Sarcoma (Sarcoma)

Publisher: Hindawi Publishing Corporation

Journal description

Sarcoma is dedicated to publishing papers covering all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology and the clinical sciences of epidemiology, surgery, radiotherapy and chemotherapy. High-quality papers concerning the entire range of bone and soft tissue sarcomas in both adults and children, including Kaposi's sarcoma, are published as well as preclinical and animal studies. This journal provides a central forum for the description of advances in diagnosis, assessment and treatment of this rarely seen, but often mismanaged, group of patients. It is of interest to all those working with bone and soft tissue tumours, including medical, surgical and paediatric oncologists, radiotherapists, pathologists and research scientists.

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Website Sarcoma website
Other titles Sarcoma (Online), Sarcoma
ISSN 1357-714X
OCLC 37915580
Material type Document, Periodical, Internet resource
Document type Internet Resource, Computer File, Journal / Magazine / Newspaper

Publisher details

Hindawi Publishing Corporation

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    • Publisher's version/PDF may be used
    • Creative Commons Attribution License
    • Eligible UK authors may deposit in OpenDepot
    • All titles are open access journals
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Publications in this journal

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    ABSTRACT: Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach.
    Sarcoma 11/2015; 2015(16):1-14. DOI:10.1155/2015/243298
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    ABSTRACT: Purpose . To describe epidemiological, clinical characteristics and treatment outcomes of low-grade osteosarcoma (LGOS), including dedifferentiated osteosarcoma (DLGOS). Method . We analysed a nationwide cohort comprised of patients with histologically verified LGOS and DLGOS between 1975 and 2009, based on registry sources supplemented with clinical records from hospitals involved in sarcoma management. Results . Fifty-four patients were identified, 12 of whom had DLGOS. The annual incidence for all patients was 0.3 per million, with the peak incidence in the third decade of the life. Fifteen patients experienced local relapses during follow-up and ten developed metastatic diseases, including three at primary diagnosis. Patients with DLGOS dominated the metastatic relapse group. The five-year sarcoma-specific survival rate was 91%, with no documented improvement over time. Free margin following surgical resection of the primary tumour had a positive impact on survival. As expected, both local relapse and metastasis during follow-up were associated with an unfavourable outcome. Radiotherapy predicted poor survival due to the selection of high-risk patients in need of such treatment. Neither higher age nor axial tumour localisation was adverse prognostic factors. Conclusion . LGOS has an excellent prognosis when surgically resected with a free margin; however, LGOS has the potential to dedifferentiate and metastasize with a poor outcome.
    Sarcoma 09/2015; 2015(7):917679. DOI:10.1155/2015/917679
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    ABSTRACT: Background. Ewing sarcoma family of tumors (ESFT) are rare but deadly cancers of unknown etiology. Few risk factors have been identified. This study was undertaken to ascertain any possible association between exposure to therapeutic drugs and ESFT. Methods. This is a retrospective, descriptive study. A query of the FDA Adverse Event Reporting System (FAERS) was conducted for all reports of ESFT, January 1, 1998, through December 31, 2013. Report narratives were individually reviewed for patient characteristics, underlying conditions and drug exposures. Results. Over 16 years, 134 ESFT reports were identified, including 25 cases of ESFT following therapeutic drugs and biologics including immunosuppressive agents and hormones. Many cases were confounded by concomitant medications and other therapies. Conclusions. This study provides a closer look at medication use and underlying disorders in patients who later developed ESFT. While this study was not designed to demonstrate any clear causative association between ESFT and prior use of a single product or drug class, many drugs were used to treat immune-related disease and growth or hormonal disturbances. Further studies may be warranted to better understand possible immune or neuroendocrine abnormalities or exposure to specific classes of drugs that may predispose to the later development of ESFT.1. Introduction and Study Objective Ewing sarcoma family of tumors (ESFT) is a group of small, round blue cell tumors that arise in the bone or soft tissue. ESFT include classic Ewing sarcoma (ES) of bone, extraskeletal ES, skin tumors of the chest wall, and primitive neuroectodermal tumors (PNET) of the bone or soft tissue. About 90% of ESFT cases are characterized by the chromosome translocation t(11;22)(q24;q12) which involves the EWS/FLI-1 fusion gene and may share a common neural histogenesis [1–4]. While ESFT is rare, it is the second most common primary malignant bone tumor occurring in children and young adults and accounts for 10–15% of all primary bone tumors and 3% of all pediatric malignancies. ESFT occurs with a male predominance and highest incidence rates among whites with considerably lower rates in Blacks and East Asian populations. Age of onset is normally in the second decade of life, with 80% occurring in the first 2 decades of life and 80% occur in the skeleton [2–5]. Each year up to 400 patients in the US will be diagnosed with ESFT. The incidence has been steady over the past few decades [6].While few risk factors for ESFT have been identified, the racial difference and specific chromosomal translocation might suggest a genetic predisposition [1, 7]. However, unlike other bone cancers (e.g., osteosarcoma) and soft tissue sarcomas (e.g., rhabdomyosarcoma) ESFT has not been found to be associated with any genetic disease or hereditary cancer syndrome [2, 7, 8]. Epidemiological studies have examined a wide array of possible risk factors, including childhood conditions and various parental exposures [9–16]. There is limited data about drug exposures as possible risk factors for ESFT. One study reported a possible association with poison or overdose of medications [11]. Another study by Valery et al. 2003 [16] found medication use to be more prevalent in controls than cases. While few if any studies have found an association with medications and ESFT (with exposure to medications), two studies [10, 16] found an inverse association with asthma, but this has not been confirmed by the other studies [9, 11, 12, 16]. Despite continuing efforts to identify risk factors for ESFT, its etiology is still unknown.The aim of this study was to review all cases of ESFT that have been reported to FDA from January 1, 1998, through December 31, 2013, and to assess any possible association with therapeutic products. (FAERS includes therapeutic agents which include drugs and also biologics.) We also conducted an extensive literature search of articles that pertained to the epidemiology of ESFT.2. Methods All case reports for drug products that included ESFT from the FDA Adverse Event Reporting System (FAERS) database were analyzed. The FAERS database, including foreign and domestic reports and all age groups, was searched for the time period, January 1, 1998, through December 31, 2013. The following Medical Dictionary for Regulatory Activities (MedDRA) search terms (MedDRA website, see [17]) were used to identify any reports of cases with ESFT: Ewing’s sarcoma, Ewing’s sarcoma metastatic, Ewing’s sarcoma recurrent, extraosseous Ewing’s sarcoma, extraosseous Ewing’s sarcoma metastatic, extraosseous Ewing’s sarcoma nonmetastatic, extraosseous Ewing’s sarcoma recurrent, neuroectodermal neoplasm, primitive neuroectodermal tumour, primitive neuroectodermal tumour metastatic, and peripheral primitive neuroectodermal tumour of soft tissue. Cases were stratified by year of occurrence and defined by the reporter as definite, probable, possible, and unlikely in terms of the diagnosis of ES and potential association with drug exposure. Reports of ESFT as a secondary malignancy, as a primary tumor preceding a secondary malignancy, adverse events related to chemotherapy, and other treatments for ESFT and report duplications were excluded. Case reports of medication use pre-ESFT were analyzed by two independent reviewers.Data Source. This study utilized the FAERS database of adverse event (AE) reports. Since 1969, FDA has maintained this passive surveillance system to detect problems with drugs and biological products used by humans. Manufacturers are required by US law to report AEs associated with their products, and AEs may also be spontaneously reported by health care professionals and consumers. The database contains reports from the US and non-US countries. Reports that the reporter thought to be related to the use of a product are reviewed, coded, and accumulated into an electronic database. The adverse events are codified by MedDRA terminology. This passive surveillance program functions as an early warning system for the detection of serious AEs not identified during the premarket testing or clinical trials. A special feature of this program includes follow-up reports of cases following FDA’s request for further information. Some of the reports in this database may also be found in the published literature.3. Results3.1. Overall FAERS Reports SummaryOver the 16-year time period, 134 reports were retrieved with mention of ESFT. After clinical review of the narrative reports 25 cases were identified with a history of medication use prior to ESFT diagnosis, most with a history of ≥18 months. Age range was 5–68 years of age (median 24 years) (Table 1). Many of our cases were outside the adolescent age group.
    Sarcoma 08/2015; 2015(15 Supplement):948159. DOI:10.1155/2015/948159
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    ABSTRACT: Gemcitabine (G) and docetaxel (D) are commonly used to treat recurrent/metastatic soft tissue sarcoma. This study tested the hypothesis that outcomes would be improved by addition of bevacizumab (B). The initial design was randomized double-blind trial of G + D + B versus G + D + placebo. Due to slow accrual this was modified to single-arm open-label G + D + B. Eligible patients had diagnosis of leiomyosarcoma, pleomorphic undifferentiated sarcoma, pleomorphic liposarcoma, or angiosarcoma. Treatment was B 15 mg/kg on d1, G 900 mg/m2 on d1 and d8, and D 75 mg/m2 on d8, q21d. Primary endpoint was progression-free survival (PFS) at 6 months and would be met if ≥17 patients were progression-free at 6 m. Secondary endpoints are response rate, PFS at 3 m, overall survival, and toxicity. Of 44 patients enrolled, 35 were treated with GDB and evaluable for safety and efficacy. Median age was 55, 50% male, most ECOG 0. Toxicity is mostly myelosuppression with one deep vein thrombosis and one small bowel perforation possibly related to B. There were 17 partial responses (49%) by RECIST 1.1. Among 35 patients, the number who remained on study and progression-free was 24 at 3 m and 15 at 6 m. 9 withdrew prior to 6 m for reasons other than toxicity or progression. PFS at 6 m was 65% (95% CI: 51–85%). The primary endpoint of 6 m PFS was not met due to censoring of patients who withdrew. However PFS at 3 m (76%) was promising and response rate was higher than expected from G + D.
    Sarcoma 05/2015; 2015:1-7. DOI:10.1155/2015/532478
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    ABSTRACT: Desmoplastic small round cell tumor (DSRCT), a rare, aggressive neoplasm, has a poor prognosis. In this prospective study, we evaluated the role of myeloablative chemotherapy, followed by autologous stem cell transplant in improving survival in DSRCT. After high-dose induction chemotherapy and surgery, 19 patients with chemoresponsive DSRCT underwent autologous stem cell transplant. Myeloablative chemotherapy consisted of carboplatin (400-700 mg/m2/day for 3 days) + thiotepa (300 mg/m2/day for 3 days) ± topotecan (2 mg/m2/day for 5 days). All patients were engrafted and there was no treatment-related mortality. Seventeen patients received radiotherapy to sites of prior or residual disease at a median of 12 weeks after transplant. Five-year event-free and overall survival were 11 ± 7% and 16 ± 8%, respectively. Two patients survive disease-free 16 and 19 years after transplant (both in complete remission before transplant). 14 patients had progression and died of disease at a median of 18 months following autologous transplant. These data do not justify the use of myeloablative chemotherapy with carboplatin plus thiotepa in patients with DSRCT. Alternative therapies should be considered for this aggressive neoplasm.
    Sarcoma 05/2015; 2015:1-9. DOI:10.1155/2015/269197
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    ABSTRACT: A patient presented with a recurrent sarcoma (diagnosed as leiomyosarcoma) 12 years after the removal of an initial cancer (diagnosed as extracompartmental osteosarcoma) distally on the same limb. Following surgery, the sarcoma and unaffected muscle and bone were subjected to measurements of DNA exome sequence, RNA and protein expression, and transcription factor binding. The investigation provided corroboration of the diagnosis leiomyosarcoma, as the major upregulations in this tumor comprise muscle-specific gene products and calcium-regulating molecules (calcium is an important second messenger in smooth muscle cells). A likely culprit for the disease is the point mutation S181G in FAF1, which may cause a loss of apoptotic function consecutive to transforming DNA damage. The RNA levels of genes for drug transport and metabolism were extensively skewed in the tumor tissue as compared to muscle and bone. The results suggest that the tumor represents a recurrence of a dormant metastasis from an originally misdiagnosed neoplasm. A loss of FAF1 function could cause constitutive WNT pathway activity (consistent with the downstream inductions of IGF2BP1 and E2F1 in this cancer). While the study has informed on drug transport and drug metabolism pharmacogenetics, it has fallen short of identifying a suitable target for molecular therapy.
    Sarcoma 04/2015; 2015:1-20. DOI:10.1155/2015/839182
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    ABSTRACT: Several patient demographic factors, including marital status, have been demonstrated to have prognostic significance for survival in extremity soft tissue sarcoma (ESTS). A study population of 12,546 adult patients diagnosed with ESTS from 1991 to 2010 was identified from the SEER database, a large population-based registry, in order to determine whether overall survival had changed over this recent 20-year period. The study population was divided into three groups by year of diagnosis: 1991-1996, 1997-2003, and 2004-2010. We used the Kaplan-Meier method and Cox proportional hazards regression to assess survival differences between different demographic groups and prognostic clinical characteristics. Over the course of time, the 5-year overall survival rates have increased from 28% in the earliest time period to 62% in the latest (P < 0.0001). On multivariate analysis, the mortality rate progressively declined from the 1991-1996 group (HR: 3.02, CI: 2.78-3.29) to the 1997-2003 group (HR: 2.21, CI: 2.06-2.37), with the 2004-2010 group having the best overall survival, despite increases in the proportion of patients with tumors greater than 5 cm in size (P < 0.0001), and those presenting with metastasis (P < 0.0001).
    Sarcoma 03/2015; 2015:279601. DOI:10.1155/2015/279601
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    ABSTRACT: . Superficial soft tissue sarcomas (S-STS) are generally amenable to wide excision. We hypothesized that local recurrence (LR) should be low, even without radiation therapy (RT), and sought to examine the contribution of depth to LR and OS. Methods . Patients with S-STS were retrospectively reviewed. Demographics, tumor features, treatment received, and outcomes were analyzed. Results . 103 patients were identified. Median age was 55 years; 53% of patients were female. Tumor site was 39% in trunk, 38% in the lower extremity, 14% in the upper extremity, and 9% in other locations. The most common histology was 36% leiomyosarcoma. Median tumor size was 2.8 cm (range 0.2–14 cm). Sixty-six percent of tumors were of intermediate/high grade. RT was administered preoperatively in 6% of patients and postoperatively in 15% of patients. An R0 resection was accomplished in 92%. At a median follow-up of 34.2 months (range 2.3–176), 9 patients had a LR (8.7%). Tumor size and grade were not associated with LR. OS was not associated with any tumor or patient variables on univariate analysis. Conclusions . LR was low for S-STS, even with large or high grade tumors and selective use of RT. Surgical resection alone may be adequate therapy for most patients. Superficial location seems to supersede other factors imparting a good prognosis for this group of tumors.
    Sarcoma 01/2015; 2015(3):1-7. DOI:10.1155/2015/325049
  • Jody E. Hooper · Emma L. Cantor · Macgregor S. Ehlen · Avirup Banerjee · Suman Malempati · Peter Stenzel · Randy L. Woltjer · Regina Gandour-Edwards · Neal C. Goodwin · Yan Yang · Pali Kaur · Carol J. Bult · Susan D. Airhart · Charles Keller ·
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    ABSTRACT: Embryonal rhabdomyosarcoma (eRMS) is one of the most common soft tissue sarcomas in children and adolescents. Parameningeal eRMS is a variant that is often more difficult to treat than eRMS occurring at other sites. A 14-year-old female with persistent headaches and rapid weight loss was diagnosed with parameningeal eRMS. She progressed and died despite chemotherapy with vincristine, actinomycin-D, and cyclophosphamide plus 50.4 Gy radiation therapy to the primary tumor site. Tumor specimens were acquired by rapid autopsy and tumor tissue was transplanted into immunodeficient mice to create a patient-derived xenograft (PDX) animal model. As autopsy specimens had an ALK R1181C mutation, PDX tumor bearing animals were treated with the pan-kinase inhibitor lestaurtinib but demonstrated no decrease in tumor growth, suggesting that single agent kinase inhibitor therapy may be insufficient in similar cases. This unique parameningeal eRMS PDX model is publicly available for preclinical study.
    Sarcoma 01/2015; 2015(3):1-7. DOI:10.1155/2015/826124