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Bone sarcoma

Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
March 14, 2018: American Journal of Surgical Pathology
Nicholas Calvert, Jiansha Wu, Sophie Sneddon, Jennifer Woodhouse, Richard Carey-Smith, David Wood, Evan Ingley
Background: Soft tissue and bone sarcoma represent a broad spectrum of different pathology and genetic variance. Current chemotherapy regimens are derived from randomised trials and represent empirical treatment. Chemosensitivity testing and whole exome sequencing (WES) may offer personalized chemotherapy treatment based on genetic mutations. Methods: A pilot, prospective, non-randomised control experimental study was conducted. Twelve patients with metastatic bone or soft tissue sarcoma that had failed first line chemotherapy treatment were enrolled for this study...
2018: Clinical Sarcoma Research
Wen-Tong Zhang, Wen-Wen Zhang, Zhen-Yu He, Jia-Yuan Sun, Lei Zhang, Qing Xia, San-Gang Wu
BACKGROUND: To investigate the optimal local treatment strategies for patients with non-metastatic Ewing sarcoma (ES) of bone. METHODS: Patients with ES of bone were identified using the Surveillance Epidemiology and End Results database. Kaplan-Meier log-rank test and Cox regression models were performed to assess the effect of the types of local treatment strategies on cause-specific survival and overall survival. RESULTS: 560 patients were included with a median age of 16 years...
March 14, 2018: Expert Review of Anticancer Therapy
Christina Enciso Holm, Christian Bardram, Anja Falk Riecke, Peter Horstmann, Michael Mørk Petersen
PURPOSE: Previous studies reported variable outcome and failure rates after mega-prosthetic reconstructions in the lower extremities. The purpose of this study was to make a long-term single-center evaluation of patients treated with limb-sparing surgery and reconstruction with mega-prostheses in the lower extremities. METHODS: We identified 50 patients (osteosarcoma (n = 30), chondrosarcoma (n = 9), osteoclastoma (n = 6), Ewing sarcoma (n = 4), angiosarcoma (n = 1)), who underwent limb-sparing reconstruction of the lower extremities (proximal femur (n = 9), distal femur (n = 29), proximal tibia (n = 9), and the entire femur (n = 3)) between 1985 and 2005...
March 12, 2018: International Orthopaedics
Richard W Nicholas
No abstract text is available yet for this article.
March 2018: Clinical Orthopaedics and related Research
Matthew J Thompson, John C Shapton, Stephanie E Punt, Christopher N Johnson, Ernest U Conrad
BACKGROUND: The quantitative accuracy of MRI in predicting the intraosseous extent of primary sarcoma of bone has not been definitively confirmed, although MRI is widely accepted as an accurate tool to plan limb salvage resections. Because inaccuracies in MRI determination of tumor extent could affect the ability of a tumor surgeon to achieve negative margins and avoid local recurrence, we thought it important to assess the accuracy of MR-determined tumor extent to the actual extent observed pathologically from resected specimens in pediatric patients treated for primary sarcomas of bone...
March 2018: Clinical Orthopaedics and related Research
Luis A Aponte-Tinao, Jose I Albergo, Miguel A Ayerza, D Luis Muscolo, Federico Milano Ing, German L Farfalli
BACKGROUND: Preservation of limb function after resection of malignant bone tumors in skeletally immature children is challenging. Resection of bone sarcomas and reconstruction with an allograft in patients younger than 10 years old is one reconstructive alternative. However, long-term studies analyzing late complications and limb length discrepancy at skeletal maturity are scarce; this information would be important, because growth potential is altered in these patients owing to the loss of one physis during tumor resection...
March 2018: Clinical Orthopaedics and related Research
John A Abraham, Barry Kenneally, Kamil Amer, David S Geller
BACKGROUND: Navigation-assisted resection has been proposed as a useful adjunct to resection of malignant tumors in difficult anatomic sites such as the pelvis and sacrum where it is difficult to achieve tumor-free margins. Most of these studies are case reports or small case series, but these reports have been extremely promising. Very few reports, however, have documented benefits of navigation-assisted resection in series of pelvic and sacral primary tumors. Because this technology may add time and expense to the surgical procedure, it is important to determine whether navigation provides any such benefits or simply adds cost and time to an already complex procedure...
March 2018: Clinical Orthopaedics and related Research
Stefan Sleijfer, Elisa Rizzo, Saskia Litière, Ron H J Mathijssen, Ian Robert Judson, Hans Gelderblom, Winette T A Van Der Graaf, Alessandro Gronchi
INTRODUCTION: As both anti-tumour effects and toxicity are thought to be dose-dependent, patients with the greatest toxicity may also have the best outcome. We assessed whether severity of doxorubicin-induced hematological toxicity is associated with outcome in advanced soft tissue sarcoma (STS) patients. In addition, risk factors for hematological toxicity were explored. METHODS: Worst haematological toxicities (anaemia, leukopenia, neutropenia and thrombocytopenia) seen during treatment were scored according to CTCAE toxicity score...
March 9, 2018: Acta Oncologica
Mark J Landau, Ido Badash, Christine Yin, Ram K Alluri, Ketan M Patel
BACKGROUND AND OBJECTIVES: Vascularized bone grafting after tumor resection can be an important component in the treatment of bony neoplasms of the upper extremity. The purpose of this study was to determine the outcomes of free vascularized fibula grafting (FVFG) in the treatment of upper extremity sarcomas. METHODS: A systematic review of the literature of FVFG used in the treatment of upper extremity sarcomas was performed. RESULTS: A total of 56 studies were included in final analysis...
March 7, 2018: Journal of Surgical Oncology
Aparna Gorthi, July Carolina Romero, Eva Loranc, Lin Cao, Liesl A Lawrence, Elicia Goodale, Amanda Balboni Iniguez, Xavier Bernard, V Pragathi Masamsetti, Sydney Roston, Elizabeth R Lawlor, Jeffrey A Toretsky, Kimberly Stegmaier, Stephen L Lessnick, Yidong Chen, Alexander J R Bishop
Ewing sarcoma is an aggressive paediatric cancer of the bone and soft tissue. It results from a chromosomal translocation, predominantly t(11;22)(q24:q12), that fuses the N-terminal transactivation domain of the constitutively expressed EWSR1 protein with the C-terminal DNA binding domain of the rarely expressed FLI1 protein. Ewing sarcoma is highly sensitive to genotoxic agents such as etoposide, but the underlying molecular basis of this sensitivity is unclear. Here we show that Ewing sarcoma cells display alterations in regulation of damage-induced transcription, accumulation of R-loops and increased replication stress...
March 7, 2018: Nature
Kenji Nakano, Shunji Takahashi
Systemic treatment options for bone and soft tissue sarcomas remained unchanged until the 2000s. These cancers presented challenges in new drug development partly because of their rarity and heterogeneity. Many new molecular targeting drugs have been tried in the 2010s, and some were approved for bone and soft tissue sarcoma. As one of the first molecular targeted drugs approved for solid malignant tumors, imatinib's approval as a treatment for gastrointestinal stromal tumors (GISTs) has been a great achievement...
March 5, 2018: International Journal of Molecular Sciences
Suoyuan Li, Tao Zhang, Weiguo Xu, Jianxun Ding, Fei Yin, Jing Xu, Wei Sun, Hongsheng Wang, Mengxiong Sun, Zhengdong Cai, Yingqi Hua
PURPOSE: Osteosarcoma is the most common primary bone cancer and is notorious for pulmonary metastasis, representing a major threat to pediatric patients. An effective drug targeting osteosarcoma and its lung metastasis is urgently needed. DESIGN: In this study, a sarcoma-targeting peptide-decorated disulfide-crosslinked polypeptide nanogel (STP-NG) was exploited for enhanced intracellular delivery of shikonin (SHK), an extract of a medicinal herb, to inhibit osteosarcoma progression with minimal systemic toxicity...
2018: Theranostics
Xiaohong Li, Wenchao Li, Wuning Mo, Zheng Yang
RATIONALE: Both acute lymphoblastic leukemia (ALL) and Ewing sarcoma (ES) are small round cell tumors, and it is difficult to differential diagnose them because of overlapping clinical, radiographic, histologic, and immunophenotypic features. PATIENT'S CONCERNS: A 5-year-old boy was admitted to our hospital because of pains in his left leg without obvious inducement and lameness worsening with walking over a two 2-month period. DIAGNOSES: Based on the comprehensive analysis of radiography, magnetic resonance imaging (MRI), pathology biopsy and immunohistochemistry, the lesion was confirmed to be ES...
January 2018: Medicine (Baltimore)
S Chauhan, S A Khan, A Prasad
The present study investigates Raman scattering of human bone irradiated with 50 Gy single dose during therapeutic treatment of Ewing and Osteosarcoma. Bone quality was evaluated via mineral-to-matrix ratio, degree of crystallinity, change in amount of calcium, and carbonate substitution. Alteration in collagen and its cross-links was quantified through second-derivative deconvolution of Amide I peak. A dose of 50 Gy radiation leads to almost 50% loss of mineral content, while maintaining mineral crystallinity, and small changes in carbonate substitution...
March 2, 2018: Calcified Tissue International
Monique J L Mastboom, Floortje G M Verspoor, Daniël Uittenbogaard, Gerard R Schaap, Paul C Jutte, H W Bart Schreuder, Michiel A J van de Sande
BACKGROUND: Tenosynovial giant cell tumor (TGCT) is a rare, benign, monoarticular entity. Many case-series in adults are described, whereas TGCT is only incidentally reported in children. Therefore, its incidence rate and natural history in children are unknown. QUESTIONS/PURPOSES: (1) How many cases have been reported of this condition, and what were their characteristics? (2) What is the standardized pediatric incidence rate for TGCT? (3) Is there a clinical difference in TGCT between children and adults? (4) What is the risk of recurrence after open resection in children compared with adults? METHODS: Data were derived from three sources: (1) a systematic review on TGCT in children, seeking sources published between 1990 and 2016, included 17 heterogeneous, small case-series; (2) the nationwide TGCT incidence study: the Dutch pediatric incidence rate was extracted from this nationwide study by including patients younger than 18 years of age...
February 8, 2018: Clinical Orthopaedics and related Research
T L Yogesh, Akshay Shetty, Hema Keswani, Diljith Rishi
Ewing's sarcoma (ES) is an uncommon malignancy of childhood and adults that constitutes 6%-8% of all primary malignant tumors and the third-most common tumor after osteosarcoma and chondrosarcoma, frequently involves the mandible among jaw bones. This article presents a rare case of ES of maxilla in a 22-year-old male patient showing extensive lesion into skull base which was confirmed with computed tomography, dilemmatic histopathologic features in H and E which is not a frequent presentation. Histopathologic features showed monotonous round cells with hypo- and hyper-cellular areas, intralesional hemorrhage and necrosis with lesional cells positive for CD99...
January 2018: Journal of Oral and Maxillofacial Pathology: JOMFP
Mohamed Atef ElKordy, Tarek Sherif ElBaradie, Hisham Ismael ElSebai, Ayman Abd ElWahab Amin, Sheriff Mohamed KhairAlla
PURPOSE: Osteosarcomas rarely affect jaw bones. Patients are usually older than those who suffer long bone sarcomas, with a rare incidence of metastasis. This is suggestive of a different pattern of behavior compared with long bone sarcomas. This study aimed to present NCI, Cairo University experience in treating patients diagnosed with osteosarcomas of the jaw, including the diagnostic challenges and treatment outcome. PATIENTS AND METHODS: This is a retrospective case series study of all cases of osteosarcomas of mandible and maxilla that were treated at the NCI, in the period between 2006 and 2013...
February 26, 2018: Journal of the Egyptian National Cancer Institute
Chang Su, Cuiying Peng, Ena Agbodza, Harrison X Bai, Yuqian Huang, Giorgos Karakousis, Paul J Zhang, Zishu Zhang
BACKGROUND: The utilization and impact of the studies published using the National Cancer Database (NCDB) is currently unclear. In this study, we aim to characterize the published studies, and identify relatively unexplored areas for future investigations. METHODS: A literature search was performed using PubMed in January 2017 to identify all papers published using NCDB data. Characteristics of the publications were extracted. Citation frequencies were obtained through the Web of Science...
March 2018: Medicine (Baltimore)
Naohiro Makise, Masaya Sekimizu, Takashi Kubo, Susumu Wakai, Shun-Ichi Watanabe, Tomoyasu Kato, Takayuki Kinoshita, Nobuyoshi Hiraoka, Masashi Fukayama, Akira Kawai, Hitoshi Ichikawa, Akihiko Yoshida
AIMS: Extraskeletal osteosarcoma (ESOS) is a sarcoma in the non-skeletal tissue that directly produces neoplastic osteoid or bone. Dedifferentiated liposarcoma (DDLPS) and malignant peripheral nerve sheath tumor (MPNST) are the two most common types of sarcoma that can harbor heterologous osteosarcomatous differentiation. We aimed to determine the potential relationship of ESOS to DDLPS and MPNST. METHODS AND RESULTS: We investigated MDM2 and H3K27me3 status in 19 cases of ESOS, 2 of which contained a low-grade component...
February 28, 2018: Histopathology
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