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Annelies Requilé, Paul M Clement, Oliver E Bechter, Herlinde Dumez, Annelies Verbiest, Raf Sciot, Daphne Hompes, Friedl Sinnaeve, Erik Van Limbergen, Patrick Schöffski
The treatment of Ewing sarcoma (ES) in adult patients requires a multidisciplinary approach. Systemic therapy remains an important component of clinical management of this disease. ES is extremely rare in adult patients. Due to the rarity of the disease, no standard of care in terms of chemotherapy for the adult population exists, and the level of evidence for individual agents or some multidrug combinations is limited. Most regimens that are used in both adults and children include anthracyclines, etoposide, vincristine, cyclophosphamide, and ifosfamide...
2017: Sarcoma
Michael J Nathenson, Anthony P Conley, Heather Lin, Nicole Fleming, Vinod Ravi
Purpose: This study retrospectively evaluated overall survival (OS) by treatment of recurrent or metastatic uterine adenosarcoma including surgery, radiation, chemotherapy, and hormonal therapy and evaluated OS and progression-free survival (PFS) after 1st line systemic chemotherapy. Methods: 78 patients with recurrent or metastatic adenosarcoma comprised the study population. The Kaplan-Meier method was used to estimate OS and PFS. The log-rank test was performed to test the difference in survival between groups...
2017: Sarcoma
Konstantin A Kovtun, Wee-Pin Yeo, Catherine H Phillips, Akila Viswanathan, Elizabeth H Baldini
Background and Objectives: Attention to ovary dose is important for premenopausal women undergoing radiation therapy (RT) and must not be overlooked when treating extremity sarcoma. We assessed whether ovary-sparing RT plans could decrease ovary dose without compromising target coverage. Methods: Standard sarcoma target volumes and organs at risk (OAR) were contoured by a sarcoma dedicated radiation oncologist on CT planning scans for 23 women with thigh or buttock sarcoma...
2017: Sarcoma
Ajaz Bulbul, Bridget Noel Fahy, Joanne Xiu, Sadaf Rashad, Asrar Mustafa, Hatim Husain, Andrea Hayes-Jordan
Desmoplastic small round blue cell tumors (DSRCTs) originate from a cell with multilineage potential. A molecular hallmark of DSRCT is the EWS-WT1 reciprocal translocation. Ewing sarcoma and DSRCT are treated similarly due to similar oncogene activation pathways, and DSRCT has been represented in very limited numbers in sarcoma studies. Despite aggressive therapy, median survival ranges from 17 to 25 months, and 5-year survival rates remain around 15%, with higher survival reported among those undergoing removal of at least 90% of tumor in the absence of extraperitoneal metastasis...
2017: Sarcoma
Christine S Higham, Seth M Steinberg, Eva Dombi, Arie Perry, Lee J Helman, Scott M Schuetze, Joseph A Ludwig, Arthur Staddon, Mohammed M Milhem, Daniel Rushing, Robin L Jones, Michael Livingston, Stewart Goldman, Christopher Moertel, Lars Wagner, David Janhofer, Christina M Annunziata, Denise Reinke, Lauren Long, David Viskochil, Larry Baker, Brigitte C Widemann
Background: Worse chemotherapy response for neurofibromatosis type 1- (NF1-) associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST) has been reported. Methods: We evaluated the objective response (OR) rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST versus sporadic MPNST after 4 cycles of neoadjuvant chemotherapy, 2 cycles of ifosfamide/doxorubicin, and 2 cycles of ifosfamide/etoposide. A Simon optimal two-stage design was used (target response rate 40%)...
2017: Sarcoma
Raymundo A Quintana, Jose Banchs, Ridhi Gupta, Heather Y Lin, Sean D Raj, Anthony Conley, Vinod Ravi, Dejka Araujo, Robert S Benjamin, Shreyaskumar Patel, Saroj Vadhan-Raj, Neeta Somaiah
BACKGROUND: Despite the dose-dependent response rate of sarcomas to doxorubicin, clinicians limit its cumulative dose due to cardiotoxicity. This study evaluates early evidence of cardiotoxicity in patients treated with high-dose doxorubicin given as a continuous infusion. METHODS: Data was collected on patients who received 90 mg/m(2) doxorubicin as a continuous infusion and 10 gm/m(2) ifosfamide for up to 6 cycles as part of a phase II study. Cardiotoxicity was assessed with serial echocardiograms or multigated acquisition scans and serum brain natriuretic peptide and troponin levels...
2017: Sarcoma
Vittoria Colia, Elena Fumagalli, Salvatore Provenzano, Rossella Bertulli, Silvia Stacchiotti, Carlo Morosi, Paola Collini, Alessandro Gronchi, Paolo G Casali, Roberta Sanfilippo
BACKGROUND: To report on the activity of high-dose prolonged-infusion ifosfamide (HDIFX) chemotherapy in a retrospective series of patients affected by myxoid liposarcoma treated at Fondazione IRCCS Istituto Nazionale dei Tumori in Milan, Italy. PATIENTS AND METHODS: Patients with an advanced myxoid liposarcoma treated with HDIFX (14 g/sqm, i.v., prolonged infusion of 14 days every 28 days) as a single agent between May 2002 and April 2017 were retrospectively reviewed...
2017: Sarcoma
S Secondino, V Grazioli, F Valentino, M Pin, A Pagani, A Sciortino, C Klersy, M G Callegari, P Morbini, R Dore, M Paulli, P Pedrazzoli, A M D'armini
INTRODUCTION: Pulmonary artery sarcoma (PAS) is a rare tumor, whose therapeutic approach is mainly based on surgery, either pneumonectomy or pulmonary endarterectomy (PEA). The prognosis reported in published series is very poor, with survival of 1.5 months without any kind of treatment. PATIENTS AND METHODS: From January 2010 to January 2016, 1027 patients were referred to our hospital for symptoms of acute or chronic pulmonary thromboembolic disease. Twelve patients having a confirmed diagnosis of PAS underwent PEA...
2017: Sarcoma
Julie J Willeumier, C W P G van der Wal, Robert J P van der Wal, P D S Dijkstra, Thea P M Vliet Vlieland, Michiel A J van de Sande
PURPOSE: The aim of this study was to translate and culturally adapt the Toronto Extremity Salvage Score (TESS) to Dutch and to validate the translated version. METHODS: The TESS lower and upper extremity versions (LE and UE) were translated to Dutch according to international guidelines. The translated version was validated in 98 patients with surgically treated bone or soft tissue tumors of the LE or UE. To assess test-retest reliability, participants were asked to fill in a second questionnaire after one week...
2017: Sarcoma
Jong Woong Park, Han-Soo Kim, Cheol Lee, Hye Jin Yoo, Ji Yeon Yun, Ilkyu Han
Soft tissue sarcoma (STS) with an infiltrative histologic growth pattern, when compared to STS with an expansile pattern, may pose difficulties in local control. Preoperative assessment of the presence of infiltrative histologic growth pattern would be helpful in deciding treatment strategies. A review of 144 patients who underwent surgery for extremity STS was performed. Microscopically, the histologic growth pattern was defined as infiltrative if the penetration of the tumor cells into the surrounding tissue was observed...
2017: Sarcoma
Everett J Moding, Lynn Million, Raffi Avedian, Pejman Ghanouni, Christian Kunder, Kristen N Ganjoo
Desmoid tumors are locally aggressive fibroproliferative neoplasms that can lead to pain and dysfunction due to compression of nerves and surrounding structures. Desmoid tumors often progress through medical therapy, and there is frequently a delay of multiple months before radiation can provide symptomatic relief. To achieve more rapid symptomatic relief and tumor regression for unresectable desmoid tumors causing significant morbidity such as brachial plexus impingement with loss of extremity function, we have selectively utilized a combination of imatinib and radiation therapy...
2017: Sarcoma
Drake G LeBrun, David M Guttmann, Jacob E Shabason, William P Levin, Stephen J Kovach, Kristy L Weber
Wound complications represent a major source of morbidity in patients undergoing radiation therapy (RT) and surgical resection of soft tissue sarcomas (STS). We investigated whether factors related to RT, surgery, patient comorbidities, and tumor histopathology predict the development of wound complications. An observational study of patients who underwent STS resection and RT was performed. The primary outcome was the occurrence of any wound complication up to four months postoperatively. Significant predictors of wound complications were identified using multivariable logistic regression...
2017: Sarcoma
Seung Yong Lee, Dae-Geun Jeon, Wan Hyeong Cho, Won Seok Song, Chang-Bae Kong, Bum Suk Kim
BACKGROUND: Among various types of composite biological reconstruction, pasteurized autograft-prosthesis composite (PPC) is popular when allograft is unavailable. Previous limited cohort study indicated result comparable to tumor prosthesis. However, as case number and follow-up increase, we experienced more complications than anticipated. We questioned the usefulness of PPC as a viable reconstructive option. METHODS: We reviewed 142 PPCs and analyzed overall and location-related survival and factors associated with the failure of PPC...
2017: Sarcoma
Vancheswaran Gopalakrishnan, Behrang Amini, Michael J Wagner, Erica N Nowell, Alexander J Lazar, Patrick P Lin, Robert S Benjamin, Dejka M Araujo
BACKGROUND: The prognosis and clinical characteristics of head and neck synovial sarcomas (HNSS) are unclear. Herein, we present an update using a cohort of patients treated at our institution. METHODS: We performed a retrospective chart review of 44 patients diagnosed with primary HNSS between March 1990 and June 2012. Overall survival (OS) and progression-free survival (PFS) curves were estimated and hazard ratios (HRs) were calculated. RESULTS: The entire cohort's median PFS was 4...
2017: Sarcoma
AeRang Kim, Douglas R Stewart, Karlyne M Reilly, David Viskochil, Markku M Miettinen, Brigitte C Widemann
Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality in patients with neurofibromatosis type 1. In 2002, an MPNST consensus statement reviewed the current knowledge and provided guidance for the diagnosis and management of MPNST. Although the improvement in clinical outcome has not changed, substantial progress has been made in understanding the natural history and biology of MPNST through imaging and genomic advances since 2002. Genetically engineered mouse models that develop MPNST spontaneously have greatly facilitated preclinical evaluation of novel drugs for translation into clinical trials led by consortia efforts...
2017: Sarcoma
Anne M Skalicky, Sameer R Ghate, Jose Ricardo Perez, Anne M Rentz
OBJECTIVE: The objective of this research was to develop a disease-specific symptom inventory for soft tissue sarcoma. METHODS: Literature review and clinical expert and patient interviews were conducted to determine disease-specific symptoms important to patients with one of the four STS subtypes. Clinical experts identified the most relevant STS symptom items from the item pool developed from literature review. Concept elicitation interviews were conducted with patients to elicit their STS symptom experiences followed by a completion of the draft symptom list via web survey...
2017: Sarcoma
Angela C Hirbe, Pippa F Cosper, Sonika Dahiya, Brian A Van Tine
Background and Objectives. Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with poor overall survival. Response to chemotherapy has been debated for these tumors. Methods. We performed a retrospective analysis of the patients at our institution with a biopsy-proven diagnosis of MPNST that underwent neoadjuvant chemotherapy prior to surgery. Results. We retrospectively identified five patients who received neoadjuvant chemotherapy with epirubicin and ifosfamide that demonstrated a 30% reduction in tumor growth and a 60% response rate by RECIST criteria...
2017: Sarcoma
Stacie Hudgens, Anna Forsythe, Ilias Kontoudis, David D'Adamo, Ashley Bird, Hans Gelderblom
Introduction. Soft Tissue Sarcoma (STS) is a rare malignancy of mesodermal tissue, with international incidence estimates between 1.8 and 5 per 100,000 per year. Understanding quality of life (QoL) and the detrimental impact of disease progression is critical for long-term care and survival. Objectives. The primary objective was to explore the relationship between disease progression and health-related quality of life (HRQoL) using data from Eisai's study (E7389-G000-309). Methods. This was a 1 : 1 randomized, open-label, multicenter, Phase 3 study comparing the efficacy and safety of eribulin versus dacarbazine in patients with advanced STS...
2017: Sarcoma
Jeremy Lewin, Kate Thompson, Susie Bae, Jayesh Desai, Robyn Strong, Denise Caruso, Deborah Howell, Alan Herschtal, Michael Sullivan, Lisa Orme
Introduction. After treatment, bone sarcoma patients carry a high chance of relapse and late effects from multimodal therapy. We hypothesize that significant variation in surveillance practice exists between pediatric medical oncology (PO) and nonpediatric medical oncology (NP) sarcoma disciplines. Methods. Australian sarcoma clinicians were approached to do a web based survey that assessed radiologic surveillance (RS) strategies, late toxicity assessment, and posttreatment psychosocial interventions. Results...
2017: Sarcoma
Thomas Cash, Hong Yin, Courtney McCracken, Zhi Geng, Steven G DuBois, Bahig M Shehata, Thomas A Olson, Howard M Katzenstein, Cynthia Wetmore
Background. Ezrin is a membrane-cytoskeleton linker protein that has been associated with metastasis and poor outcomes in osteosarcoma and high-grade soft tissue sarcomas. The prognostic value of ezrin expression in Ewing sarcoma is unknown. Methods. The relationship between ezrin expression and outcome was analyzed in a cohort of 53 newly diagnosed Ewing sarcoma patients treated between 2000 and 2011. The intensity and proportion of cells with ezrin immunoreactivity were assessed in diagnostic tumor tissue using a semiquantitative scoring system to yield intensity and positivity scores for each tumor...
2017: Sarcoma
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