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Pediatric sarcoma

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https://www.readbyqxmd.com/read/28816792/development-of-secondary-acute-myeloid-leukemia-in-a-pediatric-patient-concurrently-receiving-primary-therapy-for-ewing-sarcoma
#1
Brandon R McNew, Benjamin W Darbro, Deqin Ma, David J Gordon
Ewing sarcoma is a pediatric bone and soft tissue sarcoma that requires intensive therapy, which can cause secondary malignancies. We present a rare case of early, treatment-related AML in a pediatric patient concurrently receiving primary therapy for Ewing sarcoma. Despite AML-directed therapy, our patient died secondary to complications of hyperleukocytosis. Cytogenetic and mutation profiling of the leukemia cells revealed the DNA-topoisomerase-II-inhibitor-associated t(9;11)(p22;q23) translocation and clonal KRAS and BRAF mutations...
August 14, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28806574/quantitative-diffusion-weighted-magnetic-resonance-imaging-assessment-of-chemotherapy-treatment-response-of-pediatric-osteosarcoma-and-ewing-sarcoma-malignant-bone-tumors
#2
Andrew J Degnan, Chul Y Chung, Amisha J Shah
OBJECTIVE: Assessment of tumor response to chemotherapy is essential in managing malignant pediatric bone tumors prior to resection. SUBJECTS AND METHODS: Pre-chemotherapy and post-chemotherapy osteosarcoma and Ewing sarcoma cases (n=18) were analyzed with apparent diffusion coefficient (ADC) values measured by two readers. RESULTS: Treated tumors demonstrated significantly greater ADC values compared to untreated tumors (p<0.001). Intraclass correlation coefficients ranged between 0...
August 5, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28803261/advantages-in-prognosis-of-adult-patients-with-ewing-sarcoma-11-years-experiences-and-current-treatment-management
#3
Dagmar Adamkova Krakorova, Katerina Kubackova, Ladislav Dusek, Tomas Tomas, Pavel Janicek, Stepan Tucek, Jana Prausova, Igor Kiss, Iva Zambo
Ewing sarcoma (ES) is an exceptionally rare tumor in adults. Data regarding outcomes of adult patients with ES and experiences with age-adapted therapeutic strategies are very limited. The aim of this study was to evaluate prognostic factors and clinical outcome in a cohort of adult patients treated according to pediatric protocols in the Czech Republic. The records of 58 adult ES patients diagnosed between 2002 and 2013 were reviewed and factors relevant to prognosis and survival were analyzed. The median age of study cohort was 29 years (range, 18-59)...
August 12, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28802390/pediatric-head-and-neck-bone-sarcomas-an-analysis-of-204-cases
#4
Jacob S Brady, Sei Y Chung, Emily Marchiano, Jean Anderson Eloy, Soly Baredes, Richard Chan Woo Park
OBJECTIVES: To analyze the demographics, survival, and treatment efficacy of pediatric sarcomas of the facial skeleton and skull. METHODS: Retrospective study of cases from the US National Cancer Institute's Surveillance, Epidemiology, and End Results database. Pediatric patients between the ages of 0 and 18 diagnosed with a malignant sarcoma of either the mandible or the bones of skull, face, and associated joints from 1973 to 2013 were studied. RESULTS: In total, 204 patients were included in the analysis...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28766068/pediatric-soft-tissue-sarcoma-of-the-limbs-clinical-outcome-of-97-patients
#5
Andrea Sambri, Giuseppe Bianchi, Ilaria Cucurnia, Marco Gambarotti, Davide Maria Donati
PURPOSE: Soft tissue sarcomas (STS) of the extremities in children are a heterogeneous group of tumors with a very different prognosis for which optimal treatment remains controversial. PATIENTS AND METHODS: We retrospectively evaluated 97 patients younger than 15 years old affected by limb soft tissue sarcomas. All cases were histologically revised, and tumor grade was assessed according to the FNLCC system. Thirty-two were rhabdomyosarcoma (RMS) and 65 non-rhabdomyosarcoma (NRMSTS); among these, 40 (61...
August 1, 2017: European Journal of Orthopaedic Surgery & Traumatology: Orthopédie Traumatologie
https://www.readbyqxmd.com/read/28765612/pediatric-sarcoma-data-forms-a-unique-cluster-measured-via-the-earth-mover-s-distance
#6
Yongxin Chen, Filemon Dela Cruz, Romeil Sandhu, Andrew L Kung, Prabhjot Mundi, Joseph O Deasy, Allen Tannenbaum
In this note, we combined pediatric sarcoma data from Columbia University with adult sarcoma data collected from TCGA, in order to see if one can automatically discern a unique pediatric cluster in the combined data set. Using a novel clustering pipeline based on optimal transport theory, this turned out to be the case. The overall methodology may find uses for the classification of data from other biological networking problems.
August 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28764925/profile-of-european-proton-and-carbon-ion-therapy-centers-assessed-by-the-eortc-facility-questionnaire
#7
Damien C Weber, André Abrunhosa-Branquinho, Alessandra Bolsi, Andrzej Kacperek, Rémi Dendale, Dirk Geismar, Barbara Bachtiary, Annika Hall, Jens Heufelder, Klaus Herfarth, Jürgen Debus, Maurizio Amichetti, Mechthild Krause, Roberto Orecchia, Vladimir Vondracek, Juliette Thariat, Tomasz Kajdrowicz, Kristina Nilsson, Cai Grau
BACKGROUND: We performed a survey using the modified EORTC Facility questionnaire (pFQ) to evaluate the human, technical and organizational resources of particle centers in Europe. MATERIAL AND METHODS: The modified pFQ consisted of 235 questions distributed in 11 sections accessible on line on an EORTC server. Fifteen centers from 8 countries completed the pFQ between May 2015 and December 2015. RESULTS: The average number of patients treated per year and per particle center was 221 (range, 40-557)...
July 29, 2017: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/28762137/a-clinicopathologic-study-of-head-and-neck-malignant-peripheral-nerve-sheath-tumors
#8
Adepitan A Owosho, Cherry L Estilo, Joseph M Huryn, Ping Chi, Cristina R Antonescu
Head and neck high grade malignant peripheral nerve sheath tumors (HN-MPNSTs) are rare highly aggressive soft tissue sarcomas that show overlapping morphologic and immunophenotypic features with melanoma and other high grade sarcomas, resulting in diagnostic challenges, particularly in sporadic settings. Recent discoveries have implicated loss of function mutations in the polycomb repressive complex 2 (PRC2) components, including EED or SUZ12 genes, as one of the leading pathogenetic mechanisms in high grade MPNST...
July 31, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28759114/nonrhabdomyosarcoma-soft-tissue-sarcoma-nrsts-in-pediatric-and-young-adult-patients-results-from-a-prospective-study-using-limited-margin-radiotherapy
#9
Christopher L Tinkle, Israel Fernandez-Pineda, April Sykes, Zhaohua Lu, Chia-Ho Hua, Michael D Neel, Armita Bahrami, Barry L Shulkin, Sue C Kaste, Alberto Pappo, Sheri L Spunt, Matthew J Krasin
BACKGROUND: Indications for and delivery of adjuvant therapies for pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) have been derived largely from adult studies; therefore, significant concern remains regarding radiation exposure to normal tissue. The authors report long-term treatment outcomes and toxicities for pediatric and young adult patients with high-grade NRSTS who were treated on a prospective trial using limited-margin radiotherapy. METHODS: Sixty-two patients (ages 3-22 years) with predominantly high-grade NRSTS requiring radiation were treated on a phase 2 institutional study of conformal external-beam radiotherapy and/or brachytherapy using a 1...
July 31, 2017: Cancer
https://www.readbyqxmd.com/read/28756644/the-role-of-anaplastic-lymphoma-kinase-in-pediatric-cancers
#10
REVIEW
Junko Takita
The anaplastic lymphoma kinase (ALK) was initially identified as a fusion partner of the nucleophosmin gene in anaplastic large cell lymphoma (ALCL) with t(2;5)(p23;q35) translocation, and then described with different genetic abnormalities in a number of tumors. Although ALK is known to be involved in the pathogenesis of neuroblastoma through activating mutations or gene amplification, its role in the pathogenesis of other pediatric cancers is still elusive. In addition to neuroblastoma, the high-grade amplification of ALK has been described in a subset of rhabdomyosarcoma cases...
July 30, 2017: Cancer Science
https://www.readbyqxmd.com/read/28748542/pediatric-intracerebral-histiocytic-sarcoma-with-rhabdoid-features-case-report-and-literature-review
#11
Young Hye Kim, Gie-Taek Yie, Na Rae Kim, In-Sang Jeon, Hyun Yee Cho, Jae Yeon Seok, Eung Yeop Kim, Kyu Chan Lee
A 16-year-old boy presented with marked weight loss, weakness of the left extremities and dizziness of 2 months duration and vomiting for 2 days. Brain MRI showed an approximately 6.5 × 5.3 cm-sized huge heterogeneous enhancing mass located in the corpus callosum, extending into the lateral ventricle. Open biopsy showed that the lesion consisted of lymphoplasmacytes and plump histiocytes with rhabdoid morphology, which were stained with S-100 protein, CD68 (KP1) and negative for CD1a. Histiocytic tumor was initially diagnosed...
July 27, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28744074/multimodality-imaging-of-vaginal-rhabdomyosarcoma
#12
Richa S Chauhan, Dheeraj K Singh, Bishwarup Guha, Ishan Kumar, Ashish Verma
Rhabdomyosarcoma (RMS) is a malignant mesenchymal tumor arising from the embryonal muscle cells (rhabdomyoblasts), and is the most common soft tissue sarcoma in children and young adults accounting for 4-6% of all malignancies in this age group. Though rare overall, embryonal rhabdomyosarcoma is the most common malignancy arising in the pediatric female genitourinary tract with sarcoma botryoides being the most common variant of the tumor. In young and adolescent individuals, the cervix and uterus are affected; whereas in infants, vaginal lesions are more common...
April 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/28735378/a-phase-i-ii-trial-and-pharmacokinetic-study-of-mithramycin-in-children-and-adults-with-refractory-ewing-sarcoma-and-ews-fli1-fusion-transcript
#13
Patrick J Grohar, John Glod, Cody J Peer, Tristan M Sissung, Fernanda I Arnaldez, Lauren Long, William D Figg, Patricia Whitcomb, Lee J Helman, Brigitte C Widemann
PURPOSE: In a preclinical drug screen, mithramycin was identified as a potent inhibitor of the Ewing sarcoma EWS-FLI1 transcription factor. We conducted a phase I/II trial to determine the dose-limiting toxicities (DLT), maximum tolerated dose (MTD), and pharmacokinetics (PK) of mithramycin in children with refractory solid tumors, and the activity in children and adults with refractory Ewing sarcoma. PATIENTS AND METHODS: Mithramycin was administered intravenously over 6 h once daily for 7 days for 28 day cycles...
July 22, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/28724045/spinal-tumors-in-children
#14
Andrei Fernandes Joaquim, Enrico Ghizoni, Marcelo Gomes Cordeiro Valadares, Simone Appenzeller, Simone Dos Santos Aguiar, Helder Tedeschi
Introduction: Spinal tumors are rare in the pediatric population, presenting many specific peculiarities when compared to adults. We have performed a broad narrative review to describe the most common spinal tumors in children, discussing their main characteristics and management options. Method: The authors have performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives. Results: Multimodality radiological studies (plain films, 3D computed tomography scan and magnetic resonance imaging) are necessary for proper evaluation and differential diagnosis of spinal tumors in children...
May 2017: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/28710342/vincristine-ifosfamide-and-doxorubicin-for-initial-treatment-of-ewing-sarcoma-in-adults
#15
Michael J Wagner, Vancheswaran Gopalakrishnan, Vinod Ravi, J Andrew Livingston, Anthony P Conley, Dejka Araujo, Neeta Somaiah, Maria A Zarzour, Ravin Ratan, Wei-Lien Wang, Shreyaskumar R Patel, Alexander Lazar, Joseph A Ludwig, Robert S Benjamin
BACKGROUND: There are no clinical trials specifically addressing chemotherapy for adults with Ewing sarcoma (ES). Five-year event-free survival (EFS) of adults on pediatric studies of ES (44%-47%) is worse than that of children treated with the same therapy (69%). The object of this study was to review the results of therapy with vincristine, ifosfamide, and doxorubicin (VID) in the multidisciplinary treatment of adults with ES at our institution. MATERIALS AND METHODS: Charts for adults treated for ES from 1995 to 2011 were retrospectively reviewed...
July 14, 2017: Oncologist
https://www.readbyqxmd.com/read/28699149/is-the-clavicula-pro-humero-technique-of-value-for-reconstruction-after-resection-of-the-proximal-humerus-in-children
#16
Dominique Barbier, Benoît De Billy, Philippe Gicquel, Sophie Bourelle, Pierre Journeau
BACKGROUND: There are several options for reconstruction of proximal humerus resections after wide resection for malignant tumors in children. The clavicula pro humero technique is a biologic option that has been used in the past, but there are only scant case reports and small series that comment on the results of the procedure. Because the longevity of children mandates a reconstruction with potential longevity not likely to be achieved by other techniques, the clavicula pro humero technique may be a potential option in selected patients...
July 11, 2017: Clinical Orthopaedics and related Research
https://www.readbyqxmd.com/read/28697168/pretreatment-neutrophil-to-lymphocyte-ratio-and-lymphocyte-recovery-independent-prognostic-factors-for-survival-in-pediatric-sarcomas
#17
Liliana Vasquez, Esmeralda León, Brady Beltran, Ivan Maza, Monica Oscanoa, Jenny Geronimo
BACKGROUND: Pretreatment neutrophil-to-lymphocyte ratio (NLR) and absolute lymphocyte count (ALC) recovery have been shown to be associated with prognosis in several types of cancer in adults. However, evidence in pediatric cancer is scarce. The aim of our study was to evaluate whether pretreatment NLR and lymphocyte recovery are prognostic factors in pediatric sarcomas. MATERIALS AND METHODS: Study participants were identified from a retrospective cohort of 100 children with osteosarcoma (n=55), rhabdomyosarcoma (n=22), and Ewing sarcoma (n=23)...
July 10, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28696047/a-randomized-trial-of-the-effectiveness-of-the-neutropenic-diet-versus-food-safety-guidelines-on-infection-rate-in-pediatric-oncology-patients
#18
Karen M Moody, Rebecca A Baker, Ruth O Santizo, Inan Olmez, Jeanie M Spies, Amanda Buthmann, Linda Granowetter, Robin Y Dulman, Kanyalakshmi Ayyanar, Jonathan B Gill, Aaron E Carroll
BACKGROUND: The neutropenic diet (ND) is prescribed to avoid introduction of bacteria into a host's gastrointestinal tract and reduce infection. Due to a lack of evidence to support the ND, there continues to be debate among pediatric oncologists regarding its usefulness. This prospective randomized controlled trial evaluated the difference in neutropenic infection rates in pediatric oncology patients randomized to Food and Drug Administration approved food safety guidelines (FSGs) versus the ND plus FSGs during one cycle of chemotherapy...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28696044/practice-patterns-of-palliative-radiation-therapy-in-pediatric-oncology-patients-in-an-international-pediatric-research-consortium
#19
Avani Dholakia Rao, Qinyu Chen, Ralph P Ermoian, Sara R Alcorn, Maria Luisa S Figueiredo, Michael J Chen, Karin Dieckmann, Shannon M MacDonald, Matthew M Ladra, Daria Kobyzeva, Alexey V Nechesnyuk, Kristina Nilsson, Eric C Ford, Brian A Winey, Rosangela C Villar, Stephanie A Terezakis
BACKGROUND/OBJECTIVES: The practice of palliative radiation therapy (RT) is based on extrapolation from adult literature. We evaluated patterns of pediatric palliative RT to describe regimens used to identify opportunity for future pediatric-specific clinical trials. DESIGN/METHODS: Six international institutions with pediatric expertise completed a 122-item survey evaluating patterns of palliative RT for patients ≤21 years old from 2010 to 2015. Two institutions use proton RT...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28690908/primary-occipital-ewing-s-sarcoma-with-subsequent-spinal-seeding
#20
Ali Alqahtani, Roaa Amer, Eman Bakhsh
Ewing's sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing's sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis...
2017: Case Reports in Pediatrics
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