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Pediatric sarcoma

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https://www.readbyqxmd.com/read/29244667/periosteal-ewing-sarcoma-in-a-65-year-old-man-a-case-report
#1
Julia Bedard, Jessica Burns, Amalia de Comas
CASE: Ewing sarcoma is predominantly a pediatric bone malignancy involving the medullary canal. Periosteal Ewing sarcoma, a subtype arising from the periosteum, is exceedingly rare, with approximately 30 reported cases in the literature. We describe a case of periosteal Ewing sarcoma in the tibia of a 65-year-old man. Magnetic resonance imaging revealed a heterogeneous mass in the proximal tibial diaphysis without medullary involvement. The diagnosis was confirmed histologically after biopsy...
April 2017: JBJS Case Connector
https://www.readbyqxmd.com/read/29238940/seasonality-in-pediatric-cancer
#2
Rubayed Nurullah, Stefan Kuhle, Bryan Maguire, Ketan Kulkarni
Although seasonal trends in incidence and diagnosis of pediatric cancers have been widely investigated, the results have been inconclusive. A consistent seasonal trend may possibly provide etiological insights into pediatric cancers. This study aims to determine if there is a seasonal variation in cancer diagnoses in the pediatric population at the IWK Health Centre, a tertiary care center serving three Canadian provinces: Nova Scotia, New Brunswick, and Prince Edward Island. All pediatric cancer patients aged 0-20 y diagnosed from 1995 to 2015 at the center were included in this study...
December 14, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/29228868/solitary-fibrous-tumors-in-pediatric-patients-a-rare-and-potentially-overdiagnosed-neoplasm-confirmed-by-stat6-immunohistochemistry
#3
Serena Y Tan, Linda J Szymanski, Carlos Galliani, David Parham, Eduardo Zambrano
Pathological diagnosis of solitary fibrous tumor (SFT) in the pediatric population is challenging, as it occurs uncommonly in this age-group and resembles other spindle cell neoplasms. SFT contains a NAB2-STAT6 fusion gene, which can be reliably detected using STAT6 immunohistochemistry. Positive staining is highly sensitive and specific. We sought to investigate the utility of STAT6 immunohistochemistry, to show how commonly SFT was historically recognized at 3 academic pediatric institutions, to reclassify them when appropriate, and to demonstrate features of major mimics of SFT...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29226200/long-term-results-of-osteoarticular-allograft-reconstruction-in-children-with-distal-femoral-bone-tumors
#4
Khodamorad Jamshidi, Mehrdad Bahrabadi, Alireza Mirzaei
Background: There is no consensus regarding the best method of reconstruction in pediatric population following the wide resection of malignant bone tumors. More exploration of the complications of osteoarticular reconstruction leads to less existing controversy of this type of reconstruction, which is the main point of this article. Methods: Long-term outcomes and complications of osteoarticular allograft reconstruction of primary distal femoral bone sarcomas in 22 children with mean age of 10...
September 2017: Archives of Bone and Joint Surgery
https://www.readbyqxmd.com/read/29226049/unresectable-undifferentiated-embryonal-sarcoma-of-the-liver-in-an-adult-male-treated-with-chemotherapy-and-orthotopic-liver-transplantation
#5
Zarak H Khan, Kamran Ilyas, Hamza H Khan, Haider Ghazanfar, Qulsoom Hussain, Faisal Inayat, Muhammad Yasir, Rizwan Asim
Undifferentiated embryonal sarcoma of the liver (UESL) is a malignancy of mesenchymal origin observed predominantly in the pediatric population and very rarely in adults. We describe the case of a 21-year-old male who presented with acute onset of right upper quadrant pain and distention. Physical examination of the patient revealed right upper quadrant tenderness with the lower border of the liver palpable, 4 cm below the right costal margin. Laboratory tests performed on admission showed that the patient's liver function tests, urinalysis, complete blood count, and basic metabolic panel were within reference range...
October 8, 2017: Curēus
https://www.readbyqxmd.com/read/29221844/gabapentin-as-an-adjuvant-therapy-for-prevention-of-acute-phantom-limb-pain-in-pediatric-patients-undergoing-amputation-for-malignant-bone-tumors-a-prospective-double-blind-randomized-controlled-trial
#6
Xiandi Wang, Ying Yi, Dingbo Tang, Yi Chen, Yanhua Jiang, Jun Peng, Jiwei Xiao
CONTEXT: Gabapentin is reported to have an analgesic effect of reducing phantom-limb pain(PLP) in adult patients. There's no study on preoperative use of gabapentin in pediatric population in term of PLP prevention. OBJECTIVE: To determine whether gabapentin could be used as an adjuvant agent of opioid-based pain control to lower the rate of phantom-limb pain in pediatric patients undergoing amputation for malignant bone tumors in observation period of 60 days post-operatively...
December 5, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/29219854/reconstruction-of-the-scapula-in-pediatric-and-adolescent-patients-after-total-scapulectomy-a-report-of-10-patients-treated-by-extracorporeal-irradiation-and-reimplantation-of-the-scapula
#7
Ahmed M El Ghoneimy, Mohamed S Zaghloul, Iman Zaky, Hala Taha, Ahmed Elgammal, Magdy El Sherbiny, Nehal Kamal
BACKGROUND: Ewing sarcoma of the scapula is uncommon. Restoration of shoulder function after total scapulectomy is challenging. Extracorporeal irradiation and reimplantation is a well-known method of biological reconstruction in orthopaedic oncology; yet very few reports in the literature describe its specific use in tumors of the scapula. PATIENTS AND METHODS: Ten patients with the diagnosis of Ewing sarcoma of the scapula were surgically treated by total scapulectomy and extracorporeal irradiation and reimplantation of the scapula...
December 6, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29218302/canine-soft-tissue-sarcomas-can-being-a-dog-s-best-friend-help-a-child
#8
Bernard Séguin
Soft tissue sarcomas (STSs) remain a therapeutic challenge for pediatric and adolescent and young adult (AYA) patients. Still today, surgery, radiation therapy, and chemotherapy remain the mainstay of treatment. Obstacles in developing new treatment approaches to improve the outcome are: few patients to enroll in clinical trials, and the diversity of tumor biology between histologic subtypes. Pet dogs may offer an additional strategy to discover and test new therapeutic avenues. The number of dogs diagnosed with a STS each year in the United States is estimated to be around 27,000 to 95,000...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/29204207/primary-ewing-s-sarcoma-of-cranium-in-a-pediatric-patient
#9
Ashutosh Das Sharma, Jayesh Singh, Jigna Bhattacharya
Ewing's sarcoma primarily involving the skull is an unusual occurrence (1% of all Ewing's sarcoma cases) with very few reported cases in the published literature. The challenge in such cases is to establish a definitive diagnosis before starting the multidisciplinary treatment approach. We report such a rare case in a 13-year-old boy who is being treated at our institute with an intention of adding to the limited information we currently have on diagnosis, management, and the outcome of such cases.
July 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29201484/intracranial-myeloid-sarcoma-metastasis-mimicking-acute-subdural-hematoma
#10
Amandip S Gill, Rabina Gill, Paul Kaloostian, Dina Elias, John S Roufail, Aurora S Cruz, Panayiotis E Pelargos, Frank P K Hsu, Ronald C Kim, Robert E Ayer, Samer Ghostine
Myeloid sarcoma, a rare consequence of myeloproliferative disorders, is rarely seen in the central nervous system, most commonly in the pediatric population. Although there are a handful of case reports detailing initial presentation of CNS myeloid sarcoma in the adult population, we have been unable to find any reports of CNS myeloid sarcoma presenting as a large mass lesion in a herniating patient. Here, we present the case of a patient transferred to our facility for a very large subdural hematoma. Based on imaging characteristics, it was felt to be a spontaneous hematoma secondary to coagulopathy...
2017: Case Reports in Surgery
https://www.readbyqxmd.com/read/29200670/clinico-radiological-and-pathological-characteristics-of-inflammatory-myofibroblastic-tumors-in-children-a-retrospective-study
#11
Mohamad Hussain Mir, Waseem Dar, Sheikh Aejaz Aziz, Gul Mohamad, Burhan Wani
Purpose: Inflammatory myofibroblastic tumors (IMTs) are rare, benign lesions most often seen in the lung of young adults but can occur in children, in various sites. They mimic, clinically and radiologically, malignant tumors - especially sarcomas and lymphomas. The aim was to review the clinical, radiological, and pathological data of children with a diagnosis of IMT referred to our department. Materials and Methods: This retrospective study was conducted at the Department of Medical and Paediatric Oncology, Regional Cancer Centre, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India from January 2014 to December 2015...
July 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29200103/bcor-internal-tandem-duplication-in-high-grade-uterine-sarcomas
#12
Adrián Mariño-Enriquez, Alexandra Lauria, Joanna Przybyl, Tony L Ng, Magdalena Kowalewska, Maria Debiec-Rychter, Raji Ganesan, Vaiyapuri Sumathi, Suzanne George, W Glenn McCluggage, Marisa R Nucci, Cheng-Han Lee, Jonathan A Fletcher
Endometrial stromal sarcomas (ESSs) are mesenchymal uterine tumors characterized by recurrent genetic events, most commonly chromosomal rearrangements, that create oncogenic gene fusions. High-grade endometrial stromal sarcomas (HG-ESSs), as defined in the 2014 World Health Organization Classification, typically contain oncogenic YWHAE-NUTM2 fusions; however, although not well characterized, there are tumors morphologically overlapping with HG-ESS that do not contain the YWHAE-NUTM2 fusions. These fusions are also found in certain pediatric primitive sarcomas, including clear cell sarcoma of the kidney and soft tissue undifferentiated round cell sarcoma of infancy...
December 1, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29192742/biliary-tract-rhabdomyosarcoma-a-report-from-the-soft-tissue-sarcoma-committee-of-the-associazione-italiana-ematologia-oncologia-pediatrica
#13
Katia Perruccio, Valerio Cecinati, Angela Scagnellato, Massimo Provenzi, Giuseppe Maria Milano, Eleonora Basso, Carla Manzitti, Giovanni Cecchetto, Rita Alaggio, Martina Di Martino, Amalia Schiavetti, Fraia Melchionda, Maria Carmen Affinita, Stefano Chiaravalli, Lucia Miglionico, Rita Balter, Angela Tamburini, Gianni Bisogno, Andrea Ferrari
INTRODUCTION: Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor frequent in children. Biliary duct localization is extremely rare, but it is the most common cause of malignant obstructive jaundice in pediatric patients. METHODS: This report describes a series of 10 patients under 18 years of age with biliary tract rhabdomyosarcoma who were enrolled, from 1979 to 2004, in 3 consecutive Italian pediatric cooperative protocols that had been drawn up by the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP)...
December 1, 2017: Tumori
https://www.readbyqxmd.com/read/29191969/olaratumab-exerts-anti-tumor-activity-in-preclinical-models-of-pediatric-bone-and-soft-tissue-tumors-through-inhibition-of-platelet-derived-growth-factor-receptor-alpha
#14
Caitlin D Lowery, Wayne Blosser, Michele Dowless, Shelby Knoche, Jennifer Stephens, Huiling Li, David Surguladze, Nick Loizos, Debra Luffer-Atlas, Gerard Joseph Oakley, Qianxu Guo, Seema Iyer, Brian P Rubin, Louis F Stancato
PURPOSE: Platelet-derived growth factor receptor alpha (PDGFRα) is implicated in several adult and pediatric malignancies, where activated signaling in tumor cells and/or cells within the microenvironment drive tumorigenesis and disease progression. Olaratumab (LY3012207/IMC-3G3) is a human monoclonal antibody that exclusively binds to PDGFRα and recently received accelerated FDA approval and conditional EMA approval for treatment of advanced adult sarcoma patients in combination with doxorubicin...
November 30, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29183026/late-effects-and-long-term-follow-up-after-cancer-in-childhood
#15
Thorsten Langer, Desiree Grabow, Diana Steinmann, Bernhard Wörmann, Gabriele Calaminus
Today, 80% of children and adolescents with cancer survive their disease. From the results of aftercare research arises the question: Are the survivors also healthy? Many late effects depend on the type of cancer and its treatment. Patients with brain tumors and with malignant sarcomas are very often affected by secondary diseases. Data from the USA report that around 2/3 of all patients still living 30 years after their cancer treatment in childhood suffer from late complications. Equivalent figures for Germany were previously unavailable...
2017: Oncology Research and Treatment
https://www.readbyqxmd.com/read/29177676/extracranial-extra-cns-spread-of-embryonal-tumor-with-multilayered-rosettes-etmr-case-series-and-systematic-review
#16
Ashish H Shah, Ziad Khatib, Toba Niazi
BACKGROUND: Embryonal tumors with multilayered rosettes (ETMR) is a rare variant of embryonal tumor of infancy with nearly 200 cases reported in the literature. Leptomeningeal spread of this tumor is well known; however, extracranial metastasis has been sparsely reported in the literature. METHODS: Our study was divided into two sections: (1) We conducted a retrospective review of our patient series of ETMR and screened for patients with evidence of ETMR over the last 10 years 2007-2017 at a single tertiary referral pediatric hospital, and (2) we conducted a systematic review according to PRISMA guidelines of all reported cases of ETMR to determine the incidence of extracranial metastasis and treatment paradigms...
November 25, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29168368/a-congenital-soft-tissue-ewing-sarcoma-in-a-newborn-patient
#17
Mustafa Akçalı, Hacer Yapıcıoğlu, Eray Akay, Ferda Özlü, Banu Kozanoğlu, Kıvılcım Erdoğan, Gülfiliz Gönlüşen, Mehmet Satar
Akçalı M, Yapıcıoğlu H, Akay E, Özlü F, Kozanoğlu B, Erdoğan K, Gönlüşen G, Satar M. A congenital soft tissue Ewing sarcoma in a newborn patient. Turk J Pediatr 2017; 59: 76-79. < p < Congenital Ewing sarcoma is extremely rare. Here we present a newborn baby born with a mass on the left shoulder. Immunohistochemical staining showed congenital Ewing sarcoma. Chemotherapy and then surgical operation were planned, however the patient died before initiation of chemotherapy on the 30th day of life...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29168356/single-institution-experience-on-cancer-among-adolescents-15-19-years-of-age
#18
Begül Yağcı-Küpeli, Canan Akyüz, Bilgehan Yalçın, Ali Varan, Tezer Kutluk, Münevver Büyükpamukçu
Yağcı-Küpeli B, Akyüz C, Yalçın B, Varan A, Kutluk T, Büyükpamukçu M. Single institution experience on cancer among adolescents 15-19 years of age. Turk J Pediatr 2017; 59: 1-5. Adolescent cancers differ from other age groups in terms of cancer types, treatment and outcome. We aimed to present our institutional data on survival of certain types of cancer in adolescents. Hospital files were retrospectively evaluated for distribution of tumor types, clinical features, and overall and event-free survival (OS and EFS) rates in adolescents with malignant tumors...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29152063/acquisition-of-an-oncogenic-fusion-protein-is-sufficient-to-globally-alter-the-landscape-of-mirna-expression-to-inhibit-myogenic-differentiation
#19
Jacob M Loupe, Patrick J Miller, Judy S Crabtree, Jovanny Zabaleta, Andrew D Hollenbach
The differentiation status of tumors is used as a prognostic indicator, with tumors comprised of less differentiated cells exhibiting higher levels of aggressiveness that correlate with a poor prognosis. Although oncogenes contribute to blocking differentiation, it is not clear how they globally alter miRNA expression during differentiation to achieve this result. The pediatric sarcoma Alveolar Rhabdomyosarcoma, which is primarily characterized by the expression of the PAX3-FOXO1 oncogenic fusion protein, consists of undifferentiated muscle cells...
October 20, 2017: Oncotarget
https://www.readbyqxmd.com/read/29093183/therapeutic-targeting-of-pgbd5-induced-dna-repair-dependency-in-pediatric-solid-tumors
#20
Anton G Henssen, Casie Reed, Eileen Jiang, Heathcliff Dorado Garcia, Jennifer von Stebut, Ian C MacArthur, Patrick Hundsdoerfer, Jun Hyun Kim, Elisa de Stanchina, Yasumichi Kuwahara, Hajime Hosoi, Neil J Ganem, Filemon Dela Cruz, Andrew L Kung, Johannes H Schulte, John H Petrini, Alex Kentsis
Despite intense efforts, the cure rates of childhood and adult solid tumors are not satisfactory. Resistance to intensive chemotherapy is common, and targets for molecular therapies are largely undefined. We have found that the majority of childhood solid tumors, including rhabdoid tumors, neuroblastoma, medulloblastoma, and Ewing sarcoma, express an active DNA transposase, PGBD5, that can promote site-specific genomic rearrangements in human cells. Using functional genetic approaches, we discovered that mouse and human cells deficient in nonhomologous end joining (NHEJ) DNA repair cannot tolerate the expression of PGBD5...
November 1, 2017: Science Translational Medicine
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