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https://www.readbyqxmd.com/read/28538090/late-effects-in-pediatric-high-risk-neuroblastoma-survivors-after-intensive-induction-chemotherapy-followed-by-myeloablative-consolidation-chemotherapy-and-triple-autologous-stem-cell-transplants
#1
Amy E Armstrong, Karina Danner-Koptik, Shannon Golden, Jennifer Schneiderman, Morris Kletzel, Jennifer Reichek, Yasmin Gosiengfiao
Multimodal treatment in high-risk neuroblastoma has modestly improved survival; limited data exist on the late effects from these regimens. We report the sequelae of treatment incorporating 3 consecutive cycles of high-dose therapy and autologous stem cell transplants (ASCTs) without the use of total body irradiation (TBI). We reviewed the medical records of 61 patients treated on or following the Chicago Pilot 2 protocol between 1991 and 2008. Of the 25 patients who are alive (41%), 19 had near complete data to report...
May 23, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28529554/novel-drugs-in-pediatric-gliomas
#2
Dongli Zhang, Xiaoming Liu, Conghai Fan, Jiao Chen
Astrocytomas (gliomas) are the most common primary brain tumors among adults and second most frequent neoplasm among children. New ideas and novel approaches are being explored world over with aim to devise better management strategeies for this deadly pathological state. We searched the electronic database PubMed for pre-clinical as well as clinical controlled trials reporting importance of various therapeutic drugs against gliomas. It was observed clearly that this approach of using therapeutic drugs is clearly evolving and has been observed to be promising future therapeutic avenue against gliomas...
May 2017: Oncology Letters
https://www.readbyqxmd.com/read/28521633/cd30-expression-in-pediatric-neoplasms-study-of-585-cases
#3
Jinjun Cheng, Haiqing Zhu, John Kim Choi
CD30 is a member of the tumor necrosis factor receptor superfamily, member 8 (TNFRSF8), and its normal expression is restricted to activated T and B cells. In tumor cells, CD30 expression is most commonly associated with lymphoid malignancies (Hodgkin and non-Hodgkin lymphomas) and is a therapeutic target using anti-CD30 antibody. CD30 expression has been reported also in mostly adult non-lymphoid malignancies, raising the possibility of CD30-targeted therapy for additional tumors. In this study, we examined the incidence of CD30 expression in 251 hematopoietic and 334 non-hematopoietic cases of pediatric tumors...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28521631/immunohistochemical-profile-of-myc-protein-in-pediatric-small-round-blue-cell-tumors
#4
Karen M Chisholm, Chandra Krishnan, Amy Heerema-McKenney, Yasodha Natkunam
Deregulation of MYC oncoprotein in cancers can result from multiple oncogenic mechanisms. Although MYC translocations define Burkitt lymphoma and MYC protein expression is a poor prognostic factor in undifferentiated neuroblastomas, the distribution of MYC protein (c-MYC) across other pediatric small round blue cell tumors (SRBCT) has not been well characterized. We undertook this study to assess MYC protein expression in a large cohort of pediatric lymphomas, sarcomas, and other SRBCT. Tissue microarrays containing 302 SRBCT were successfully evaluated by immunohistochemistry using anti-MYC clone Y69, with nuclear positivity scored as 0%, 1%-25%, 26%-50%, 51%-75%, or 76%-100%...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28521629/pediatric-intraocular-immature-teratoma-associated-with-sacrococcygeal-teratoma
#5
Kristen E Zhelnin, Grant M Gebhard, David M Mirsky, Scott Cn Oliver, Mark A Lovell, Csaba Galambos, Timothy M Crombleholme, Emily A McCourt
Intraocular teratomas are rare neoplasms with only three previously reported cases. We present the fourth case of intraocular teratoma and the second associated with sacrococcygeal teratoma. While the nature of the association between intraocular teratomas and sacrococcygeal teratomas is unclear, it suggests a need for careful ophthalmologic follow-up of infants with congenital sacrococcygeal teratomas.
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28521077/clinical-case-series-of-pediatric-hepatic-angiosarcoma
#6
Kalee L Grassia, Caitlin M Peterman, Ionela Iacobas, Judith F Margolin, Ewa Bien, Bhavna Padhye, Rebecka L Meyers, Denise M Adams
Hepatic angiosarcoma is a rare, aggressive, malignant neoplasm with fewer than 50 cases reported in children. Prognosis is poor, with a minority surviving beyond 2 years after diagnosis. We report eight cases of pediatric hepatic angiosarcoma, diagnosed at a mean age of 3 years. Seven were initially diagnosed with an infantile hepatic hemangioendothelioma (IHHE) or hemangioma and the eighth with a "vascular tumor." Two patients, who received liver transplant, survived. We suggest hepatic hemangiomas can rarely transform into angiosarcomas and a subset of IHHEs (Type II) are actually a low-grade form of angiosarcoma rather than a benign lesion...
May 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28502829/primary-ovarian-fibrosarcoma-an-unusual-tumor-in-an-adolescent-case-report-and-review-of-the-pediatric-literature
#7
Bade T Kurtmen, Zafer Dokumcu, Emre Divarci, Gürdeniz Serin, Orkan Ergun, Geylani Ozok, Ahmet Celik
BACKGROUND: Primary ovarian fibrosarcomas are rare and usually observed in perimenopausal and postmenopausal women. Up-to-date, there are only 3 reports of ovarian fibrosarcoma in childhood and adolescence in English literature. In this report, we aimed to present the first pediatric case with advanced staged primary ovarian fibrosarcoma and to compare with previous cases. CASE: A 14-year-old teenage girl was admitted due to a giant abdominal mass. Imaging techniques revealed a giant heterogeneous and vascular ovarian mass...
May 11, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28498454/identification-of-setd2-nf1-fusion-gene-in-a-pediatric-spindle-cell-tumor-with-the-chromosomal-translocation-t-3-17-p21-q12
#8
Ioannis Panagopoulos, Ludmila Gorunova, Ingvild Lobmaier, Bodil Bjerkehagen, Sverre Heim
Spindle cell tumors are clinically heterogeneous but morphologically similar neoplasms. The term refers to the tumor cells' long and slender microscopic appearance. Distinct subgroups of spindle cell tumors are characterized by chromosomal translocations and also fusion genes. Other spindle cell tumors exist that have not yet been found to have characteristic, let alone pathognomonic, genetic or pathogenetic features. Continuous examination of spindle cell tumors is likely to reveal other subgroups that may, in the future, be seen to correspond to meaningful clinical differences and may even be therapeutically decisive...
May 4, 2017: Oncology Reports
https://www.readbyqxmd.com/read/28488624/papillary-craniopharyngioma-a-clinicopathologic-study-of-a-rare-entity-from-a-major-tertiary-care-center-in-pakistan
#9
Muhammad Usman Tariq, Nasir Ud Din, Zubair Ahmad, Wasim Memon
BACKGROUND: Papillary craniopharyngioma (PCP) are uncommon variants of craniopharyngiomas (CP), which are benign epithelial neoplasms of the sellar and suprasellar region. Histologically, PCPs are typically composed of well-differentiated stratified squamous epithelium; however, focal variations are not uncommon. A distinction from other lesions of the region, despite being difficult to achieve due to the overlapping radiological and clinical features, is important for adequate treatment to be administered...
May 2017: Neurology India
https://www.readbyqxmd.com/read/28485351/pediatric-brain-tumors-an-analysis-of-5-years-of-data-from-a-tertiary-cancer-care-center-india
#10
R Madhavan, B P Kannabiran, A M Nithya, J Kani, P Balasubramaniam, S Shanmugakumar
BACKGROUND: Pediatric brain tumors are the most common solid tumors in children and a leading cause of mortality and morbidity in children worldwide. Even though there are enough data about the epidemiology of pediatric brain tumors in western population, there are only a few reports from developing countries like India. AIMS: To study the epidemiological patterns of brain tumors in children, to study the patterns of care, and to assess the treatment response. MATERIALS AND METHODS: A retrospective epidemiological approach is used...
October 2016: Indian Journal of Cancer
https://www.readbyqxmd.com/read/28474981/pediatric-meningiomas-65-year-experience-at-a-single-institution
#11
Andrew J Grossbach, Kelly B Mahaney, Arnold H Menezes
OBJECTIVE Meningiomas are relatively common, typically benign neoplasms in adults; however, they are relatively rare in the pediatric population. Pediatric meningiomas behave very differently from their adult counterparts, tending to have more malignant histological subtypes and recur more frequently. The authors of this paper investigate the risk factors, pathological subtypes, and recurrence rates of pediatric meningiomas. METHODS A retrospective chart review was conducted at the University of Iowa to identify patients 20 years old and younger with meningiomas in the period from 1948 to 2015...
May 5, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28474256/pediatric-imaging-in-dicer1-syndrome
#12
Marta Tijerin Bueno, Claudia Martínez-Ríos, Alejandro De la Puente Gregorio, Rayan A Ahyad, Anita Villani, Harriet Druker, Kalene van Engelen, Bailey Gallinger, Laura Aronoff, Ronald Grant, David Malkin, Mary-Louise C Greer
BACKGROUND: DICER1 syndrome, arising from a mutation in the DICER1 gene mapped to chromosome 14q32, is associated with an increased risk of a range of benign and malignant neoplasms. OBJECTIVE: To determine the spectrum of abnormalities and imaging characteristics in patients with DICER1 syndrome at a tertiary pediatric hospital. MATERIALS AND METHODS: This retrospective analysis evaluated imaging in patients ≤18 years with DICER1 germline variants between January 2004 and July 2016...
May 4, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28468559/pediatric-mesenchymal-hamartomas-of-the-liver-can-show-both-foregut-and-hindgut-phenotype
#13
Hao Wu, William Ferguson, Eumenia Castro, Milton Finegold, Kalyani Patel
Cystic epithelium in mesenchymal hamartoma (MH) is typically biliary type. Mucinous differentiation of the epithelium and increased hepatocellular component may pose a diagnostic challenge. We studied MH in 7 children (6 M, 1 F; age 4 months to 8 years, median 1 year). Resected tumors varied from 3.0 to 17.0 cm. All tumors showed biliary epithelium in the cystic component with strong and diffuse reactivity for CK7 and CK19. Expression of CK20 and CDX2 was additionally seen in 2 tumors, rare/focal in 1, and diffuse with mucinous differentiation in the other...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28464318/examination-of-phox2b-in-adult-neuroendocrine-neoplasms-reveals-relatively-frequent-expression-in-pheochromocytomas-and-paragangliomas
#14
John P Lee, Yin P Hung, Thomas M O'Dorisio, James R Howe, Jason L Hornick, Andrew M Bellizzi
AIMS: Paired-like homeobox 2b (PHOX2B) is a transcription factor with expression outside of the central nervous system restricted to neurons and chromaffin cells of the autonomic nervous system. Germline mutations cause congenital central hypoventilation syndrome and predispose to neuroblastoma and Hirschsprung disease. Among pediatric small round cell tumors, PHOX2B is neuroblastoma-specific. Two studies of adult autonomic nervous system tumors (n=62) produced conflicting results (all tumors stained in one; expression was restricted to 40% of paragangliomas in the other)...
May 2, 2017: Histopathology
https://www.readbyqxmd.com/read/28458966/pediatric-melanoma-a-35-year-population-based-review
#15
Paige H Dean, Marija Bucevska, Caron Strahlendorf, Cynthia Verchere
BACKGROUND: Melanoma is a rare neoplasm in the pediatric population. Recent publications suggest a possible increase in incidence over the past few decades. The purpose of this study was to analyze trends in pediatric patients diagnosed with malignant melanoma in British Columbia (BC) in the past 35 years. METHODS: A retrospective review was performed. All patients in BC diagnosed with melanoma before 18 years of age from 1979 to 2014 were included. Patient demographics, melanoma description, treatment details, and survival data were collected...
March 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28453743/targeted-next-generation-sequencing-of-pediatric-neuro-oncology-patients-improves-diagnosis-identifies-pathogenic-germline-mutations-and-directs-targeted-therapy
#16
Cassie N Kline, Nancy M Joseph, James P Grenert, Jessica van Ziffle, Eric Talevich, Courtney Onodera, Mariam Aboian, Soonmee Cha, David R Raleigh, Steve Braunstein, Joseph Torkildson, David Samuel, Michelle Bloomer, Alejandra G de Alba Campomanes, Anuradha Banerjee, Nicholas Butowski, Corey Raffel, Tarik Tihan, Andrew W Bollen, Joanna J Phillips, W Michael Korn, Iwei Yeh, Boris C Bastian, Nalin Gupta, Sabine Mueller, Arie Perry, Theodore Nicolaides, David A Solomon
Background.: Molecular profiling is revolutionizing cancer diagnostics and leading to personalized therapeutic approaches. Herein we describe our clinical experience performing targeted sequencing for 31 pediatric neuro-oncology patients. Methods.: We sequenced 510 cancer-associated genes from tumor and peripheral blood to identify germline and somatic mutations, structural variants, and copy number changes. Results.: Genomic profiling was performed on 31 patients with tumors including 11 high-grade gliomas, 8 medulloblastomas, 6 low-grade gliomas, 1 embryonal tumor with multilayered rosettes, 1 pineoblastoma, 1 uveal ganglioneuroma, 1 choroid plexus carcinoma, 1 chordoma, and 1 high-grade neuroepithelial tumor...
May 1, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28450183/hematopoietic-stem-cell-transplant-activity-in-pediatric-cancer-between-2008-and-2014-in-the-united-states-a-cibmtr-report
#17
Pooja Khandelwal, Heather R Millard, Elizabeth Thiel, Hisham Abdel-Azim, Allistair A Abraham, Jeffery J Auletta, Farid Boulad, Valerie I Brown, Bruce M Camitta, Ka Wah Chan, Sonali Chaudhury, Morton J Cowan, Miguel Angel-Diaz, Shahinaz M Gadalla, Robert Peter Gale, Gregory Hale, Kimberly A Kasow, Amy K Keating, Carrie L Kitko, Margaret L MacMillan, Richard F Olsson, Kristin M Page, Adriana Seber, Angela R Smith, Anne B Warwick, Baldeep Wirk, Parinda A Mehta
This CIBMTR report describes the use of hematopoietic stem cell transplantation (HSCT) in 4408 pediatric patients with cancer undergoing allogeneic (allo) and 3076 undergoing autologous (auto) HSCT in the United States between 2008 and 2014. In both settings, there was a greater proportion of males (n=4327; 57%), children<10 years of age (n=4412; 59%), Caucasians (n=5787; 77%) and children with a performance score ≥ 90% at HSCT (n=6187; 83%). Leukemia was the most common indication for an allo-transplant (n=4170; 94%), and among these, acute lymphoblastic leukemia (ALL) in second complete remission (n=829; 20%) and acute myeloid leukemia in first complete remission (n=800; 19%) were the most common...
April 24, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28445376/defining-temporal-hairline-landmarks
#18
Purushottam A Nagarkar, Jonathan Cheng
Brow dermoids are benign pediatric neoplasms. Endoscopic resection requires incisions placed within the temporal hairline, which is often difficult to reliably locate in infants. The authors studied adult and pediatric patients to define the location of the hairline in relationship to simple facial landmarks. Adult and pediatric patients who were seen in preoperative consultation for facial surgical procedures were identified and included in the study. Patients with a history of facial trauma, surgery, or congenital anomalies were excluded...
May 2017: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28434637/clinical-heterogeneity-in-children-with-gonadal-dysgenesis-associated-with-non-mosaic-46-xy-karyotype
#19
Y S Wong, Y H Tam, K K Y Pang, K F To, S S C Chan, K W Chan, K H Lee
INTRODUCTION: Gonadal dysgenesis is unique in disorders of sex development (DSD), in that it can be associated with 46,XX, 46,XY or mosaic 45,X/46,XY karyotypes. Gonadal dysgenesis can be partial or complete. Gonadal dysgenesis associated with the Y-chromosome has increased risk of gonadal germ cell neoplasms. Most of the literature focus on 45,X/46,XY gonadal dysgenesis, while there are scanty data on the condition when the karyotype is non-mosaic 46,XY. OBJECTIVE: To investigate the diversity of clinical pictures of children presenting with 46,XY DSD due to gonadal dysgenesis...
April 10, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28431179/mirna-regulation-in-gliomas-usual-suspects-in-glial-tumorigenesis-and-evolving-clinical-applications
#20
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
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