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Shailaja Raj, Lance D Miller, Pierre L Triozzi
Sarcoma is comprised of a heterogeneous group of tumors originating from the mesenchyme. Sarcoma is also the first tumor that responded to immunotherapeutic agents often termed as "Coley's toxins." However, immunotherapy is yet to establish its presence in sarcomas. Complex interactions between tumor and immune cells in the tumor microenvironment play a crucial role in response to immunotherapy. There is a dynamic equilibrium created by the immune cells infiltrating the tumor, and this forms the basis of tumor evasion...
2018: Sarcoma
Kevin Campbell, David Shulman, Katherine A Janeway, Steven G DuBois
Background: As of 2013, the WHO has classified peripheral primitive neuroectodermal tumors (PNETs) within the umbrella of Ewing sarcoma family of tumors (ESFTs) given their shared biology. Histologic features differ between PNET and Ewing sarcoma (ES), and potential clinical differences between PNET and ES have not been fully elucidated. Methods: Through the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database, we identified 3,575 patients identified with histologic diagnosis of ES or PNET from 1973 to 2014...
2018: Sarcoma
Sanjay P Bagaria, Matthew Neville, Richard J Gray, Emmanuel Gabriel, Jonathan B Ashman, Steven Attia, Nabil Wasif
Background: We sought to study the association between RPS case volume and outcomes. Although a relationship has been demonstrated between case volume and patient outcomes in some cancers, such a relationship has not been established for retroperitoneal sarcomas (RPSs). Study Design: The National Cancer Database (NCDB) was queried for patients undergoing treatment for primary RPS diagnosed between 2004 and 2013. Mean annual patient volume for RPS resection was calculated for all hospitals and divided into low volume (<5 cases/year), medium volume (5-10 cases/year), and high volume (>10 cases/year)...
2018: Sarcoma
Eunsun Oh, Sung Wook Seo, Kwang Joon Han
Background: Many studies have reported on the surgical outcomes of soft tissue sarcoma. However, there was no longitudinal cohort study. Because time is the most valuable factor for functional recovery, adjusting time value was the key for finding the causal relationship between other risk factors and postoperative function. Therefore, existing cross-sectional studies can neither fully explain the causal relationship between the risk factors and the functional score nor predict functional recovery...
2018: Sarcoma
Danique L M van Broekhoven, Arie J Verschoor, Thijs van Dalen, Dirk J Grünhagen, Michael A den Bakker, Hans Gelderblom, Judith V M G Bovee, Rick L M Haas, Han J Bonenkamp, Frits van Coevorden, Diederik Ten Oever, Winette T A van der Graaf, Uta E Flucke, Elisabeth Pras, Anna K L Reyners, Anneke M Westermann, Foppe Oldenburger, Cornelis Verhoef, Neeltje Steeghs
Introduction: Nonsurgical management of patients with desmoid-type fibromatosis (DF) is increasing. This study tries to provide insight on type, usage, and outcome of first-line nonsurgical management strategies. Patients and Methods: From the Dutch Pathology Registry (PALGA), patients with extra-abdominal or trunk/abdominal wall DF, diagnosed between 1993 and 2013, were identified. First-line treatment was analyzed. Best response (BR) using RECIST criteria from start of treatment/surveillance until change of treatment or last follow-up was analyzed...
2018: Sarcoma
J Brock Walker, Erin Stockwell, Kellen Worhacz, Paul Kang, Amalia Decomas
Background: Percutaneous needle biopsy has been found to be a safe and accurate method for the initial investigation of soft tissue masses. The notion exists that needle biopsies should be performed in specialized sarcoma centers, which can place a financial burden on patients without a sarcoma center near their place of residence. There is no consensus in the current literature regarding the diagnostic accuracy and clinical utility of clinic-based percutaneous core needle biopsy performed by community orthopedic surgeons with fellowship training in musculoskeletal oncology...
2018: Sarcoma
Saurabh P Nagar, Daniel S Mytelka, Sean D Candrilli, Yulia D'yachkova, Maria Lorenzo, Bernd Kasper, Jose Antonio Lopez-Martin, James A Kaye
Objective: To describe real-world treatment patterns and outcomes for patients with advanced soft tissue sarcoma (STS) not amenable to surgery or radiotherapy in the United Kingdom, Spain, Germany, and France. Methods: Physicians completed a web-based medical record abstraction for adult patients with advanced STS (other than Kaposi's sarcoma or gastrointestinal stromal tumor) who received ≥1 line of systemic therapy. Clinical characteristics, treatments, tumor responses, and mortality data were recorded...
2018: Sarcoma
Pavan Tenneti, Umar Zahid, Ahmad Iftikhar, Seongseok Yun, Atif Sohail, Zabih Warraich, Faiz Anwer
Background: Relapsed Ewing's sarcoma (RES) is an aggressive malignancy with poor survival. Although high-dose chemotherapy (HDCT) with autologous stem cell transplantation (ASCT) given after conventional chemotherapy (CC) has shown survival benefits, it is not generally used in the United States for RES. We performed a systemic review to evaluate the benefits of HDCT for RES. Methods: Literature search involved Medline, Embase, and Cochrane database. We included studies with RES patients treated with HDCT/ASCT...
2018: Sarcoma
Abdulazeez Salawu, Kristin Wright, Afnan Al-Kathiri, Lynda Wyld, Malcolm Reed, Karen Sisley
Sarcomas are rare heterogeneous malignancies of mesenchymal origin characterised by complex karyotypes but no specific abnormalities. Recurrence is common, and metastatic disease carries poor survival despite standard DNA-damaging radiotherapy or chemotherapy. DNA double-strand breaks (DSBs) are either repaired by mechanisms such as homologous recombination (HR) or result in cell death by apoptosis. Endogenous γ H2AX formation and SCE formation are early and late events, respectively, and their levels are considered surrogate measures of genomic instability...
2018: Sarcoma
Kayleen Bailey, Michael Roth, Daniel Weiser, Jonathan Gill
Purpose: Desmoplastic small round cell tumor (DSRCT) is a rare cancer that predominantly affects males averaging 21 years of age at the time of diagnosis. We describe four cases from our institution and place them within the context of a comprehensive review of the literature. Patients and Methods: Study population included any patient who received treatment at Children's Hospital at Montefiore (CHAM) with histologic diagnosis of DSRCT. A search of the electronic databases PubMed, Cochrane Central Register of Controlled Trials, MEDLINE, and EMBASE for the terms "desmoplastic" AND "small" AND "round" AND "cell" AND "tumor" was performed...
2018: Sarcoma
Anne J Schuster, Leo Kager, Peter Reichardt, Daniel Baumhoer, Monika Csóka, Stefanie Hecker-Nolting, Susanna Lang, Sylvie Lorenzen, Regine Mayer-Steinacker, Thekla von Kalle, Matthias Kevric, Mathias Werner, Reinhard Windhager, Thomas Wirth, Stefan S Bielack
Osteosarcoma of the foot is a very rare presentation of a rare tumor entity. In a retrospective analysis, we investigated tumor- and treatment-related variables and outcome of patients registered in the Cooperative Osteosarcoma Study Group (COSS) database between January 1980 and April 2016 who suffered from primary high-grade osteosarcoma of the foot. Among the 23 eligible patients, median age was 32 years (range: 6-58 years), 10 were female, and 13 were male. The tarsus was the most commonly affected site ( n =16)...
2018: Sarcoma
Shoko Marshall, Kenji Nakano, Yoshiya Sugiura, Shinichiro Taira, Makiko Ono, Junichi Tomomatsu, Shunji Takahashi
Background: Doxorubicin is the key drug for treatment of advanced soft tissue sarcoma (STS). The appropriate dosage of doxorubicin, regarding monotherapy or the role of combination therapy, is unclear. Methods: We retrospectively reviewed patients with advanced or metastatic STS of nonextremities who were treated with doxorubicin-based chemotherapies in our institution. Time to treatment failure (TTF), overall survival (OS), overall response, and prognostic factors for OS were evaluated...
2018: Sarcoma
Sanjay P Bagaria, Yu-Hui Chang, Richard J Gray, Jonathan B Ashman, Steven Attia, Nabil Wasif
Introduction: Optimization of outcomes of extra-abdominal STS is not clearly understood. We sought to determine whether hospital surgical volume and adherence to NCCN guidelines, or both, are associated with outcomes in the treatment of extra-abdominal soft tissue sarcoma (STS). Methods: The National Cancer Database (NCDB) was queried for patients undergoing surgery for extra-abdominal STS diagnosed from 2003 to 2007. Mean annual hospital volume for STS surgery was divided into volume terciles (1T ≤3, 2T 4-10, and 3T ≥11 cases/year)...
2018: Sarcoma
Catherine W Saltus, Brian Calingaert, Sean Candrilli, Maria Lorenzo, Yulia D'yachkova, Thorsten Otto, Uwe Wagner, James A Kaye
We conducted a retrospective cohort study using data compiled from the regional German cancer registries by the Centre for Cancer Registry Data (ZfKD) at the Robert Koch Institut (RKI) to describe the epidemiology of adult soft-tissue sarcomas (STS) in Germany in 2003-2012, focusing on advanced STS. We identified 33,803 incident adult cases of STS (other than the Kaposi sarcoma and gastrointestinal stromal tumors). The incidence of STS was 6.05 (95% confidence interval (CI), 5.82-6.29) per 100,000 in 2012 (4,079 cases)...
2018: Sarcoma
Adam D Lindsay, Edward E Haupt, Chung M Chan, Andre R Spiguel, Mark T Scarborough, Robert A Zlotecki, Parker C Gibbs
Background: The most common site of sarcoma metastasis is the lung. Surgical resection of pulmonary metastases and chemotherapy are treatment options that have been employed, but many patients are poor candidates for these treatments for multiple host or tumor-related reasons. In this group of patients, radiation might provide a less morbid treatment alternative. We sought to evaluate the efficacy of radiotherapy in the treatment of metastatic sarcoma to the lung. Methods: Stereotactic body radiotherapy (SBRT) was used to treat 117 pulmonary metastases in 44 patients...
2018: Sarcoma
Johnathan R Lex, Ahmed Aoude, Jonathan D Stevenson, Jay S Wunder, Scott Evans, Peter C Ferguson, Nikolaos A Stavropoulos, Lee Jeys, Krista Goulding, Robert E Turcotte
Developing multiple soft tissue sarcomas (STSs) is a rare process, sparsely reported in the literature to date. Little is known about the pattern of disease development or outcomes in these patients. Patients were identified from three tertiary orthopaedic oncology centres in Canada and the UK. Patients who developed multiple extremity STSs were collated retrospectively from prospective oncology databases. A literature review using MEDLINE was also performed. Six patients were identified in the case series from these three institutions, and five studies were identified from the literature review...
2018: Sarcoma
Saurabh Bansal, Kunal Das, Navneet Jain, Vipul Nautiyal, Meenu Gupta, Nadia Shirazi, Sanjiv Verma, Mushtaq Ahmad, Sunil Saini
Introduction: The most common site for soft tissue sarcoma is extremity. As complete surgical resection is possible in majority, outcome of this subset is relatively better. There is paucity of data regarding extremity soft tissue sarcoma (STS) from sub-Himalayan and hilly geographical regions. Materials and Methods: Retrospective analysis was done for extremity STS visiting the study center over a period of 5 years. Data were collected and analyzed for demography, disease characteristics, treatment modalities, and outcome...
2018: Sarcoma
Santiago Zuluaga-Sanchez, Lisa M Hess, Sorrel E Wolowacz, Yulia D'yachkova, Emma Hawe, Adrian D Vickers, James A Kaye, David Bertwistle
Background: Standard first-line treatments for advanced soft tissue sarcoma (STS) have changed little for 40 years, and outcomes have been poor. Recently, the United States (US) Food and Drug Administration conditionally approved olaratumab in combination with doxorubicin (Olara + Dox) based on a randomized phase II trial that reported a significant 11.8-month improvement in median survival versus single-agent doxorubicin (Dox). The present study investigated the cost-effectiveness of Olara + Dox compared with Dox and five other standard-of-care regimens from the US payer perspective...
2018: Sarcoma
Sean Ryan, Julia Visgauss, David Kerr, Joshua Helmkamp, Nicholas Said, Emily Vinson, Patrick O'Donnell, Xuechan Li, Sin-Ho Jung, Diana Cardona, William Eward, Brian Brigman
Introduction: Extremity lipomas and well-differentiated liposarcomas (WDLs) are difficult to distinguish on MR imaging. We sought to evaluate the accuracy of MRI interpretation using MDM2 amplification, via fluorescence in-situ hybridization (FISH), as the gold standard for pathologic diagnosis. Furthermore, we aimed to investigate the utility of a diagnostic formula proposed in the literature. Methods: We retrospectively collected 49 patients with lipomas or WDLs utilizing MDM2 for pathologic diagnosis...
2018: Sarcoma
Julie L Koenig, C Jillian Tsai, Katherine Sborov, Kathleen C Horst, Erqi L Pollom
Private insurance is associated with better outcomes in multiple common cancers. We hypothesized that insurance status would significantly impact outcomes in primary breast sarcoma (PBS) due to the additional challenges of diagnosing and coordinating specialized care for a rare cancer. Using the National Cancer Database, we identified adult females diagnosed with PBS between 2004 and 2013. The influence of insurance status on overall survival (OS) was evaluated using the Kaplan-Meier estimator with log-rank tests and Cox proportional hazard models...
2018: Sarcoma
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