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https://www.readbyqxmd.com/read/29468476/current-treatment-of-pediatric-bladder-and-prostate-rhabdomyosarcoma
#1
REVIEW
Amanda F Saltzman, Nicholas G Cost
PURPOSE OF REVIEW: Rhabdomyosarcoma (RMS) is the most common sarcoma diagnosed in the first 20 years of life; bladder/prostate (BP) RMS accounts for 5% of all cases. Through efforts from multiple cooperative study groups, survival has improved significantly. This article aims to review the complex RMS classification system and treatment of BP RMS, with a focus on developing aspects of treatment. RECENT FINDINGS: Recent advancements in technology are responsible for most of the progress in RMS treatment...
February 22, 2018: Current Urology Reports
https://www.readbyqxmd.com/read/29464349/treatment-and-outcome-of-patients-with-localized-intrathoracic-and-chest-wall-rhabdomyosarcoma-a-report-of-the-cooperative-weichteilsarkom-studiengruppe-cws
#2
Jörg Fuchs, Cristian Urla, Monika Sparber-Sauer, Andreas Schuck, Ivo Leuschner, Thomas Klingebiel, Gunnar Blumenstock, Guido Seitz, Ewa Koscielniak
PURPOSE: Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma. In 7% of the cases it is localized at the chest wall or intrathoracically. The aim of this study was to analyze the multimodal treatment concepts and outcomes of children suffering from intrathoracic and chest wall RMS treated within three different Cooperative Soft Tissue Sarcoma (CWS) trials and one registry (Soft Tissue Sarcoma Registry, SoTiSaR). METHODS: Data of 51 patients with thoracic RMS enrolled in three different CWS trials (CWS-86, -91, -2002P) and one registry (SoTiSaR) were analyzed retrospectively...
February 20, 2018: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/29443850/does-an-algorithmic-approach-to-using-brachytherapy-and-external-beam-radiation-result-in-good-function-local-control-rates-and-low-morbidity-in-patients-with-extremity-soft-tissue-sarcoma
#3
Jason Klein, Alex Ghasem, Samuel Huntley, Nathan Donaldson, Martin Keisch, Sheila Conway
BACKGROUND: High-dose-rate brachytherapy (HDR-BT) and external-beam radiation therapy (EBRT) are two modalities used in the treatment of soft tissue sarcoma. Previous work at our institution showed early complications and outcomes for patients treated with HDR-BT, EBRT, or a combination of both radiation therapy modalities. As the general indications for each of these approaches to radiation therapy differ, it is important to evaluate the use of each in an algorithmic way, reflecting how they are used in contemporary practice at sites that use these treatments...
March 2018: Clinical Orthopaedics and related Research
https://www.readbyqxmd.com/read/29434937/prognostic-value-and-functional-role-of-rock2-in-pediatric-ewing-sarcoma
#4
Gabriela Maciel Vieira, Gabriela Molinari Roberto, Régia Caroline Lira, Edgard Eduard Engel, Luiz Gonzaga Tone, María Sol Brassesco
Ewing's sarcoma (EWS) is a highly aggressive bone cancer that affects children and adolescents. Despite advances in multimodal management, 5-year event-free survival rates for patients presenting with metastases at diagnosis remain at 25%. As key regulators of actin organization, the Rho-associated coiled-coil containing protein kinases, ROCK1 and ROCK2, have been associated with cancer dissemination and poorer prognosis. Recently, in vitro data indicating ROCK2 as a molecular target for the treatment of EWS has been presented...
February 2018: Oncology Letters
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/29431250/esophagitis-associated-with-multimodality-management-of-pediatric-ewing-sarcoma-of-thorax
#6
Vibhuti Agarwal, Natalie Logie, Christopher G Morris, Julie A Bradley, Ronny L Rotondo, Scott M Bradfield, Daniel J Indelicato
BACKGROUND: Ewing sarcoma of the thoracic spine and chest wall is frequently treated with concurrent chemotherapy and radiation therapy (RT). Treatment-related acute esophagitis can lead to hospitalization and treatment delays. The aim of this study was to analyze the incidence, risk factors, and management of esophagitis in pediatric patients with Ewing sarcoma of the thoracic region. METHODS: We conducted a single-institution retrospective review of patients treated over a 10-year period...
February 12, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29426275/primary-renal-hybrid-low-grade-fibromyxoid-sarcoma-sclerosing-epithelioid-fibrosarcoma-an-unusual-pediatric-case-with-ewsr1-creb3l1-fusion
#7
Yingting Mok, Yin Huei Pang, Jain Sudhanshi Sanjeev, Chik Hong Kuick, Kenneth Tou-En Chang
Low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF) are rare tumors with distinct sets of morphological features, both characterized by MUC4 immunoreactivity. Tumors exhibiting features of both entities are considered hybrid LGFMS-SEF lesions. While the majority of LGFMS cases are characterized by FUS-CREB3L2 gene fusions, most cases of pure SEF show EWSR1 gene rearrangements. In the largest study of hybrid LGFMS-SEF tumors to date, all cases exhibited FUS rearrangements, a similar genetic profile to LGFMS...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29418008/double-barrel-vascularized-dual-fibula-transfer-with-epiphyseal-growth-plate-for-hip-reconstruction-a-case-report
#8
Fabio Santanelli di Pompeo, Gennaro Selvaggi, Benedetto Longo, Rosaria Laporta, Vittoria Amorosi, Michail Sorotos
We present a case report of a 10-year-old girl diagnosed with Ewing sarcoma treated with intra-articular wide resection of the right femur and reconstruction with a series-connected double-barrel bilateral vascularized fibula graft (db-BVFG), including fibular head for articulation with the acetabulum of the pelvic bone and preservation of the epiphyseal growth plates for eventual limb growth. No postoperative complications were observed and bone union was achieved with fibular graft hypertrophy, allowing for full weight bearing...
February 8, 2018: Microsurgery
https://www.readbyqxmd.com/read/29416174/considerations-for-the-long-term-treatment-of-pediatric-sarcoma-survivors
#9
Kurt R Weiss, Melissa N Zimel
Sarcomas are primary malignancies of the connective tissues. They are exceedingly rare in adults, but much more common in children. The historically recent advent of cytotoxic chemotherapy for pediatric sarcomas has revolutionized the treatment of these diseases and dramatically improved their prognoses. There is thus a population of pediatric sarcoma survivors that are "coming of age" as adults. However, this progress is not without consequences. Due to aggressive treatment protocols that include various combinations of surgery, chemotherapy, and radiation therapy, pediatric sarcoma survivors are at risk of myriad physical, medical, and psychological difficulties as they enter adulthood...
January 2018: Indian Journal of Orthopaedics
https://www.readbyqxmd.com/read/29398803/therapy-related-aml-mds-following-treatment-for-childhood-cancer-experience-from-a-tertiary-care-centre-in-north-india
#10
Chintan Vyas, Sandeep Jain, Gauri Kapoor
Therapy-related acute myeloid leukemia/myelodysplastic syndrome (t-AML/MDS) is a devastating late effect of cancer treatment. There is limited data on incidence of t-AML/MDS from India. We retrospectively studied pediatric t AML/MDS at our institute between January 1996 and December 2015. Among 1285 children, 8 patients developed t-AML with a median age of 15.5 years. Overall incidence of t-AML/MDS was 0.62% [0.99% (4/402) in solid tumours and 0.45% (4/883) in leukemia/lymphoma, P = 0.26] with 6390 patient years of follow up...
January 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29397557/targeting-histone-deacetylase-activity-to-arrest-cell-growth-and-promote-neural-differentiation-in-ewing-sarcoma
#11
Bárbara Kunzler Souza, Patrícia Luciana da Costa Lopez, Pâmela Rossi Menegotto, Igor Araujo Vieira, Nathalia Kersting, Ana Lúcia Abujamra, André T Brunetto, Algemir L Brunetto, Lauro Gregianin, Caroline Brunetto de Farias, Carol J Thiele, Rafael Roesler
There is an urgent need for advances in the treatment of Ewing sarcoma (EWS), an aggressive childhood tumor with possible neuroectodermal origin. Inhibition of histone deacetylases (HDAC) can revert aberrant epigenetic states and reduce growth in different experimental cancer types. Here, we investigated whether the potent HDAC inhibitor, sodium butyrate (NaB), has the ability to reprogram EWS cells towards a more differentiated state and affect their growth and survival. Exposure of two EWS cell lines to NaB resulted in rapid and potent inhibition of HDAC activity (1 h, IC50 1...
February 3, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29397238/hereditary-swi-snf-complex-deficiency-syndromes
#12
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/29391313/post-transplant-malignancies-in-pediatric-liver-transplant-recipients-experience-of-two-centers-in-turkey
#13
Miray Karakoyun, Şebnem Önen, Maşallah Baran, Murat Çakır, Çiğdem Ömür Ecevit, Murat Kılıç, Mehmet Kantar, Serap Aksoylar, Funda Özgenç, Sema Aydoğdu
BACKGROUND/AIMS: A liver transplant is the preferred treatment for patients with end-stage liver disease, as it usually results in longterm survival. However, due to the use of chronic immunosuppressive therapy, which is necessary to prevent rejection, de novo cancer is a major risk after transplantation. The aim of this study was to assess the incidence of post-transplant malignancies in children after liver transplantations. MATERIALS AND METHODS: The study group consisted of 206 liver transplant recipients, with no history of cancer, including hepatocellular carcinoma, in two liver transplantation centers in Turkey between 1997 and 2015...
January 2018: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/29390436/endostar-combined-with-chemotherapy-in-a-pediatric-osteosarcoma-with-pulmonary-metastasis-and-malignant-pleural-effusion-a-case-report
#14
Sujing Jiang, Guannan Wang, Ying Dong
RATIONALE: Angiogenesis is a key factor for tumor growth and metastasis both in cancer and sarcoma. Endostar, a novel safe and well-tolerated recombinant human endostatin, can suppress the expression of VEGF and the activation of ERK, MAPK, and AKT, and then inhibit tumor progression. PATIENT CONCERNS: A pediatric osteosarcoma with pulmonary metastasis and malignant pleural effusion. DIAGNOSES: Osteosarcoma with pulmonary metastasis and malignant pleural effusion...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29387307/bone-allograft-segment-covered-with-a-vascularized-fibular-periosteal-flap-a-new-technique-for-pediatric-mandibular-reconstruction
#15
Nicolas E Sierra, Paula Diaz-Gallardo, Jorge Knörr, Vasco Mascarenhas, Eloy García-Diez, Montserrat Munill-Ferrer, Maria S Bescós-Atín, Francisco Soldado
The free vascularized fibular graft is nowadays the preferred technique for pediatric mandibular reconstruction. Despite the versatility and proven efficacy for restoring the facial appearance and maxillomandibular function, those mandibular reconstructions with free vascularized fibula associate difficulties for a simultaneous restoration of the alveolar height and facial contour, which are derived from the height discrepancy between the fibula and the native mandible. In addition, the donor-site growth and morbidity are of special concern in the pediatric patient...
March 2018: Craniomaxillofacial Trauma & Reconstruction
https://www.readbyqxmd.com/read/29383611/desmoplastic-small-round-cell-tumor-treated-with-cytoreductive-surgery-and-hyperthermic-intraperitoneal-chemotherapy-results-of-a-phase-2-trial
#16
Andrea A Hayes-Jordan, Brian A Coakley, Holly L Green, LianChun Xiao, Keith F Fournier, Cynthia E Herzog, Joseph A Ludwig, Mary F McAleer, Peter M Anderson, Winston W Huh
BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma that primarily affects adolescents and young adults. Patients can present with many peritoneal implants. We conducted a phase 2 clinical trial utilizing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) with cisplatin for DSRCT and pediatric-type abdominal sarcomas. PATIENTS AND METHODS: A prospective cohort study was performed on 20 patients, who underwent CRS-HIPEC procedures, with cisplatin from 2012 to 2013...
January 30, 2018: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/29375950/embryonal-rhabdomyosarcoma-of-the-cervix-a-rare-disease-at-an-uncommon-age
#17
Uroosa Ibrahim, Amina Saqib, Farhan Mohammad, Juan Ding, Blerina Salman, Fady K Collado, Meekoo Dhar
Embryonal rhabdomyosarcoma (RMS) is a rare type of sarcoma, primarily seen in the pediatric and adolescent population. Three subtypes of embryonal RMS are described, with the botryoid type being the most common. The incidence of this disease in adult females is 0.4% to 1% with the affected age group being patients in the third to fourth decade of life. It is exceedingly rare in patients above 40 years of age. We describe the case of a 48-year-old female, gravida 9 para 5, who presented with abnormal vaginal bleeding and an exophytic mass on examination...
November 21, 2017: Curēus
https://www.readbyqxmd.com/read/29368010/importance-of-whole-body-imaging-with-complete-coverage-of-hands-and-feet-in-alveolar-rhabdomyosarcoma-staging
#18
Monika Scheer, Tobias Dantonello, Peter Brossart, Dagmar Dilloo, Lothar Schweigerer, Simone Feuchtgruber, Monika Sparber-Sauer, Christian Vokuhl, Stefan S Bielack, Thomas Klingebiel, Ewa Koscielniak, Thekla von Kalle
BACKGROUND: Alveolar rhabdomyosarcoma commonly arises in the extremities and is characterized by aggressive biology and high frequency of metastases. Whole-body imaging is increasingly employed in pediatric oncology but not recommended as standard in the staging of soft-tissue sarcomas. OBJECTIVE: After observing patients with a large symptomatic alveolar rhabdomyosarcoma lesion and a smaller silent lesion in the more distal part of an extremity we sought to estimate the frequency of this constellation...
January 24, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29343557/dicer1-and-associated-conditions-%C3%A2-identification-of-at-risk-individuals-and-recommended-surveillance-strategies
#19
Kris Ann P Schultz, Gretchen M Williams, Junne Kamihara, Douglas R Stewart, Anne K Harris, Andrew J Bauer, Joyce Turner, Rachana Shah, Katherine Schneider, Kami Wolfe Schneider, Ann Garrity Carr, Laura A Harney, Shari Baldinger, A Lindsay Frazier, Daniel Orbach, Dominik T Schneider, David Malkin, Louis P Dehner, Yoav H Messinger, Ashley Hill
Pathogenic germline DICER1 variants cause a hereditary cancer predisposition syndrome with a variety of manifestations. In addition to conferring increased cancer risks for pleuropulmonary blastoma (PPB) and ovarian sex cord-stromal tumors, particularly Sertoli-Leydig cell tumor, individuals with pathogenic germline DICER1 variants may also develop lung cysts, cystic nephroma, renal sarcoma and Wilms tumor, nodular hyperplasia of the thyroid, nasal chondromesenchymal hamartoma, ciliary body medulloepithelioma, genitourinary embryonal rhabdomyosarcoma and brain tumors including pineoblastoma and pituitary blastoma...
January 17, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29335623/sinusoidal-obstruction-syndrome-veno-occlusive-disease-after-high-dose-intravenous-busulfan-melphalan-conditioning-therapy-in-high-risk-ewing-sarcoma
#20
Massimo Eraldo Abate, Anna Paioli, Sivlia Cammelli, Marilena Cesari, Alessandra Longhi, Emanuela Palmerini, Stefano Ferrari, Elisa Carretta, Piero Picci, Fabio Piscaglia
This mono-institutional observational study was conducted to determine incidence, severity, risk factors, and outcome of sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) in high-risk Ewing sarcoma (ES) patients treated with intravenous busulfan and melphalan (BU-MEL) followed by autologous stem cell transplantation (ASCT). During the past 10 years, 75 consecutive ES patients resulted evaluable for the analysis. After diagnosis of SOS/VOD, defibrotide therapy was started as soon as the medication was available...
January 15, 2018: Bone Marrow Transplantation
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