiovascular Surgeon Reports
https://www.readbyqxmd.com/read/28401334/h3-idh-wild-type-pediatric-glioblastoma-is-comprised-of-molecularly-and-prognostically-distinct-subtypes-with-associated-oncogenic-drivers
#8
Andrey Korshunov, Daniel Schrimpf, Marina Ryzhova, Dominik Sturm, Lukas Chavez, Volker Hovestadt, Tanvi Sharma, Antje Habel, Anna Burford, Chris Jones, Olga Zheludkova, Ella Kumirova, Christof M Kramm, Andrey Golanov, David Capper, Andreas von Deimling, Stefan M Pfister, David T W Jones
Pediatric glioblastoma (pedGBM) is an extremely aggressive pediatric brain tumor, accounting for ~6% of all central nervous system neoplasms in children. Approximately half of pedGBM harbor recurrent somatic mutations in histone 3 variants or, infrequently, IDH1/2. The remaining subset of pedGBM is highly heterogeneous, and displays a variety of genomic and epigenetic features. In the current study, we aimed to further stratify an H3-/IDH-wild type (wt) pedGBM cohort assessed through genome-wide molecular profiling...
April 11, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28377665/peripheral-odontogenic-fibroma-a-rare-tumor-mimicking-a-gingival-reactive-lesion
#9
Komal Khot, Swati Deshmane, Kriti Bagri-Manjrekar, Paresh Khot
Gingival growths are one of the most frequently encountered lesions in the oral cavity. A plethora of lesions can be seen having similar clinical presentation, making diagnosis a dilemma. Peripheral odontogenic tumors are rare neoplasms to occur on gingiva, the most common among them being the peripheral odontogenic fibroma (POdF). The POdF is a benign, slow-growing, exophytic lesion. Although considered to have a recurrence potential after excision, the actual recurrence rate is not known due to paucity of reported cases...
January 2017: International Journal of Clinical Pediatric Dentistry
https://www.readbyqxmd.com/read/28377663/pediatric-palatal-fibroma
#10
Rahul Mishra, Tayyeb S Khan, Tarannum Ajaz, Mamta Agarwal
Fibroma is one of the most common soft tissue benign tumors of the oral cavity. These masses represent hyperplasias instead of true neoplasm, which develop due to irritation to the mucosal tissue resulting in proliferation of the cells. Although so common in the oral cavity, its occurrence on the palate is rare, mainly due to fewer chances of trauma. Here, we report a case of palatal fibroma in a child diagnosed on the basis of clinical, radiological, and histological features. The case represents an extremely rare occurrence as unusual trauma due to thumb sucking seemed to be the only apparent traumatic factor in the palatal region...
January 2017: International Journal of Clinical Pediatric Dentistry
https://www.readbyqxmd.com/read/28363472/surgical-management-and-surveillance-of-pediatric-appendiceal-carcinoid-tumor
#11
Inna N Lobeck, Neelum Jeste, James Geller, Joseph Pressey, Daniel von Allmen
PURPOSE: Appendiceal carcinoid tumors are rare neuroendocrine neoplasms. The aim of this study was to determine if postoperative oncologic follow-up was necessary for this tumor. METHODS: A retrospective review was performed of patients with appendiceal carcinoid 2000-2015. RESULTS: 8382 patients underwent appendectomy 2000-2015. 30 (0.3%) had appendiceal carcinoid. 70% were female (n=21) with an average age of 13.5±2.8 years (range 8-18)...
March 15, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28362182/pediatric-spinal-cord-astrocytomas-a-retrospective-study-of-348-patients-from-the-seer-database
#12
Andrew S Luksik, Tomas Garzon-Muvdi, Wuyang Yang, Judy Huang, George I Jallo
OBJECTIVE Intramedullary spinal cord tumors comprise 1%-10% of all childhood central nervous system neoplasms, with astrocytomas representing the most common subtype. Due to their rarity and poor prognosis, large population-based studies are needed to assess the epidemiology and survival risk factors associated with these tumors in the hope of improving outcome. The authors undertook this retrospective study to explore factors that may influence survival in pediatric patients with spinal cord astrocytomas. METHODS Utilizing the Surveillance, Epidemiology, and End Results (SEER) database, a prospective cancer registry, the authors retrospectively assessed survival in histologically confirmed, primary spinal cord astrocytomas in patients 21 years of age and younger...
March 31, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28357093/triple-phase-99m-tc-3p-rgd2-imaging-of-peripheral-primitive-neuroectodermal-tumor-in-the-hip-muscle-group-with-bone-metastasis
#13
Jingjing Fu, Jinhua Song, Youcai Zhao, Feng Wang, Guoqiang Shao
Peripheral primitive neuroectodermal tumors (pPNETs) are a group of aggressive neoplasms that are most commonly encountered in pediatric patients and may be located in the abdomen, pelvis, thoracopulmonary region and, rarely, in the head and neck region. pPNETs in adults are extremely rare. The present study reports a case of pPNET located in the hip muscles with bone metastasis. The patient was a 44-year-old woman who complained of progressive pain and swelling with a mass near the left hip. Computed tomography (CT) and enhanced CT revealed a soft tissue mass lesion in the hip muscle group measuring 4...
February 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28347527/thyroid-neoplasms-incidental-findings-on-extent-of-disease-evaluation-ct-for-other-pediatric-malignancies
#14
William J Hammond, Todd E Heaton, Benjamin A Farber, Usman A Mahmood, Jill S Gluskin, Suzanne L Wolden, Michael P La Quaglia, Charles A Sklar, Anita P Price
PURPOSE: We performed a retrospective analysis to evaluate the risk of thyroid cancer in incidental thyroid nodules (ITNs) discovered on CT in patients with a history of pediatric cancer. METHODS: With IRB approval we reviewed the records of pediatric oncology patients age ≤21y with newly detected thyroid nodules on surveillance CT of the neck, chest, chest/abdomen/pelvis, or PET/CT performed between April 2008 and March 2015. Patients with <6months of follow-up after incidental findings, a history of primary thyroid malignancy, or incomplete records were excluded...
March 15, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28343661/variability-in-surgical-management-of-benign-ovarian-neoplasms-in-children
#15
Dani O Gonzalez, Jennifer N Cooper, Jennifer H Aldrink, Geri D Hewitt, Mary E Fallat, Peter C Minneci, Katherine J Deans
BACKGROUND/PURPOSE: Although most pediatric ovarian neoplasms are benign and may be treated with ovary-sparing surgery (OSS), consensus is lacking on the optimal surgical approach. We aimed to determine the proportion of pediatric benign ovarian neoplasms managed with OSS and to assess variability in management across hospitals and specialties. METHODS: Using the Pediatric Health Information System, we studied patients aged 6-21 years treated in 2006-2014 for a benign ovarian neoplasm with oophorectomy or OSS...
March 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28333115/modeling-and-targeting-myc-genes-in-childhood-brain-tumors
#16
REVIEW
Sonja Hutter, Sara Bolin, Holger Weishaupt, Fredrik J Swartling
Brain tumors are the second most common group of childhood cancers, accounting for about 20%-25% of all pediatric tumors. Deregulated expression of the MYC family of transcription factors, particularly c-MYC and MYCN genes, has been found in many of these neoplasms, and their expression levels are often correlated with poor prognosis. Elevated c-MYC/MYCN initiates and drives tumorigenesis in many in vivo model systems of pediatric brain tumors. Therefore, inhibition of their oncogenic function is an attractive therapeutic target...
March 23, 2017: Genes
https://www.readbyqxmd.com/read/28327291/solid-pseudopapillary-neoplasm-of-the-pancreas-a-clinicopathological-review-of-20-cases-including-rare-examples
#17
Ayca Ersen, Anil Aysal Agalar, Erdener Ozer, Cihan Agalar, Tarkan Unek, Tufan Egeli, Mucahit Ozbilgin, Ibrahim Astarcioglu, Mustafa Olguner, Funda Obuz, Ozgul Sagol
AIMS: Solid-pseudopapillary neoplasm (SPN) is an uncommon malignant tumor of the pancreas with a favorable prognosis unlike other pancreatic neoplasms. We investigated the clinicopathological features of 20 patients with SPN in details. METHODS: The patients diagnosed as SPN in Dokuz Eylul University Hospital between January 2005 and March 2016 were reviewed in terms of clinical and histopathological data. RESULTS: Mean age of the patients was 33...
November 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28326824/osteopontin-its-potential-role-in-cancer-of-children-and-young-adults
#18
Gabrielle Karpinsky, Aleksandra Fatyga, Malgorzata Anna Krawczyk, Madeleine Chamera, Natalia Sande, Dagmara Szmyd, Ewa Izycka-Swieszewska, Ewa Bien
OBJECTIVE: Osteopontin (OPN) is aglyco-phosphoprotein, involved in tissue remodeling, inflammation and boneresorption. In various adult neoplasms OPN was shown to correlate with cancer progression, invasiveness and metastasis. AIM: to define the role of OPN in malignancies of children and young adults. MATERIAL AND METHODS: a structured PubMed and Google Scholar literature analysis based on reports published in English between I'1995 and XII'2015...
March 14, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/28322484/tfe3-positive-renal-cell-carcinoma-occurring-in-three-children-with-dysfunctional-kidneys-on-immunosuppression
#19
Beverly A Schaefer, Theodore S Johnson, David K Hooper, Jaimie D Nathan, James I Geller
Pediatric RCC is a rare pediatric neoplasm and is distinctly different compared to adult RCC, often demonstrating translocation morphology evidenced by unique histopathological features and TFE3 or TFEB nuclear expression. We report three cases of pediatric TFE3 positive RCC (TFE3-RCC) occurring in the setting of chronic kidney disease and long-term pharmacological immunosuppression, including two cases that developed in the native kidney following kidney transplantation. Together, these cases suggest that the kidney microenvironment in combination with immune dysregulation is likely contributing factors in the pathogenesis of some pediatric RCC, warranting further study...
March 21, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28321121/dnmt3a-regulates-t-cell-development-and-suppresses-t-all-transformation
#20
A C Kramer, A Kothari, W C Wilson, H Celik, J Nikitas, C Mallaney, E L Ostrander, E Eultgen, A Martens, M C Valentine, A L Young, T E Druley, M E Figueroa, B Zhang, G A Challen
T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive hematopoietic neoplasm resulting from the malignant transformation of T-cell progenitors, and comprises ~15% and 25% of pediatric and adult ALL cases, respectively. It is well-established that activating NOTCH1 mutations are the major genetic lesions driving T-ALL in most patients, but efforts to develop targeted therapies against this pathway have produced limited success in decreasing leukemic burden and come with significant clinical side effects...
April 11, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
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