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https://www.readbyqxmd.com/read/28643220/denosumab-an-emerging-therapy-in-pediatric-bone-disorders
#1
REVIEW
Alison M Boyce
PURPOSE OF REVIEW: Denosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand (RANKL), and has emerged as an important novel therapy for skeletal disorders. This article examines the use of denosumab in children. RECENT FINDINGS: Considerable safety and efficacy data exists for denosumab treatment of adults with osteoporosis, bone metastases, and giant cell tumors. Pediatric data is limited; however, evidence suggests denosumab may be beneficial in decreasing bone turnover, increasing bone density, and preventing growth of certain skeletal neoplasms in children...
June 22, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28642445/identification-of-a-cytogenetic-and-molecular-subgroup-of-acute-myeloid-leukemias-showing-sensitivity-to-l-asparaginase
#2
Salvatore Nicola Bertuccio, Salvatore Serravalle, Annalisa Astolfi, Annalisa Lonetti, Valentina Indio, Anna Leszl, Andrea Pession, Fraia Melchionda
L-Asparaginase (L-Asp) is an enzyme that catalyzes the hydrolysis of L-asparagine to L-aspartic acid, and its depletion induces leukemic cell death. L-Asp is an important component of treatment regimens for Acute Lymphoblastic Leukemia (ALL). Sensitivity to L-Asp is due to the absence of L-Asparagine synthetase (ASNS), the enzyme that catalyzes the biosynthesis of L-asparagine. ASNS gene is located on 7q21.3, and its increased expression in ALLs correlates with L-Asp resistance. Chromosome 7 monosomy (-7) is a recurrent aberration in myeloid disorders, particularly in adverse-risk Acute Myeloid Leukemias (AMLs) and therapy-related myeloid neoplasms (t-MN), that leads to a significant downregulation of the deleted genes, including ASNS...
June 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/28639735/neonates-with-cancer-and-causes-of-death-lessons-from-615-cases-in-the-seer-databases
#3
Ahmad S Alfaar, Waleed M Hassan, Mohamed Sabry Bakry, Ibrahim Qaddoumi
Neonatal tumors are rare with no standard treatment approaches to these diseases, and the patients experience poor outcomes. Our aim was to determine the distribution of cancers affecting neonates and compare survival between these cancers and older children. We analyzed SEER data (1973-2007) from patients who were younger than 2 years at diagnosis of malignancy. Special permission was granted to access the detailed (i.e., age in months) data of those patients. The Chi-square Log-rank test was used to compare survival between neonates (aged <1 month) and older children (>1 month to <2 years)...
June 22, 2017: Cancer Medicine
https://www.readbyqxmd.com/read/28638563/extraskeletal-myxoid-chondrosarcoma-of-the-masticator-space-in-a-pediatric-patient
#4
Mário-José Romañach, Román Carlos, Michel Nuyens, Bruno-Augusto-Benevenuto de Andrade, Oslei-Paes de Almeida
Extraskeletal myxoid chondrosarcoma (EMC) is a malignant soft-tissue neoplasm rarely described in the head and neck region of children and adolescents. We describe a case of EMC affecting the masticator space and a literature review. A 13-year-old boy who presented a large painless, diffuse mass causing progressive midfacial asymmetry of 6 months duration. Histopathological evaluation revealed a multinodular lesion, containing scattered round vacuolated tumor cells dispersed in an abundant myxoid stroma, separated by fibrous septae...
June 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28633738/sebaceous-carcinoma-on-the-arm-of-a-10-year-old-girl
#5
Stephen K Stacey, Tyler A Moss, Todd T Kobayashi
We report a case of a 10 year-old girl diagnosed with sebaceous carcinoma of the posterior left arm. The presented case reviews the histopathological and immunohistochemical characteristics of this malignancy, including a review of the literature in pediatric patients regarding prognosis and treatment. Sebaceous carcinoma is a malignant neoplasm with sebaceous differentiation, typically occurring in the sixth-to-seventh decades of life. It most commonly arises in the periocular region. It is extremely rare in the pediatric population...
June 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28626255/underlying-cause-of-death-recorded-during-2013-to-2015-at-a-tertiary-general-hospital-in-vientiane-capital-lao-pdr
#6
Bounfeng Phoummalaysith, Viengsakhone Louangpradith, Tavanh Manivon, Bounxou Keohavong, Eiko Yamamoto, Nobuyuki Hamajima
In Lao People's Democratic Republic (Lao PDR), the cause of death is not registered in death reports. As a result, the government cannot produce official reports that show mortality according to cause of death. This study aimed to report the underlying cause of death in a tertiary general hospital (Mittaphab Hospital) in Vientiane capital. Mittaphab hospital is a governmental teaching hospital with 300 beds for inpatient services specialized in orthopedics, neurology, and hemodialysis. Since a children hospital exists beside Mittaphab Hospital, severe pediatric cases are referred to the child hospital...
February 2017: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/28620008/pten-dicer1-fh-and-their-associated-tumor-susceptibility-syndromes-clinical-features-genetics-and-surveillance-recommendations-in-childhood
#7
REVIEW
Kris Ann P Schultz, Surya P Rednam, Junne Kamihara, Leslie Doros, Maria Isabel Achatz, Jonathan D Wasserman, Lisa R Diller, Laurence Brugières, Harriet Druker, Katherine A Schneider, Rose B McGee, William D Foulkes
PTEN hamartoma tumor syndrome (PHTS), DICER1 syndrome, and hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome are pleiotropic tumor predisposition syndromes that include benign and malignant neoplasms affecting adults and children. PHTS includes several disorders with shared and distinct clinical features. These are associated with elevated lifetime risk of breast, thyroid, endometrial, colorectal, and renal cancers as well as melanoma. Thyroid cancer represents the predominant cancer risk under age 20 years...
June 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28601179/pediatric-urinary-system-neoplasms-an-overview-and-update
#8
REVIEW
Michael George, Jeannette M Perez-Rosello, Ali Yikilmaz, Edward Y Lee
Pediatric urinary system neoplasms are a diverse group of tumors that frequently overlap in their clinical and radiologic features. By contrast, the histopathologic classification and treatment of these entities have become increasingly refined, resulting in improved outcomes, with the overall survival of Wilms tumors now exceeding 90%. Significantly, many contemporary protocols rely on radiologic diagnosis in the absence of tissue confirmation. This review article provides up-to-date clinical, epidemiologic, and imaging findings of pediatric urinary system neoplasms and their mimics frequently encountered in daily clinical practice...
July 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28601178/pediatric-hepatobiliary-neoplasms-an-overview-and-update
#9
REVIEW
Ali Yikilmaz, Michael George, Edward Y Lee
Recent developments regarding the treatment of pediatric liver tumors have significantly improved patient care. Stimulated by collaboration between international pediatric groups, advances have been made in surgical techniques, transplantation options, and chemotherapy schemas. In light of this progress, clear understanding of the state-of-the-art imaging evaluation of hepatobiliary tumors has become even more integral to the effective management of children with hepatic neoplasms. The unique imaging features of hepatic neoplasms in the pediatric population, when coupled with supportive demographic data and laboratory findings, can lead to accurate diagnosis and proper treatment of hepatobiliary tumors...
July 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28601174/thoracic-neoplasms-in-children-contemporary-perspectives-and-imaging-assessment
#10
REVIEW
Matthew A Zapala, Victor M Ho-Fung, Edward Y Lee
This article focuses on commonly encountered primary lung, airway, mediastinal, and chest wall neoplasms that occur in the pediatric population. Although primary pediatric thoracic neoplasms are rare, imaging is critical in their diagnostic work-up. An overview of the latest imaging techniques specific to evaluate these pediatric thoracic neoplasms is presented across the spectrum of modalities from radiography to PET/MR imaging. In addition, the characteristic imaging appearances of these pediatric primary thoracic neoplasms are discussed with an emphasis on what the radiologist needs to know in routine clinical practice...
July 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28599697/paraneoplastic-cushing-syndrome-due-to-wilm-s-tumor
#11
Mahwish Faizan, Jaida Manzoor, Muhammad Saleem, Saadia Anwar, Qaiser Mehmood, Ambreen Hameed, Agha Shabbir Ali
Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28597093/-pediatric-salivary-gland-tumors-and-tumor-like-lesions
#12
REVIEW
A Agaimy, H Iro, J Zenk
Salivary gland tumors and tumor-like lesions in the pediatric population are uncommon. They comprise a heterogeneous group of infectious/inflammatory and neoplastic conditions. Pediatric salivary neoplasms include benign tumors of mesenchymal or epithelial origin as well as malignancies of epithelial (carcinomas), mesenchymal (sarcoma) or hematolymphoid (lymphoma) derivation. Infectious/inflammatory conditions and hematolymphoid malignancies may represent either genuine parenchymal pathology or conditions involving intraglandular lymph nodes of the parotid glands (intraglandular lymphadenopathy and intraglandular nodal lymphomas)...
June 9, 2017: Der Pathologe
https://www.readbyqxmd.com/read/28589056/vaginal-ewing-sarcoma-an-uncommon-clinical-entity-in-pediatric-patients
#13
Nathan M Cross, A Luana Stanescu, Erin R Rudzinski, Douglas S Hawkins, Marguerite T Parisi
Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/28575523/safety-and-pharmacokinetic-study-of-fidaxomicin-in-children-with-clostridium-difficile-associated-diarrhea-a-phase-2a-multicenter-clinical-trial
#14
Molly A O'Gorman, Marian G Michaels, Sheldon L Kaplan, Anthony Otley, Larry K Kociolek, Edward J Hoffenberg, Kwang Sik Kim, Sharon Nachman, Marian D Pfefferkorn, Timothy Sentongo, Janice E Sullivan, Pamela Sears
Background.: Fidaxomicin is an approved therapy for Clostridium difficile-associated diarrhea (CDAD) in adults. The safety of fidaxomicin in children has not been reported. Methods.: In this study (ClinicalTrials.gov identifier NCT01591863), pediatric patients with CDAD received twice-daily oral fidaxomicin at a dose of 16 mg/kg per day (up to 200 mg) for 10 days in an open-label study. Plasma and fecal samples were collected for pharmacokinetic assessments. The primary outcome measure was safety, which was assessed by adverse-event (AE), laboratory, and physical examination/vital-sign monitoring...
May 31, 2017: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/28562437/use-of-skin-biopsies-among-dermatologists
#15
David A Weinstein, Sasank Konda, Brett M Coldiron
BACKGROUND: Skin biopsies are essential to establish a diagnosis in many skin diseases. Utilization has been increasing rapidly and represents a significant health care cost. There are no benchmarks or baselines to guide the practice of skin biopsies. OBJECTIVE: To create a reference data set of biopsy behavior among dermatologists. METHODS: Five hundred eighty-eight dermatologists belonging to the American Dermatological Association (ADA) were surveyed...
May 25, 2017: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/28561716/breast-cancer-after-childhood-adolescent-and-young-adult-cancer-it-s-not-just-about-chest-radiation
#16
David Hodgson, Flora van Leeuwen, Andrea Ng, Lindsay Morton, Tara O Henderson
Women who have been treated for a childhood, adolescent, or young adult cancer are at an increased risk for developing breast cancer at a young age, and breast cancer accounts for the most common subsequent malignant neoplasm among female childhood and adolescent cancer survivors. Risk of breast cancer in these survivors appears to be a multifaceted relationship between constitutional factors, exposures to radiation therapy (RT) and chemotherapy, and genetic predisposition. Given the significant morbidities and mortality associated with a breast cancer diagnosis, it is imperative that health care providers understand the risks, biology and genetics, recommended surveillance guidelines for early detection, and potential prevention strategies for women who have survived pediatric and young adult cancer...
2017: American Society of Clinical Oncology Educational Book
https://www.readbyqxmd.com/read/28559940/clinical-significance-of-pretreatment-fdg-pet-ct-in-mibg-avid-pediatric-neuroblastoma
#17
Seo Young Kang, Muhammad Kashif Rahim, Yong-Il Kim, Gi Jeong Cheon, Hyoung Jin Kang, Hee Young Shin, Keon Wook Kang, June-Key Chung, E Edmund Kim, Dong Soo Lee
PURPOSE: (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET) imaging is well known to have clinical significance in the initial staging and response evaluation of the many kinds of neoplasms. However, its role in the pediatric neuroblastoma is not clearly defined. In the present study, the clinical significance of FDG-PET/computed tomography (CT) in (123)I- or (131)I-metaiodobenzylguanidine (MIBG)-avid pediatric neuroblastoma was investigated. METHODS: Twenty patients with neuroblastoma who undertook pretreatment FDG PET/CT at our institute between 2008 and 2015 and showed MIBG avidity were retrospectively enrolled in the present study...
June 2017: Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28557716/unusual-fungal-lesion-presenting-as-a-neoplastic-pediatric-tongue-mass
#18
James Naples, Alison Martin, Debora Sobelman, Scott Schoem
Tongue lesions in the pediatric population are rare. The differential diagnosis of these lesions is broad, and rapid growth of the lesion is concerning for a neoplastic process. We present a rare case of a fungal lesion mimicking a neoplastic growth in a 22-month-old girl. She underwent complete excision successfully. Full evaluation for benign and malignant neoplasms was negative. Tissue culture demonstrated growth of a rare Candida species to be the cause of the lesion. Postoperatively, she continues to do well, without regrowth 6 months later...
May 2017: Pediatrics
https://www.readbyqxmd.com/read/28554756/metanephric-stromal-tumor-with-a-rare-incidence-of-squamous-epithelium-a-case-report-and-a-brief-review-of-the-literature
#19
Jia Wang, Wenjie Jin, Xiangru Wu
Metanephric stromal tumor (MST) of the kidney, a rare benign pediatric neoplasm recognized for less than 20 years, is not widely known. The authors describe a case of MST with rare squamous epithelium in a 14-month-old female. A renal mass was discovered during her fetal period. After her birth, computerized tomography revealed that the mass was localized in the inferior pole of her left kidney. She then underwent nephrectomy. The tumor was an unencapsulated but well-defined mass with a white, solid and firm cut surface and had dimensions of 4cm×3...
March 21, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28553386/solitary-plasmacytoma-of-bone-involving-spine-in-a-12-year-old-boy-report-of-a-rare-case-and-review-of-literature
#20
Rahul S Kulkarni, Sonia K Parikh, Asha S Anand, Harsha P Panchal, Apurva A Patel, Priti Trivedi, Kshitij Joshi, Pushpak Chirmade
Solitary plasmacytoma of the bone (SPB) is a rare plasma cell neoplasm representing only about 5% of plasma cell neoplasia. It usually presents as a lytic lesion mainly localized within the axial skeleton. SPB is exceedingly rare in young individuals, and only few cases have been reported so far in patients younger than 20 years of age. In view of rarity of disease, definitive treatment guidelines have not been established. We hereby report a case of SPB involving of lumbar vertebra (L5) in a 12-year-old boy...
January 2017: Journal of Pediatric Neurosciences
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