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https://www.readbyqxmd.com/read/29036598/global-dna-methylation-analysis-reveals-mir-214-3p-contributes-to-cisplatin-resistance-in-pediatric-intracranial-nongerminomatous-malignant-germ-cell-tumors
#1
Tsung-Han Hsieh, Yun-Ru Liu, Ting-Yu Chang, Muh-Lii Liang, Hsin-Hung Chen, Hsei-Wei Wang, Yun Yen, Tai-Tong Wong
Background: Pediatric central nervous system germ cell tumors (CNSGCTs) are rare and heterogeneous neoplasms, and they can be divided into germinomas and nongerminomatous GCTs (NGGCTs). NGGCTs are further subdivided into mature teratomas and nongerminomatous malignant GCTs (NGMGCTs). Clinical outcomes suggest that NGMGCTs have poor prognosis and survival, and that they require more extensive radiotherapy and adjuvant chemotherapy. However, the mechanisms underlying this difference are still unclear...
October 3, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/29026272/incidence-of-diabetes-mellitus-and-neoplasia-in-japanese-short-statured-children-treated-with-growth-hormone-in-the-genetics-and-neuroendocrinology-of-short-stature-international-study-genesis
#2
Susumu Yokoya, Tomonobu Hasegawa, Keiichi Ozono, Hiroyuki Tanaka, Susumu Kanzaki, Toshiaki Tanaka, Kazuo Chihara, Nan Jia, Christopher J Child, Katsuichiro Ihara, Jumpei Funai, Noriyuki Iwamoto, Yoshiki Seino
The primary goal of the Genetics and Neuroendocrinology of Short Stature International Study (GeNeSIS) was to assess the safety and effectiveness of Humatrope(®), a GH preparation, in the treatment of pediatric patients with short stature. We report our findings in the GH-treated Japanese pediatric population focusing on the incidence of type 2 diabetes (T2D) and occurrence of neoplasms. A total of 2,345 Japanese patients were assessed for safety. During a mean observation period of 3.2 yr, T2D occurred in 3 patients (0...
2017: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://www.readbyqxmd.com/read/29025601/a-neoplasm-with-fip1l1-pdgfra-fusion-presenting-as-pediatric-t-cell-lymphoblastic-leukemia-lymphoma-without-eosinophilia
#3
Matthew J Oberley, Christopher Denton, Jianling Ji, Matthew Hiemenz, Deepa Bhojwani, Dejerianne Ostrow, Samuel Wu, Paul Gaynon, Gordana Raca
The 2016 World Health Organization (2016 WHO) classification of hematopoietic malignancies classifies neoplasms with a fusion between the FIP1L1 and PDGFRA genes in 4q12 into a group called "myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1 or with PCM1-JAK2". Neoplasms characterized by this fusion are pluripotent stem cell disorders that can show both myeloid and lymphoid differentiation. They typically occur in adult patients and most are characterized by eosinophilia...
October 2017: Cancer Genetics
https://www.readbyqxmd.com/read/29024469/noninvasive-follicular-thyroid-neoplasm-with-papillary-like-nuclear-features-in-the-pediatric-age-group
#4
Esther Diana Rossi, Swati Mehrotra, Ayse Irem Kilic, Iclal Erdem Toslak, Jennifer Lim-Dunham, Maurizio Martini, Guido Fadda, Celestino Pio Lombardi, Luigi Maria Larocca, Güliz A Barkan
BACKGROUND: The most common malignant thyroid neoplasm in children is papillary thyroid carcinoma (PTC). In 2015, the Endocrine Pathology Society introduced the terminology "noninvasive follicular thyroid neoplasm with papillary-like nuclear features" (NIFTP) to replace the noninvasive follicular variant of PTC. The objective of the current study was to evaluate previously diagnosed PTC in the pediatric population, reappraise it for NIFTP, and discuss the impact of NIFTP on the risk of malignancy (ROM) for each The Bethesda System for Reporting Thyroid Cytopathology category in the pediatric population...
October 12, 2017: Cancer
https://www.readbyqxmd.com/read/29019749/pearls-and-pitfalls-in-diagnosing-pediatric-urinary-bladder-masses
#5
Susan C Shelmerdine, Armando J Lorenzo, Abha A Gupta, Govind B Chavhan
Urinary bladder masses are rare in children, and the associated histologic features and prognoses in this population are different from those in adults. Most children with urinary bladder masses present with lower urinary tract symptoms, which may include hematuria, dysuria, frequent urination, and urgency to urinate. However, some of these masses may be identified incidentally or involve generic symptoms such as abdominal distention. In general, pediatric bladder tumors can be divided into those that originate from the bladder epithelium, known as urothelial neoplasms, and mesenchymal bladder neoplasms, which are more prevalent...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29019747/masses-of-the-nose-nasal-cavity-and-nasopharynx-in-children
#6
Diana P Rodriguez, Emily S Orscheln, Bernadette L Koch
A wide range of masses develop in the nose, nasal cavity, and nasopharynx in children. These lesions may arise from the nasal ala or other structures of the nose, including the mucosa covering any surface of the nasal cavity, the cartilaginous or osseous portion of the nasal septum, the nasal turbinates, and the nasal bones. Lesions may also arise from the nasopharynx or adjacent structures and involve the nose by way of direct extension. The causes of nasal masses in children include congenital and developmental disorders such as congenital nasolacrimal duct mucocele, dermoid cyst, cephalocele, and nasal neuroglial heterotopia; inflammatory and infectious processes such as mucocele, polyp, and pyogenic granuloma; benign neoplasms such as infantile hemangioma and juvenile nasopharyngeal angiofibroma; malignant lesions such as rhabdomyosarcoma and nasopharyngeal carcinoma; and masses related to prior trauma such as septal hematoma...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29019745/imaging-of-nonmalignant-adrenal-lesions-in-children
#7
Kiran M Sargar, Geetika Khanna, Rebecca Hulett Bowling
The adrenal glands in children can be affected by a variety of benign lesions. The diagnosis of adrenal lesions can be challenging, but assessment of morphologic changes in correlation with the clinical presentation can lead to an accurate diagnosis. These lesions can be classified by their cause: congenital (eg, discoid adrenal gland, horseshoe adrenal gland, and epithelial cysts), vascular and/or traumatic (eg, adrenal hemorrhage), infectious (eg, granulomatous diseases), enzyme deficiency disorders (eg, congenital adrenal hyperplasia [CAH] and Wolman disease), benign neoplasms (eg, pheochromocytomas, ganglioneuromas, adrenal adenomas, and myelolipomas), and adrenal mass mimics (eg, extralobar sequestration and extramedullary hematopoiesis)...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28994342/malignant-ectomesenchymoma-series-analysis-of-a-histologically-and-genetically-heterogeneous-tumor
#8
Brannan B Griffin, Pauline M Chou, David George, Lawrence J Jennings, Nicoleta C Arva
AIMS: Malignant ectomesenchymoma is a rare pediatric neoplasm with dual mesenchymal and neuroectodermal elements. Mesenchymal component is usually rhabdomyosarcoma, particularly embryonal subtype, whereas neuroectodermal derivatives are frequently a neuroblastic tumor. Ectomesenchymoma manifests in various sites given the wide migration of neural crest cells during development, though the pelvis/perineum is most often involved. Moreover, no unique unifying molecular abnormality has been determined...
October 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28990704/epithelioid-glioblastomas-stratify-into-established-diagnostic-subsets-upon-integrated-molecular-analysis
#9
Andrey Korshunov, Lukas Chavez, Tanvi Sharma, Marina Ryzhova, Daniel Schrimpf, Damian Stichel, David Capper, Dominik Sturm, Marcel Kool, Antje Habel, Bette K Kleinschmidt-DeMasters, Marc Rosenblum, Oksana Absalyamova, Andrey Golanov, Peter Lichter, Stefan M Pfister, David T W Jones, Arie Perry, Andreas von Deimling
Epithelioid glioblastoma (eGBM) is a newly defined and rare GBM variant in the current WHO 2016 classification. BRAF V600E mutation is overrepresented in these tumors and there is known some morphological overlap with anaplastic epithelioid PXA (ePXA). In order to further elucidate this diagnostic category, we molecularly characterized 64 pediatric and adult examples initially diagnosed as "eGBM". Tumors were analyzed using array based methylation and direct sequencing of the BRAF and TERT genes. Our results demonstrated considerable molecular and clinical heterogeneity among eGBM cohort...
October 9, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28989289/epidemiology-diagnosis-and-optimal-management-of-glioma-in-adolescents-and-young-adults
#10
REVIEW
Tejan P Diwanji, Alexander Engelman, James W Snider, Pranshu Mohindra
Neoplasms of the central nervous system (CNS) are the most frequently encountered solid tumors of childhood, but are less common in adolescents and young adults (AYA), aged 15-39 years. Gliomas account for 29%-35% of the CNS tumors in AYA, with approximately two-thirds being low-grade glioma (LGG) and the remaining being high-grade glioma (HGG). We review the epidemiology, work-up, and management of LGG and HGG, focusing on the particular issues faced by the AYA population relative to pediatric and adult populations...
2017: Adolescent Health, Medicine and Therapeutics
https://www.readbyqxmd.com/read/28987830/intracranial-meningioma-in-patients-younger-than-35-years-of-age-an-evolution-of-the-disease-in-the-era-of-human-immunodeficiency-virus-infection-a-retrospective-observational-study
#11
Mogwale Samson Motebejane, Ian Kaminsky, In Sup Choi
BACKGROUND: Intracranial meningiomas are more common among patients in their 5(th)-7(th) decades of life and rare in children and early adulthood. They constitute between 1.5% of all neoplasms in patients younger than 20 years of age but account for 13.5% of all neoplasms in patients aged between 20-34 years. They are often associated with hereditary or familial syndromes in pediatrics and young adults and tend to be of high grade. OBJECTIVES: We describe the histopathological subtypes of intracranial meningioma between HIV seropositive and the general patients population with intracranial meningiomas, younger than 35 years of age...
October 4, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28980149/hemorrhagic-presentation-of-intracranial-pilocytic-astrocytomas-literature-review
#12
REVIEW
G Lakshmi Prasad, B N Nandeesh, Girish R Menon
Pilocytic astrocytomas (PAs) are seemingly innocuous and benign tumors. However, in recent times, many case series have documented high rates of hemorrhage in these neoplasms. We hereby provide a detailed analysis on hemorrhagic pilocytic astrocytomas (HPA) in adults and report one such case managed at our institute. In addition, salient differences between adult and pediatric hemorrhagic PA have been briefed. Hospital records were retrieved for our case. Literature review was conducted by searching online databases for the following keywords-pilocytic astrocytoma, hemorrhage, cranial, pediatric, and adults...
October 4, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28978864/2016-revision-of-the-who-classification-of-lymphoid-neoplasms
#13
Jun-Ichi Tamaru
The 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms have been published in "Blood" as a review form. A major revision is being published that will be an update of the current fourth edition and not a truly new fifth edition. Because it is considered a part of the fourth edition, while some provisional entities will be promoted to definite entities and a small number of new provisional entities added, there will be no new definite entities. As with the 2001 and 2008 classifications, an all-important Clinical Advisory Committee meeting was held to obtain the advice and consent of clinical hematologists/oncologists and other physicians critical to the revision...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28976901/a-technical-problem-in-diagnosis-clarity-of-solitary-spinal-cord-neurofibroma-in-an-eleven-month-old-boy
#14
Elena G Poriazova, Oliana B Boykinova, Ivan S Ivanov, Maria I Spasova, Georgi B Bozhilov, Ani K Kevorkyan, Stoyan S Novakov
Spinal neurofibroma is one of the rarest of the neoplasms involving the spinal cord or roots and occurs much less often than neurinoma, meningioma or glioma. The sixth pediatric case of solitary intramedullary tumor was described in 2013, according to B. Eljebbouri et al. We present a rare, difficult to diagnose and may-be the seventh pediatric case of solitary neurofibroma of the cauda equine in an 11-month-old infant. The patient underwent a laminectomy of T12, L1, L2 and L3, extirpation of intradural, intramedullary and extramedullary spinal cord tumor...
September 1, 2017: Folia Medica
https://www.readbyqxmd.com/read/28975680/yolk-sac-erythromyeloid-progenitors-expressing-gain-of-function-ptpn11-have-functional-features-of-jmml-but-are-not-sufficient-to-cause-disease-in-mice
#15
Stefan P Tarnawsky, Momoko Yoshimoto, Lisa Deng, Rebecca J Chan, Mervin C Yoder
BACKGROUND: Accumulating evidence suggests the origin of Juvenile Myelomonocytic Leukemia (JMML) is closely associated with fetal development. Nevertheless, the contribution of embryonic progenitors to JMML pathogenesis remains unexplored. We hypothesized that expression of JMML-initiating PTPN11 mutations in HSC-independent yolk sac erythromyeloid progenitors (YS EMPs) would result in a mouse model of pediatric myeloproliferative neoplasm (MPN). RESULTS: E9.5 YS EMPs from VavCre+;PTPN11(D61Y) embryos demonstrated growth hypersensitivity to GM-CSF and hyperactive RAS-ERK signaling...
October 4, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/28968159/pediatric-thalamic-gliomas-an-updated-review
#16
Avneesh Gupta, Nathan Shaller, Kathryn A McFadden
CONTEXT: - Neoplasms originating in the thalamus are rare overall (1% of all brain tumors); however, they comprise approximately 5% of pediatric intracranial tumors and approach 15% of all malignant pediatric intracranial tumors in some series. OBJECTIVE: - To update readers about the current understanding of the diverse histology, biology, and behavior of pediatric thalamic tumors. Histologic verification is now thought to be critical for planning treatment, and, as a result, biopsy and total/subtotal resections are much more common today than in the past...
October 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28965982/vaginoscopic-resection-for-vaginal-rhabdomyosarcoma-during-early-infancy-a-case-report
#17
Karolina Pańczak, Dawid Gawron, Patrycja Sosnowska, Karina Kapczuk, Przemysław Mańkowski
Embryonal rhabdomyosarcoma, a malignant soft-tissue neoplasm, is one of the most common pediatric tumors of the vagina. Although radical surgery has been replaced with radiation therapy, chemotherapy, and conservative surgery, the use of vaginoscopy is still uncommon. We present the case of an infant who underwent vaginoscopic resection owing to botryoid sarcoma at 9 months of age.
September 28, 2017: Journal of Minimally Invasive Gynecology
https://www.readbyqxmd.com/read/28960912/dicer1-syndrome-approach-to-testing-and-management-at-a-large-pediatric-tertiary-care-center
#18
Kalene van Engelen, Anita Villani, Jonathan D Wasserman, Laura Aronoff, Mary-Louise C Greer, Marta Tijerin Bueno, Bailey Gallinger, Raymond H Kim, Ronald Grant, M Stephen Meyn, David Malkin, Harriet Druker
BACKGROUND: To expand the current knowledge of DICER1 syndrome and to propose criteria for genetic testing based on experience at a pediatric tertiary care center. PROCEDURE: This study involved a retrospective chart review of the 78 patients (47 probands and 31 family members) seen in the Cancer Genetics Program at The Hospital for Sick Children (SickKids) who were offered genetic testing for DICER1. RESULTS: Of 47 probands offered genetic testing for DICER1, 46 pursued testing: 11 (23...
September 27, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28942697/ovarian-masses-in-pediatric-patients-a-multicenter-study-of-98-surgical-cases-in-tunisia
#19
I Abid, M Zouari, M Jallouli, S Sahli, A Bouden, R Ben Abdallah, F Trabelsi, A Jabloun, A Charieg, C Mrad, M Marzouki, S Mosbahi, A Ezzi, R Mootamri, M Hamzaoui, N Kaabar, S Jlidi, A Nouri, R Mhiri
Ovarian masses requiring surgical intervention are uncommon in the pediatric population. Our aim is to report results of a multicentric Tunisian study concerning the clinical practice and the management of pediatric ovarian masses and to identify the factors that are associated with ovarian preservation. Between January 2000 and December 2015, 98 pediatric patients (<14 years) were surgically treated for ovarian masses at the five pediatric surgery departments in Tunisia. Ninety-eight patients were included in this study...
September 23, 2017: Gynecological Endocrinology
https://www.readbyqxmd.com/read/28931199/maxillary-sinus-mucoceles-and-other-side-effects-of-external-beam-radiation-in-the-pediatric-patient-a-cautionary-tale
#20
Anthony Sheyn, Tate Naylor, Felicity Lenes-Voit, Eric Berg
A sinonasal mucocele can develop after the obstruction of a sinus ostium, which can occur secondary to trauma, infection, a neoplasm, or an iatrogenic cause. These mucoceles typically arise in the frontal and ethmoid sinuses. Sinonasal mucoceles are epithelium-lined cysts that can gradually expand to fill a paranasal sinus. They can present with ophthalmologic, rhinologic, and neurologic symptoms. There are reports in the literature of sinonasal mucocele development after radiation for nasopharyngeal carcinoma...
September 2017: Ear, Nose, & Throat Journal
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