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pediatric neoplasm

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https://www.readbyqxmd.com/read/29454577/morphological-clues-to-the-appropriate-recognition-of-hereditary-renal-neoplasms
#1
REVIEW
Holger Moch, Riuko Ohashi, Jatin S Gandhi, Mahul B Amin
An important emerging role of the surgical pathologist besides the traditional tasks of establishment of the diagnosis and documentation of prognostic and predictive factors, is to recognize the possibility of a hereditary condition in cases where the histology is suggestive for a familial cancer syndrome. In recent years, the knowledge regarding all of the above roles, including the role of recognition of familial cancer, has particularly expanded in renal neoplasms with the close scrutiny to morphology, molecular correlates and clinical features of the different sub-types of renal cell carcinoma...
February 14, 2018: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/29453753/pediatric-astrocytic-tumor-grading-comparison-between-arterial-spin-labeling-and-dynamic-susceptibility-contrast-mri-perfusion
#2
Giovanni Morana, Domenico Tortora, Serena Staglianò, Paolo Nozza, Samantha Mascelli, Mariasavina Severino, Gianluca Piatelli, Alessandro Consales, Maarten Lequin, Maria Luisa Garrè, Andrea Rossi
PURPOSE: The aim of this study was to compare arterial spin labeling (ASL) and dynamic susceptibility contrast (DSC) MRI perfusion with respect to diagnostic performance in tumor grading in pediatric patients with low- and high-grade astrocytic tumors (AT). METHODS: We retrospectively analyzed 37 children with histologically proven treatment naive low- and high-grade AT who underwent concomitant pre-operative ASL and DSC MRI perfusion. Studies were performed on a 1...
February 16, 2018: Neuroradiology
https://www.readbyqxmd.com/read/29438212/maxillo-mandibular-reconstruction-in-pediatric-patients-how-to-do-it
#3
Valentino Valentini, Luigi Califano, Andrea Cassoni, Della Monaca Marco, Ingrid Raponi, Paolo Priore, Maria Teresa Fadda, Giovanni Dell'Aversana Orabona, Valentina Terenzi
Maxillo-mandibular reconstruction in pediatric patients involves particular functional and cosmetic implications. Attention is required for the craniofacial growth over time, involvement of the permanent dentition, facial symmetry, and donor site morbidity. Our aim is to identify the best reconstructive options for maxilla-mandibular defects in children (<18 years) based on our experience with 25 pediatric patients. We believe that reconstruction has to be performed at the same time as resection in pediatric patients...
February 12, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29436012/skin-lesions-serve-as-clues-to-relapse-of-pediatric-blastic-plasmacytoid-dendritic-cell-neoplasm
#4
Sean Dreyer, Suzanne Mednik, Allison Truong, Scott Worswick, Philip Scumpia, Daniel Neill, Swati Kannan, Marcia Hogeling
A 10-year-old girl with a history of blastic plasmacytoid dendritic cell neoplasm, a rare malignancy in children, presented with recurrent skin eruptions beginning while on maintenance chemotherapy, including mildly pruritic skin-colored plaques, tender indurated nodules, and violaceous bound-down plaques. This case highlights an unusual presentation of relapsed blastic plasmacytoid dendritic cell neoplasm on chemotherapy, with skin lesions providing important clues to the progression of systemic disease.
February 13, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29434830/pediatric-sarcomas
#5
Junhua Cao, Qi An, Lei Wang
Sarcomas arise from primitive mesenchymal cells, which are classified, into two main groups: Bone and soft tissue sarcomas. We have searched all-important electronic databases including Google scholar and PubMed for the collection of latest literature pertaining to pediatric sarcomas. Latest literature confirmed that these tumors are relatively rare and represent only 1% of all malignancies but they have higher incidence in children. Pediatric sarcomas comprise about 13% of all pediatric malignancies and are ranked third in childhood cancers...
February 2018: Oncology Letters
https://www.readbyqxmd.com/read/29426273/testosterone-and-cortisol-secreting-oncocytic-adrenocortical-adenoma-in-the-pediatric-age-group
#6
Maryam K Al Badi, Ibrahim Al-Alwan, Mohammed Al-Dubayee, Ayed Al-Anzi, Maram S Al Turki, Noura Aloudah, Khaled O Alsaad
Oncocytic tumors are epithelial neoplasms that occur in various organs, including adrenal glands. Oncocytic adrenocortical adenomas and carcinomas are uncommon but well-known pathological entities in adults. However, generally oncocytic tumors, particularly in the adrenal glands, are very rare in the pediatric age-group. Most oncocytic adrenal tumors are not functional. We present a rare case of right-sided, functional oncocytic adrenocortical adenoma in a 5-year-old boy, who presented with clinical manifestations of precocious puberty and Cushing syndrome...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29405993/molecular-approaches-identify-a-cryptic-mecom-rearrangement-in-a-child-with-a-rapidly-progressive-myeloid-neoplasm
#7
Roberto R Capela de Matos, Moneeb A K Othman, Gerson M Ferreira, Elaine S Costa, Joana B Melo, Isabel M Carreira, Mariana T de Souza, Bruno A Lopes, Mariana Emerenciano, Marcelo G P Land, Thomas Liehr, Raul C Ribeiro, Maria Luiza M Silva
Myeloid neoplasms are a heterogeneous group of hematologic disorders with divergent patterns of cell differentiation and proliferation, as well as divergent clinical courses. Rare recurrent genetic abnormalities related to this group of cancers are associated with poor outcomes. One such abnormality is the MECOM gene rearrangement that typically occurs in cases with chromosome 7 abnormalities. MECOM encodes a transcription factor that plays an essential role in cell proliferation and maintenance and also in epigenetic regulation...
February 2018: Cancer Genetics
https://www.readbyqxmd.com/read/29404451/microrna-therapy-inhibits-hepatoblastoma-growth-in-vivo-by-targeting-%C3%AE-catenin-and-wnt-signaling
#8
Emilie Indersie, Sarah Lesjean, Katarzyna B Hooks, Francis Sagliocco, Tony Ernault, Stefano Cairo, Maria Merched-Sauvage, Anne Rullier, Brigitte Le Bail, Sophie Taque, Michael Grotzer, Sophie Branchereau, Catherine Guettier, Monique Fabre, Laurence Brugières, Martin Hagedorn, Marie-Annick Buendia, Christophe F Grosset
Hepatoblastoma (HBL) is the most common pediatric liver cancer. In this malignant neoplasm, beta-catenin protein accumulates and increases Wnt signaling due to recurrent activating mutations in the catenin-beta 1 (CTNNB1) gene. Therefore, beta-catenin is a key therapeutic target in HBL. However, controlling beta-catenin production with therapeutic molecules has been challenging. New biological studies could provide alternative therapeutic solutions for the treatment of HBL, especially for advanced tumors and metastatic disease...
April 2017: Hepatology Communications
https://www.readbyqxmd.com/read/29404245/pediatric-pituitary-adenoma-case-series-review-of-the-literature-and-a-skull-base-treatment-paradigm
#9
REVIEW
Avital Perry, Christopher Salvatore Graffeo, Christopher Marcellino, Bruce E Pollock, Nicholas M Wetjen, Fredric B Meyer
Background  Pediatric pituitary adenoma is a rare skull base neoplasm, accounting for 3% of all intracranial neoplasms in children and 5% of pituitary adenomas. Compared with pituitary tumors in adults, secreting tumors predominate and longer disease trajectories are expected due to the patient age resulting in a natural history and treatment paradigm that is complex and controversial. Objectives  The aims of this study were to describe a large, single-institution series of pediatric pituitary adenomas with extensive long-term follow-up and to conduct a systematic review examining outcomes after pituitary adenoma surgery in the pediatric population...
February 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29397238/hereditary-swi-snf-complex-deficiency-syndromes
#10
REVIEW
Abbas Agaimy, William D Foulkes
The SWItch Sucrose non-fermentable (SWI/SNF) complex is a highly conserved multi-subunit complex of proteins encoded by numerous genes mapped to different chromosomal regions. The complex regulates the process of chromatin remodelling and hence plays a central role in the epigenetic regulation of gene expression, cell proliferation and differentiation. During the last three decades, the SWI/SNF complex has been increasingly recognized as a central molecular event driving the initiation and/or progression of several benign and malignant neoplasms of different anatomic origin and having diverse histomorphological appearance...
January 31, 2018: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/29396936/flow-cytometry-in-pediatric-malignancies
#11
Anil Handoo, Tina Dadu
The utility of flow cytometry as a useful diagnostic modality for the assessment of hematopoietic neoplasms has been established beyond doubt. In fact, it is now an integral part of the diagnosis and classification of various diseases like leukemias and lymphomas along with molecular studies and cytogenetics. Prognostication and disease monitoring by flow cytometry is also being recognized increasingly as one of the important fortes. This is evident by the number of articles in the published in literature on the minimal residual disease detection by flow cytometry especially in the last decade or so...
January 15, 2018: Indian Pediatrics
https://www.readbyqxmd.com/read/29393179/irradiation-of-intracranial-gliomas-in-children
#12
Rolf-Dieter Kortmann, Clemens Seidel, Klaus Müller, Franz Wolfgang Hirsch
Attainment of local control is a cornerstone in the management of brain tumors in children. Treatment of progressive low-grade and all high-grade gliomas traditionally includes resection followed by fractionated radiotherapy (FRT) or FRT alone in neoplasms not amenable to surgical removal. While in younger children chemotherapy is typically used as a first-line non-surgical management option, FRT in such patients remains the standard of salvage therapy. Recent improvements in techniques for radiation treatment planning and delivery allow conformal and selective coverage of the target volume with a prescribed dose, sparing adjacent normal tissues to assure maximal tumor control and minimal toxicity...
2018: Progress in Neurological Surgery
https://www.readbyqxmd.com/read/29391718/early-diagnosis-of-an-isolated-primary-peripheral-t-cell-lymphoma-masquerading-as-massive-gingival-enlargement-in-a-pediatric-patient
#13
Sravani Ghattamaneni, Venkateswara Rao Guttikonda, Sivaranjani Yeluri, Rajani Kolipara
Lymphomas are malignant neoplasm of the lymphocyte cell lines, classified as either Hodgkin's or non-Hodgkin's lymphoma (NHL). NHL comprises a heterogeneous group of lymphoid neoplasm arising from B-cell, T-cell or natural killer cell with a spectrum of behavior ranging from relatively indolent to highly aggressive and potentially fatal. Peripheral T-cell lymphoma, a variant of NHL, is a disease characterized by the presence of diffuse lymphadenopathy, extranodal involvement, classical B symptoms such as fever (>100...
September 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
https://www.readbyqxmd.com/read/29380524/noninvasive-encapsulated-follicular-variant-of-papillary-thyroid-carcinoma-should-it-also-be-reclassified-in-children
#14
Rachel A Mariani, Rachel Kadakia, Nicoleta C Arva
BACKGROUND: Noninvasive encapsulated follicular variant of papillary thyroid carcinoma (noniEFVPTC) has low risk of adverse outcome in adults, warranting reclassification as noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP). In children, thyroid nodules have higher risk of malignancy and it is unknown if encapsulated FVPTC (EFVPTC) and infiltrative FVPTC (IFVPTC) tumors have different behavior. We analyzed the clinicopathologic features of follicular variant of papillary thyroid carcinoma (FVPTC) subtypes in our pediatric population to determine if noniEFVPTC has an indolent course as reported in adults...
January 30, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29378601/tissue-expression-of-retinoic-acid-receptor-alpha-and-crabp2-in-metastatic-nephroblastomas
#15
Ana Paula Percicote, Gabriel Lazaretti Mardegan, Elizabeth Schneider Gugelmim, Sergio Ossamu Ioshii, Ana Paula Kuczynski, Seigo Nagashima, Lúcia de Noronha
BACKGROUND: Nephroblastoma or Wilms tumor is the most frequent kidney cancer in children and accounts for 98% of kidney tumors in this age group. Despite favorable prognosis, a subgroup of these patients progresses to recurrence and death. The retinoic acid (RA) pathway plays a role in the chemoprevention and treatment of tumors due to its effects on cell differentiation and its antiproliferative, anti-oxidant, and pro-apoptotic activities. Reports describe abnormal cellular retinoic acid-binding protein 2 (CRABP2) expression in neoplasms and its correlation with prognostic factors and clinical and pathological characteristics...
January 22, 2018: Diagnostic Pathology
https://www.readbyqxmd.com/read/29361006/pediatric-chordomas-results-of-a-multicentric-study-of-40-children-and-proposal-for-a-histopathological-prognostic-grading-system-and-new-therapeutic-strategies
#16
Kévin Beccaria, Arnault Tauziède-Espariat, Franck Monnien, Homa Adle-Biassette, Julien Masliah-Planchon, Gaëlle Pierron, Laetitia Maillot, Marc Polivka, Annie Laquerrière, Sandrine Bouillot-Eimer, Edouard Gimbert, Guillaume Gauchotte, Laurent Coffinet, Henri Sevestre, Claire Alapetite, Stéphanie Bolle, Dominic Thompson, Michel Zérah, Christian Sainte-Rose, Stéphanie Puget, Pascale Varlet
Pediatric chordomas are rare malignant neoplasms, and few data are available for optimizing therapeutic strategies and outcome. This study aimed at evaluating how best to manage them and to identify prognostic factors. This multicentric retrospective study included 40 children diagnosed with chordomas between 1966 and 2012. Clinical, radiological, and histopathological data, treatment modalities, and outcomes were reviewed. The median age was 12 years old. Most chordomas were histologically classical forms (45...
January 18, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29356889/national-changes-in-pediatric-tracheotomy-epidemiology-during-36-years
#17
Mette Sørensen Resen, Christian Grønhøj, Thomas Hjuler
BACKGROUND: Information on the incidence, indications and morbidity of pediatric tracheotomy from a nationwide setting is sparse. METHODS: From the nationwide Danish National Patient Registry, we identified all cases: 0-15-year-old children registered with a first-time tracheotomy from 1979 to 2014. We extracted the date of surgery, admission, discharge, age, gender, hospital, department, hospitalization length, hospital contacts, and diagnosis-code related to the surgery...
January 22, 2018: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/29356335/pediatric-patient-with-end-stage-kidney-disease-secondary-to-eagle-barrett-syndrome-and-metastatic-unresectable-hepatoblastoma-treated-successfully-with-chemotherapy-and-liver-kidney-transplant
#18
Daniel Ortiz, Avis Harden, Fernando F Corrales-Medina, Gaurav Saigal, Akin Tekin, Jennifer Garcia
HBL is the most common malignant liver neoplasm in children. The etiology of HBL is largely unknown but there are certain syndromes, such as Beckwith-Wiedemann syndrome, that have been clearly associated with an increased incidence of this malignancy. EBS, also known as prune belly syndrome, is a congenital anomaly characterized by lax abdominal musculature, bilateral cryptorchidism requiring, in some cases, hemodialysis due to significant kidney and urinary tract dysfunctions. Despite an improvement on the survival rates of patients with advanced-stage HBL, the presence of concomitant end-stage renal disease that occurs in patients with EBS constitutes a therapeutic challenge for the clinician not only due to the use of nephrotoxic chemotherapy but also due to the potential need for multi-organ transplant...
January 22, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29346230/chronic-hematoma-of-the-neck
#19
Kristen H Angster, Victor Da Costa
Chronic hematomas are defined as hematomas with slow, progressive growth over many weeks. They are an extremely rare cause of acute neck swelling in the pediatric population. They consist of an organized central mass of blood with granulation tissue and fibrotic changes peripherally. The presence of a capsule prevents resorption but allows for intracapsular bleeding with subsequent expansion. We describe a case of a 6-year-old girl who presented to the emergency department with a spontaneously occurring left neck mass in the supraclavicular region...
February 2018: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29346182/atypical-teratoid-rhabdoid-tumor-of-the-cauda-equina-in-a-child-report-of-a-very-unusual-case
#20
James M Shiflett, Betty L Herrington, David A Joyner, Ali G Saad
Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive malignant primitive neoplasms that commonly occur in children younger than 2 years of age. The prognosis is generally dismal with a median survival time of <1 year. The majority of AT/RT occur in the posterior fossa and less frequently the supratentorium. Primary pediatric spinal AT/RT are exceedingly rare and only 15 cases have been reported to date. Here we report a very unusual case of primary spinal AT/RT extensively involving the spinal cord from T11 down to the cauda equina...
January 16, 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
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