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Epidemiology, Treatment Patterns, and Outcomes of Metastatic Soft Tissue Sarcoma in a Community-Based Oncology Network  [PDF]
Clara Chen,Rohit Borker,James Ewing,Wan-Yu Tseng,Michelle D. Hackshaw,Shanmugapriya Saravanan,Rahul Dhanda,Eric Nadler
Sarcoma , 2014, DOI: 10.1155/2014/145764
Abstract: Purpose. To assess epidemiology, treatment patterns, and outcomes of metastatic soft tissue sarcoma (mSTS) patients in USA community oncology practices. Methods. This retrospective, descriptive study used US Oncology’s iKnowMed electronic health records database. Adults (≥18 years) with mSTS and at least two visits between July 2007 and June 2010 were included. Key outcomes were practice patterns, overall survival (OS), and progression-free survival (PFS). Results. 363 mSTS patients (174 treated and 189 untreated) met the prespecified exclusion/inclusion criteria. The most common subtypes were leiomyosarcoma ( ; 29%), liposarcoma ( ; 11%), and synovial sarcoma ( ; 3%); the remainder ( ; 57%) comprised 27 histologic subtypes. Treated patients were younger and had lower ECOG scores; 75% and 25% received first-line combination or monotherapy, respectively. Median OS of treated and untreated patients was 22 and 17 months, respectively, and 29 months in patients with the three most common subtypes. Before controlling for effects of covariates, younger age and lower ECOG scores were associated with better OS and PFS. Conclusion. This study provides insights into mSTS epidemiology, treatment patterns, and outcomes in a large community-based oncology network. These results warrant further studies with larger cohorts. 1. Introduction Soft tissue sarcomas (STS) are rare mesenchymal tumors that account for 1% of adult cancers [1–7] and comprise over 50 different histologic subtypes that differ in pathogenesis and outcomes [1–3, 6, 7]. Collectively, they are associated with a mortality rate of over 4,000 patients per year [2]. The treatment of STS is dependent upon several factors, including histologic subtype, disease stage, and patient performance status; the treatment options include surgery, radiotherapy, and/or chemotherapy [1, 3, 6–10]. Although localized resected disease can often be cured, the prognosis of patients with metastatic STS (mSTS) remains poor, with median survival of approximately one year [1, 3, 6, 11–15]. Good prognostic factors for mSTS include younger patients with good performance status and low tumor grade [3, 6, 16]. Most of our insights regarding factors that influence the outcomes following chemotherapy in mSTS have been obtained mainly from clinical trials [11–14, 16]. The purpose of this study was to gain an improved understanding of the “realworld” epidemiology as well as treatment patterns and outcomes of mSTS in the setting of community oncology clinics. 2. Methods Data was obtained from the McKesson Specialty Health (MSH)/US
Epidemiology Of Oral Kaposi’s Sarcoma In Zimbabwe 1988-1997: A Population-based Study
C Marimo
Medical Journal of Zambia , 2010,
Abstract: Objective: To date, no study has investigated the incidence of oral Kaposi’s sarcoma (OKS) in African populations affected by the human immunodeficiency virus (HIV) and the Acquired ImmunoDeficiency Syndrome AIDS epidemic. It is, therefore, the purpose of this study to assess the burden of OKS in the Zimbabwean population over a ten-year period. Design: A descriptive epidemiological study was undertaken to assess the burden of OKS by determining the frequencies, incidence and cumulative rates, the lifetime risk and odds of developing OKS according to site (topography), gender, age, race/ethnic origin of the Zimbabwean population. Incident cases of OKS from the upper and lower lips, oral vestibule, retromolar area, floor of mouth, tongue, cheek mucosa, gums, hard and soft palate were accessed from the Zimbabwe National Cancer Registry (ZNCR). Cases from the skin, pharynx, larynx and the major salivary glands were excluded from the study. Setting: This comprised the population of Zimbabwe during a ten-year period 1988 to 1997 using population figures from the 1992 Census Zimbabwe National Report. The study population was standardised by the direct method against the world standard population to calculate the age standardized incidence rate (ASIR). The SPSS statistical software program (SPSS Inc.2001, USA) was used for the statistical analysis. Results: OKS comprised 0.92% of total body malignancies and 51% of oral malignancies with a mean age of study cases of 37.6 years and median age of 32 years. Histology of the primary (64.5%) and clinical diagnosis (34.6%) were the predominant methods of diagnosis. OKS affected almost exclusively blacks and males more than females with a male to female ratio of 1.9:1. The most affected age groups by OKS were the 30-34 for males and 25-29 for both females and the whole population. OKS mostly affected the palate (70,2%) followed by, in descending order, the tongue (13.3%) and mouth (8.3%). The age adjusted age standardised incidence rate (ASIR) of OKS exponentially increased the entire study period surpassing oral squamous cell carcinoma (OSCC) as the predominant oral malignancy in 1994. Conclusions: OKS was the commonest malignancy of young adults affecting males more than females and surpassed oral squamous cell carcinoma in 1994 to become the commonest oral malignancy for the remainder of the study period. The palate was the most affected intraoral site by OKS. The possibility of human herpes virus 8 being HIV strain-specific in the aetiopathogenesis of oral Kaposi’s sarcoma warrants further investigation.
Sarcoma de Ewing: epidemiologia e prognóstico dos pacientes tratados no Instituto de Oncologia Pediátrica, IOP-GRAACC-UNIFESP Ewing's sarcoma: epidemiology and prognosis for patients treated at the Pediatric Oncology Institute, IOP-GRAACC-UNIFESP  [cached]
Davi Gabriel Bellan,Reynaldo Jesus-Garcia Filho,Jairo Greco Garcia,Marcelo de Toledo Petrilli
Revista Brasileira de Ortopedia , 2012, DOI: 10.1590/s0102-36162012000400007
Abstract: OBJETIVO: Tra ar o perfil epidemiológico e o prognóstico do sarcoma de Ewing na popula o brasileira. Material e MéTODO: Foram avaliados, retrospectivamente, os prontuários de 64 pacientes tratados, com sarcoma de Ewing intraósseo, no Instituto de Oncologia Pediátrica, IOP-GRAACC-Unifesp, no período de 1995 a 2010. RESULTADOS: A análise estatística dos dados obtidos n o correlacionou fatores como sexo, trauma, fratura patológica e tempo ao diagnóstico com o desfecho do tratamento. Fatores como metástase inicial, metástase pulmonar, local do tumor, idade, recidiva e tipo de cirurgia indicam resultados que corroboram a literatura consagrada. CONCLUS O: O prognóstico no sarcoma de Ewing foi influenciado principalmente pela presen a de metástase ao diagnóstico. OBJECTIVE: To outline the epidemiological profile and prognosis for Ewing's sarcoma in the Brazilian population. Material and METHODS: The medical records of 64 patients with intraosseous Ewing's sarcoma who were treated at the Pediatric Oncology Institute, IOP-GRAACC-Unifesp, between 1995 and 2010, were retrospectively evaluated. RESULTS: The statistical analysis on the data obtained did not correlate factors such as sex, trauma, pathological fracture and time taken for case diagnosis with the treatment outcome. Factors such as initial metastasis, lung metastasis, tumor site, age, recurrence and type of surgery showed results corroborating what has been established in the literature. CONCLUSION: The prognosis in cases of Ewing's sarcoma was mainly influenced by the presence of metastases at the time of diagnosis.
M. Farzan
Acta Medica Iranica , 1997,
Abstract: Ten cases of synovial cell sarcoma are reported. The youngest patient was a 2'A years old boy with synovial cell sarcoma of the knee and the oldest one was a man with synovial cell sarcoma of the elbow.
Histiocytic sarcoma
Machado, Eduardo Silva;Miranda, Ana Carolina de;Escopelli, Ticiane;Caron, Ruggero;Escopelli, Alessandra Cristhina;
Revista Brasileira de Hematologia e Hemoterapia , 2011, DOI: 10.5581/1516-8484.20110038
Abstract: a 59-year-old white woman, sc, after being treated for pneumonia, presented with an increase in the size of lymph nodes. the immunohistochemical examination diagnosed histiocytic sarcoma. relapse occurred 12 months after starting chemotherapy. the patient evolved with febrile neutropenia, septic shock and death.
Sarcoma Immunotherapy  [PDF]
Launce G. Gouw,Kevin B. Jones,Sunil Sharma,R. Lor Randall
Cancers , 2011, DOI: 10.3390/cancers3044139
Abstract: Much of our knowledge regarding cancer immunotherapy has been derived from sarcoma models. However, translation of preclinical findings to bedside success has been limited in this disease, though several intriguing clinical studies hint at the potential efficacy of this treatment modality. The rarity and heterogeneity of tumors of mesenchymal origin continues to be a challenge from a therapeutic standpoint. Nonetheless, sarcomas remain attractive targets for immunotherapy, as they can be characterized by specific epitopes, either from their mesenchymal origins or specific alterations in gene products. To date, standard vaccine trials have proven disappointing, likely due to mechanisms by which tumors equilibrate with and ultimately escape immune surveillance. More sophisticated approaches will likely require multimodal techniques, both by enhancing immunity, but also geared towards overcoming innate mechanisms of immunosuppression that favor tumorigenesis.
Molecular pathogenesis and targeted therapeutics in Ewing sarcoma/primitive neuroectodermal tumours
Fergal C Kelleher, David M Thomas
Clinical Sarcoma Research , 2012, DOI: 10.1186/2045-3329-2-6
Abstract: A PUBMED search was performed from 1997 to 2011. Published literature that included the topic of the Ewing sarcoma/PNET was also referenced.Insulin-like growth factor-1 receptor (IGF-1R) antagonists have demonstrated modest single agent efficacy in phase I/II clinical trials in Ewing sarcoma/PNET, but have a strong preclinical rationale. Based on in vitro and animal data, treatment using antisense RNA and cDNA oligonucleotides directed at silencing the EWS-FLI chimera that occurs in most Ewing sarcoma/PNET may have potential therapeutic importance. However drug delivery and degradation problems may limit this therapeutic approach. Protein-protein interactions can be targeted by inhibition of RNA helicase A, which binds to EWS/FLI as part of the transcriptional complex. Tumour necrosis factor related apoptosis inducing ligand induction using interferon has been used in preclinical models. Interferons may be incorporated into future chemotherapeutic treatment paradigms. Histone deacetylase inhibitors can restore TGF-β receptor II allowing TFF-β signalling, which appears to inhibit growth of Ewing sarcoma/PNET cell lines in vitro. Immunotherapy using allogeneic natural killer cells has activity in Ewing sarcoma/PNET cell lines and xenograft models. Finally, cyclin dependent kinase inhibitors such as flavopiridol may be clinically efficacious in relapsed Ewing sarcoma/PNET.Preclinical evidence exists that targeted therapeutics may be efficacious in the ESFT. IGF-1R antagonists have demonstrated efficacy in phase I/II clinical trials, although predicting responses remains a challenge. The future treatment of Ewing sarcoma/PNET is likely to be improved by these scientific advances.Ewing sarcoma/PNET is a high grade malignancy in which approximately 75% of cases are localised at diagnosis, and 25% are initially metastatic [1-3]. The Surveillance Epidemiology and End Results (SEER) program reported an annual incidence rate of 2.93 cases/1,000,000 in the interval from 1973 t
Osteogenic Sarcoma of Lower Jaw.  [PDF]
Des Raj Bhagat, Anayat Lone, Subash Bhardwaj, Aniece Choudhary.
JK Science : Journal of Medical Education & Research , 2004,
Abstract: A rare case of osteogenic sarcoma affecting the lower jaw is presented. Osteogenic sarcoma ofthe jaw is a very rare tumor of the head and neck comprising less than 0.5% of all malignanttumors of head and neck. Pertinent literature is reviewed to emphasize the various treatmentmodalities and histopathological features for diagnosis of this rare tumour.
Primary Renal Synovial Sarcoma
Mehmet Gulum,Ercan Yeni,Murat Savas,Ilyas Ozardali,Ismail Ozdemir,Dilek Mil,Adem Altunkol,Halil Ciftci
Case Reports in Urology , 2011, DOI: 10.1155/2011/810184
Abstract: Synovial sarcomas are generally deep-seated tumors that most often occur in the proximity of large joints of adolescents and young adults. We describe two cases of primary renal synovial sarcoma that were treated successfully by radical nephrectomy. Synovial sarcoma originating from the kidney is extremely rare and the histogenesis is uncertain. Surgical resection and ifosfamide based chemotherapy are the mainstay for the management of renal synovial sarcoma. Fewer than 40 patients have been described in the English literature. Physicians should be aware of the possibility of malignancy in cystic renal masses and raise the suspicion of synovial sarcoma, especially when patients with renal masses are a young adult.
Intra articular synovial sarcoma  [cached]
Sistla Radha,Tameem Afroz,Vidyasagar JVS
Indian Journal of Pathology and Microbiology , 2010,
Abstract: Synovial sarcoma is a soft tissue neoplasm with a characteristic biphasic pattern. Incidence in soft tissues is 5-10%. Intra articularly synovial sarcoma is extremely rare. Fewer than 5% of all synovial sarcomas arise within the joint space. We report a case of intra articular synovial sarcoma in a young male who presented as internal derangement of the knee.
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