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Sarcoma

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https://www.readbyqxmd.com/read/28592921/malignant-peripheral-nerve-sheath-tumors-state-of-the-science-leveraging-clinical-and-biological-insights-into-effective-therapies
#1
REVIEW
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
https://www.readbyqxmd.com/read/28588396/results-of-a-qualitative-study-to-develop-a-patient-reported-outcome-measure-for-patients-with-4-subtypes-of-soft-tissue-sarcoma
#2
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
https://www.readbyqxmd.com/read/28546782/neoadjuvant-ifosfamide-and-epirubicin-in-the-treatment-of-malignant-peripheral-nerve-sheath-tumors
#3
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
https://www.readbyqxmd.com/read/28512389/evaluation-of-quality-of-life-at-progression-in-patients-with-soft-tissue-sarcoma
#4
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
https://www.readbyqxmd.com/read/28348507/variations-of-surveillance-practice-for-patients-with-bone-sarcoma-a-survey-of-australian-sarcoma-clinicians
#5
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
https://www.readbyqxmd.com/read/28246524/correlation-of-ezrin-expression-pattern-and-clinical-outcomes-in-ewing-sarcoma
#6
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
https://www.readbyqxmd.com/read/28115911/routes-to-diagnosis-for-suspected-sarcoma-the-impact-of-symptoms-and-clinical-findings-on-the-diagnostic-process
#7
Heidi Buvarp Dyrop, Peter Vedsted, Mathias Rædkjær, Akmal Safwat, Johnny Keller
Background and Objectives. Sarcoma patients often experience delay before diagnosis. We examined the association between presenting symptoms/signs and time intervals for suspected sarcoma patients. Methods. 545 consecutive patients suspected for sarcoma referred over a one-year period were included. Median time intervals in routes to diagnosis were collected from medical records and questionnaires. Results. 102 patients (18.7%) had a sarcoma; 68 (12.5%) had other malignancies. Median interval for the patient (time from first symptom to first doctor visit), primary care, local hospital, sarcoma center, diagnostic, and total interval for sarcoma patients were 77, 17, 29, 17, 65, and 176 days, respectively...
2016: Sarcoma
https://www.readbyqxmd.com/read/28115910/cryosurgery-as-additional-treatment-in-tenosynovial-giant-cell-tumors
#8
F G M Verspoor, A Scholte, I C M van der Geest, G Hannink, H W B Schreuder
Introduction. Tenosynovial giant cell tumors (TGCT) emerge from the synovium and can behave aggressively. Surgical resection is the standard treatment. However, up to half of the patients with diffuse type show recurrences. Several additional treatments have been applied to reduce recurrences; none of these treatments was proven to be superior to surgical resection solely. This article describes the results of additional cryosurgery to surgical resection. Materials and Methods. We retrospectively evaluated 141 TGCT patients, between 1999 and 2007...
2016: Sarcoma
https://www.readbyqxmd.com/read/27999474/giant-cell-tumor-a-rare-condition-in-the-immature-skeleton-a-retrospective-study-of-symptoms-treatment-and-outcome-in-16-children
#9
Thale M Asp Strøm, Anette Torød Skeie, Ingvild Koren Lobmaier, Olga Zaikova
Background. Pediatric giant cell tumor (GCT) of bone is rare and the course of the disease in the immature skeleton is sparsely described. We performed a retrospective study addressing symptoms, treatment, and outcome in children with GCT. Methods. Review of medical records and images of patients with GCT. Patients were detected from our hospital prospective database and those with open epiphyseal cartilages were included. Results. 16 children (75% girls) from 6 to 15 years old were identified. Eight lesions (50%) were in long bones and 4 (25%) in flat bones...
2016: Sarcoma
https://www.readbyqxmd.com/read/27990096/mir-125b-and-mir-100-are-predictive-biomarkers-of-response-to-induction-chemotherapy-in-osteosarcoma
#10
Daisuke Kubota, Nobuyoshi Kosaka, Tomohiro Fujiwara, Akihiko Yoshida, Yasuhito Arai, Zhiwei Qiao, Fumitaka Takeshita, Takahiro Ochiya, Akira Kawai, Tadashi Kondo
Osteosarcoma is the most common primary malignancy in bone. Patients who respond poorly to induction chemotherapy are at higher risk of adverse prognosis. The molecular basis for such poor prognosis remains unclear. We investigated miRNA expression in eight open biopsy samples to identify miRNAs predictive of response to induction chemotherapy and thus maybe used for risk stratification therapy. The samples were obtained from four patients with inferior necrosis (Huvos I/II) and four patients with superior necrosis (Huvos III/IV) following induction chemotherapy...
2016: Sarcoma
https://www.readbyqxmd.com/read/27895527/phase-ii-trial-of-angiotensin-1-7-for-the-treatment-of-patients-with-metastatic-sarcoma
#11
Paul D Savage, James Lovato, K Bridget Brosnihan, Antonius A Miller, W Jeffrey Petty
Background. Angiotensin-(1-7) [Ang-(1-7)] is an endogenous antiangiogenic hormone with anticancer activity. In a phase I study of Ang-(1-7), two of three patients with metastatic sarcoma experienced disease stabilization. This phase II study examined clinical and biomarker outcomes for patients with metastatic sarcoma. Methods. Ang-(1-7) was administered by subcutaneous injection at a dose of 20 mg daily. If excessive toxicities occurred in the first cohort, a dose deescalation cohort was allowed. Blood samples were obtained to measure changes in biomarkers...
2016: Sarcoma
https://www.readbyqxmd.com/read/27843394/trabectedin-followed-by-irinotecan-can-stabilize-disease-in-advanced-translocation-positive-sarcomas-with-acceptable-toxicity
#12
J Herzog, F von Klot-Heydenfeldt, S Jabar, A Ranft, C Rossig, U Dirksen, J Van den Brande, M D'Incalci, I von Luettichau, P J Grohar, W E Berdel, St Burdach
Background. Preclinical data indicate that trabectedin followed by irinotecan has strong synergistic effects on Ewing sarcoma. This is presumably due to hypersensitization of the tumor cells to the camptothecin as an effect of trabectedin in addition to synergistic suppression of EWS-FLI1 downstream targets. A strong effect was also reported in a human rhabdomyosarcoma xenograft. Procedure. Twelve patients with end-stage refractory translocation-positive sarcomas were treated with trabectedin followed by irinotecan within a compassionate use program...
2016: Sarcoma
https://www.readbyqxmd.com/read/27822137/axitinib-has-antiangiogenic-and-antitumorigenic-activity-in-myxoid-liposarcoma
#13
Lauren T Kerr, Jacqueline F Donoghue, Alexander L Wilding, Terrance G Johns
Myxoid liposarcoma is a rare form of soft-tissue sarcoma. Although most patients initially respond well to treatment, approximately 21% relapse, highlighting the need for alternative treatments. To identify novel treatment regimens and gain a better understanding of myxoid liposarcoma tumor biology, we screened various candidate and approved targeted therapeutics and chemotherapeutics against myxoid liposarcoma cell lines. Therapeutics that target angiogenesis showed antitumor activity. The small molecule inhibitor axitinib, which targets angiogenesis by inhibiting the VEGFR and PDGFR families and c-Kit, inhibited cell cycle progression and induced apoptosis in vitro, as well as having significant antitumor activity against MLS 1765 myxoid liposarcoma xenografts in mice...
2016: Sarcoma
https://www.readbyqxmd.com/read/27725792/the-discrepancy-between-patient-and-clinician-reported-function-in-extremity-bone-metastases
#14
Stein J Janssen, Eva A J van Rein, Nuno Rui Paulino Pereira, Kevin A Raskin, Marco L Ferrone, Francis J Hornicek, Santiago A Lozano-Calderon, Joseph H Schwab
Background. The Musculoskeletal Tumor Society (MSTS) scoring system measures function and is commonly used but criticized because it was developed to be completed by the clinician and not by the patient. We therefore evaluated if there is a difference between patient and clinician reported function using the MSTS score. Methods. 128 patients with bone metastasis of the lower (n = 100) and upper (n = 28) extremity completed the MSTS score. The MSTS score consists of six domains, scored on a 0 to 5 scale and transformed into an overall score ranging from 0 to 100% with a higher score indicating better function...
2016: Sarcoma
https://www.readbyqxmd.com/read/27721667/molecular-targets-and-emerging-therapeutic-options-for-uterine-leiomyosarcoma
#15
REVIEW
Heather Miller, Chiemeka Ike, Jennifer Parma, Ramya P Masand, Claire M Mach, Matthew L Anderson
Uterine leiomyosarcoma (uLMS) is an aggressive malignancy characterized by its early metastasis, high rates of recurrence, and poor prognosis. Multiple obstacles complicate the clinical management of uLMS. These include the fact that most uLMS are typically identified only after a woman has undergone hysterectomy or myomectomy, the limited efficacy of adjuvant therapy for early stage disease, and the poor response of metastatic disease to current treatments. Here, we discuss recent insights into the molecular basis of uLMS and discuss emerging options for its clinical management...
2016: Sarcoma
https://www.readbyqxmd.com/read/27703409/current-immunotherapies-for-sarcoma-clinical-trials-and-rationale
#16
REVIEW
Demytra Mitsis, Valerie Francescutti, Joseph Skitzki
Sarcoma tumors are rare and heterogeneous, yet they possess many characteristics that may facilitate immunotherapeutic responses. Both active strategies including vaccines and passive strategies involving cellular adoptive immunotherapy have been applied clinically. Results of these clinical trials indicate a distinct benefit for select patients. The recent breakthrough of immunologic checkpoint inhibition is being rapidly introduced to a variety of tumor types including sarcoma. It is anticipated that these emerging immunotherapies will exhibit clinical efficacy for a variety of sarcomas...
2016: Sarcoma
https://www.readbyqxmd.com/read/27651732/hiv-1-evolutionary-patterns-associated-with-metastatic-kaposi-s-sarcoma-during-aids
#17
Susanna L Lamers, Rebecca Rose, David J Nolan, Gary B Fogel, Andrew E Barbier, Marco Salemi, Michael S McGrath
Kaposi's sarcoma (KS) in HIV-infected individuals can have a wide range of clinical outcomes, from indolent skin tumors to a life-threatening visceral cancer. KS tumors contain endothelial-related cells and inflammatory cells that may be HIV-infected. In this study we tested if HIV evolutionary patterns distinguish KS tumor relatedness and progression. Multisite autopsies from participants who died from HIV-AIDS with KS prior to the availability of antiretroviral therapy were identified at the AIDS and Cancer Specimen Resource (ACSR)...
2016: Sarcoma
https://www.readbyqxmd.com/read/27524931/ewing-s-sarcoma-as-a-second-malignancy-in-long-term-survivors-of-childhood-hematologic-malignancies
#18
Fabian Wolpert, Michael A Grotzer, Felix Niggli, Dieter Zimmermann, Elisabeth Rushing, Beata Bode-Lesniewska
Modern multimodal treatment has significantly increased survival for patients affected by hematologic malignancies, especially in childhood. Following remission, however, the risk of developing a further malignancy is an important issue. The long-term estimated risk of developing a sarcoma as a secondary malignancy is increased severalfold in comparison to the general population. Ewing's sarcoma family encompasses a group of highly aggressive, undifferentiated, intra- and extraosseous, mesenchymal tumors, caused by several types of translocations usually involving the EWSR1 gene...
2016: Sarcoma
https://www.readbyqxmd.com/read/27516726/a-systematic-literature-review-of-adverse-events-associated-with-systemic-treatments-used-in-advanced-soft-tissue-sarcoma
#19
REVIEW
Ann Colosia, Shahnaz Khan, Michelle D Hackshaw, Alan Oglesby, James A Kaye, Jeffrey M Skolnik
This systematic literature review describes adverse events (AEs) among patients with soft tissue sarcoma (STS) who received second-line or later anticancer therapies. Searches were conducted in PubMed, EMBASE, and Cochrane Central Register of Controlled Trials for studies of adults with advanced or metastatic STS who received systemic anticancer therapy before enrollment in a randomized-controlled trial of pazopanib, another targeted cancer agent, or cytotoxic chemotherapy. Of 204 publications identified, seven articles representing six unique studies met inclusion criteria...
2016: Sarcoma
https://www.readbyqxmd.com/read/27478404/surveillance-strategies-for-sarcoma-results-of-a-survey-of-members-of-the-musculoskeletal-tumor-society
#20
David D Greenberg, Brooke Crawford
Background. Surveillance is crucial to oncology, yet there is scant evidence to guide strategies. Purpose. This survey identified sarcoma surveillance strategies for Musculoskeletal Tumor Society (MSTS) members and rationales behind them. Understanding current practice should facilitate studies to generate evidence-based surveillance protocols. Methods. Permission was granted by the Research and Executive Committee of the MSTS to survey members on surveillance strategies. First, the questionnaire requested demographic and clinical practice information...
2016: Sarcoma
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