Clinical Sarcoma Research最新文献

{"title":"A rare presentation of carcinosarcoma of the bone in a young female; response with gemcitabine and docetaxel.","authors":"Nicholas King,&nbsp;Keshav Kukreja,&nbsp;Albina Murzabdillaeva,&nbsp;Yasir Ali,&nbsp;Jason Willis,&nbsp;Abhishek Maiti,&nbsp;Hilary Ma,&nbsp;Joan Bull","doi":"10.1186/s13569-019-0120-7","DOIUrl":"https://doi.org/10.1186/s13569-019-0120-7","url":null,"abstract":"<p><strong>Background: </strong>Sarcomatoid carcinoma, or carcinosarcoma, is a neoplasm that contains both sarcomatous and carcinomatous elements. It is an extremely rare cancer most often arising from visceral organs. Here we report the seventh documented de novo case of carcinosarcoma of the bone, in a young female who showed initial clinical improvement with gemcitabine and docetaxel.</p><p><strong>Case presentation: </strong>A 36-year-old Caucasian female presented with diffuse musculoskeletal pain that had progressed from her shoulder to her back, arm, and knee over 6 months. Imaging revealed diffuse sclerotic lesions of bilateral humeral heads, iliac and ischial bones, and thoracic and lumbar spine. Histopathologic examination of biopsies from the T9 vertebra and left femur showed mainly sarcomatous spindle cells with focal osteoid production. Immunostaining showed the cells to be OSCAR cytokeratin, patchy positive for pankeratin, and negative for CK7, GATA3, S100, SOX10, CD99, EMA, AE1/AE3, and HMW keratin indicative of an epithelial origin. After thorough clinical correlation, sarcomatoid carcinoma of a visceral organ was excluded and the diagnosis of primary sarcomatoid carcinoma of the bone was ultimately favored. She received chemotherapy with gemcitabine and docetaxel, and showed improvement at 6 months but ultimately passed 1 year post diagnosis.</p><p><strong>Conclusions: </strong>Primary carcinosarcoma of the bone is an extremely rare malignancy. Early diagnosis is crucial as localized disease may be curable with resection. As shown in this case, combination chemotherapy with gemcitabine and docetaxel is a potential option in patients with unresectable or metastatic disease.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"10"},"PeriodicalIF":0.0,"publicationDate":"2019-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-019-0120-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37158472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Radiotherapy resistance in chondrosarcoma cells; a possible correlation with alterations in cell cycle related genes.","authors":"Yvonne de Jong,&nbsp;Martha Ingola,&nbsp;Inge H Briaire-de Bruijn,&nbsp;Alwine B Kruisselbrink,&nbsp;Sanne Venneker,&nbsp;Ieva Palubeckaite,&nbsp;Bram P A M Heijs,&nbsp;Anne-Marie Cleton-Jansen,&nbsp;Rick L M Haas,&nbsp;Judith V M G Bovée","doi":"10.1186/s13569-019-0119-0","DOIUrl":"https://doi.org/10.1186/s13569-019-0119-0","url":null,"abstract":"<p><strong>Background: </strong>Conventional chondrosarcomas are malignant cartilage tumors considered radioresistant. Nevertheless, retrospective series show a small but significant survival benefit for patients with locally advanced disease treated with radiotherapy. And, in daily practice when considered inoperable their irradiation is an accepted indication for proton beam radiotherapy. Therefore, we investigated the sensitivity of chondrosarcoma cell lines and -tissue samples towards radiotherapy and screened for biomarkers to identify predictors of radiosensitivity.</p><p><strong>Methods: </strong>Proliferation and clonogenic assays were performed in chondrosarcoma cell lines after γ-radiation in combination with mutant IDH1 inhibitor AGI-5198. In addition, glutathione levels were measured using mass spectrometry. Chondrosarcoma tumor explants were irradiated after which γ-H2AX foci were counted. Mutation analysis was performed using the Ion AmpliSeq™ Cancer Hotspot Panel and immunohistochemical staining's were performed for P-S6, LC-3B, P53, Bcl-2, Bcl-xl and Survivin. Results were correlated with the number of γ-H2AX foci.</p><p><strong>Results: </strong>Chondrosarcoma cell lines were variably γ-radiation resistant. No difference in radiosensitivity, nor glutathione levels was observed after treatment with AGI-5198. Irradiated chondrosarcoma patient tissue presented a variable increase in γ-H2AX foci compared to non-radiated tissue. Samples were divided into two groups, high and low radioresistant, based on the amount of γ-H2AX foci. All four highly resistant tumors exhibited mutations in the pRb pathway, while none of the less radioresistant tumors showed mutations in these genes.</p><p><strong>Conclusions: </strong>Chondrosarcoma cell lines as well as primary tumors are variably radioresistant, particularly in case of a defective Rb pathway. Whether selection for radiotherapy can be based upon an intact Rb pathway should be further investigated.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"9"},"PeriodicalIF":0.0,"publicationDate":"2019-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-019-0119-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37299058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immunophenotypic expression of UCP1 in hibernoma and other adipose/non adipose soft tissue tumours.","authors":"Jessica Malzahn, Afroditi Kastrenopoulou, Ioanna Papadimitriou-Olivgeri, Dionysios J Papachristou, Jennifer M Brown, Udo Oppermann, Nick A Athanasou","doi":"10.1186/s13569-019-0118-1","DOIUrl":"10.1186/s13569-019-0118-1","url":null,"abstract":"<p><strong>Background: </strong>Uncoupling protein 1 (UCP1) is a mitochondral protein transporter that uncouples electron transport from ATP production. UCP1 is highly expressed in brown adipose tissue (BAT), including hibernomas, but its expression in other adipose tumours is uncertain. UCP1 has also been found in other tissues (e.g. smooth muscle) but whether it is expressed in non-adipose benign and malignant soft tissue tumours is unknown.</p><p><strong>Methods: </strong>Immunohistochemical staining of normal (axillary) BAT and subcutaneous/abdominal white adipose tissue (WAT) as well as a wide range of benign and malignant primary soft tissue tumours (n = 171) was performed using a rabbit polyclonal antibody to UCP1. BAT and hibernomas were also stained by immunohistochemistry with monoclonal and polyclonal antibodies to adipose/non-adipose tumour markers in order to characterise the immunophenotype of BAT cells.</p><p><strong>Results: </strong>UCP1 was strongly expressed in the cytoplasm of brown fat cells in BAT and hibernomas, both of which also expressed aP2, S100, CD31, vimentin and calponin. UCP1 was not expressed in WAT or other adipose tumours with the exception a few tumour cells in pleomorphic liposarcoma. UCP1 was variably expressed by tumour cells in a few non-adipose sarcomas including leiomyosarcoma, rhabdomyosarcoma, alveolar soft part sarcoma, synovial sarcoma and clear cell sarcoma.</p><p><strong>Conclusions: </strong>UCP1 is strongly expressed in BAT but not WAT and is found in all hibernomas and a few pleomorphic liposarcomas but not in other adipose tumours. UCP1 expression in a few non-adipose soft tissue sarcomas may possibly reflect origin of tumour cells from a common mesenchymal stem cell precursor and/or developmental pathway.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"8"},"PeriodicalIF":0.0,"publicationDate":"2019-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6515671/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37261620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction to: Survival in advanced GIST has improved over time and correlates with increased access to post-imatinib tyrosine kinase inhibitors: results from Life Raft Group Registry.","authors":"Jerry W Call,&nbsp;Yu Wang,&nbsp;Denisse Montoya,&nbsp;Norman J Scherzer,&nbsp;Michael C Heinrich","doi":"10.1186/s13569-019-0117-2","DOIUrl":"https://doi.org/10.1186/s13569-019-0117-2","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1186/s13569-019-0114-5.].</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"7"},"PeriodicalIF":0.0,"publicationDate":"2019-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-019-0117-2","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37236040","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Perspectives on treatment side effects in patients with metastatic gastrointestinal stromal tumour: a qualitative study.","authors":"Lena Fauske,&nbsp;Ivar Hompland,&nbsp;Geir Lorem,&nbsp;Hilde Bondevik,&nbsp;Øyvind S Bruland","doi":"10.1186/s13569-019-0116-3","DOIUrl":"https://doi.org/10.1186/s13569-019-0116-3","url":null,"abstract":"<p><strong>Background: </strong>This study aims to explore how patients with metastatic gastrointestinal stromal tumour (GIST) experience the adverse effects of treatment, as expressed by the individuals themselves.</p><p><strong>Methods: </strong>A qualitative, phenomenological and hermeneutic design was applied. Twenty patients with metastatic GIST participated in the study. In-depth and semi-structured interviews were conducted and then analysed by means of an inductive thematic analysis.</p><p><strong>Results: </strong>The majority of participants reported experiencing a changed life after being diagnosed with metastatic GIST and commencing systemic medical treatment. More than half of them described partially debilitating self-reported side effects and complaints that had a detrimental impact on their lives. The life-prolonging tyrosine kinase inhibitor treatment prompted the participants to adapt to 'a new normal'. Several participants also emphasised having an ambivalent relationship with the pill, although most looked upon it as 'a friend' because it kept them alive. Paradoxically, while the participants struggled with the side effects of treatment as well as the consequences of living with a chronic cancer, half of them considered themselves to be healthy and, thus, to not actually be cancer patients.</p><p><strong>Conclusions: </strong>We observed a gap between the biomedical perspective on disease that health professionals typically adopt and the individual experiences of patients living with metastatic GIST. For those patients who are living in limbo between having metastatic cancer and offered an effective treatment, a holistic view of health on the part of their healthcare providers seems crucial. A vital goal should hence be to improve communication between healthcare professionals and GIST patients so as to secure an individualised follow-up with guidance on coping with, and adapting to, their new normal.<i>Trial registration</i> The study was approved by the data protection officer of the Oslo University Hospital (Approval Number 2016/15358).</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"6"},"PeriodicalIF":0.0,"publicationDate":"2019-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-019-0116-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37215879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The challenge of finding new therapeutic avenues in soft tissue sarcomas.","authors":"Bernd Kasper","doi":"10.1186/s13569-019-0115-4","DOIUrl":"https://doi.org/10.1186/s13569-019-0115-4","url":null,"abstract":"<p><p>Soft tissue sarcomas are rare malignancies of mesenchymal origin comprising about 1% of all adult cancers. Systemic therapies for locally advanced and metastatic disease have been restricted for decades to very few effective and approved agents such as doxorubicin and ifosfamide. However, new therapeutic avenues including new drug developments and registrations such as trabectedin, pazopanib and eribulin as well as numerous clinical trial options have recently enriched the therapeutic armamentarium in the treatment of patients with advanced soft tissue sarcomas. The challenges and pitfalls of finding such new therapeutic avenues in recent years for the treatment benefit of patients with soft tissue sarcomas will be presented in this chapter within the thematic series on \"Challenges in Sarcoma\".</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"5"},"PeriodicalIF":0.0,"publicationDate":"2019-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-019-0115-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37170480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Survival in advanced GIST has improved over time and correlates with increased access to post-imatinib tyrosine kinase inhibitors: results from Life Raft Group Registry.","authors":"Jerry W Call,&nbsp;Yu Wang,&nbsp;Denisse Montoya,&nbsp;Norman J Scherzer,&nbsp;Michael C Heinrich","doi":"10.1186/s13569-019-0114-5","DOIUrl":"https://doi.org/10.1186/s13569-019-0114-5","url":null,"abstract":"<p><strong>Background: </strong>The use of imatinib, sunitinib, and regorafenib has transformed the treatment of advanced GIST. Sunitinib and regorafenib improve progression free-survival in the second (2L) and third (3L) line, respectively, compared with placebo. However, the impact of these agents on overall survival (OS) is unclear.</p><p><strong>Methods: </strong>The Life Raft Group (LRG) patient registry contains records from 1716 GIST patients; 526 have advanced to at least 2L treatment. Patient-reported treatment and outcome data were examined to determine treatment patterns and their impact on OS.</p><p><strong>Results: </strong>Median OS from start of 2L therapy was 32.4 months for sunitinib (n = 436) compared with 27.1 months for patients treated with any other 2L drug (n = 74, <i>p</i> = 0.023, HR 1.377) and 16.8 months for patients who never received sunitinib in any treatment line (n = 42, <i>p</i> = 0.028, HR 1.52). In patients reporting progression in 2L, the median OS in patients subsequently receiving 3L regorafenib (n = 53, 26.2 months) was longer than that of 3L patients who never received regorafenib in any line of therapy (n = 174, 14.3 months, <i>p</i> = 0.0002, HR 2.231), and was longer than that of patients who received any other 3L treatment (19.8 months, <i>p</i> = 0.044, HR 1.525). OS for advanced GIST patients in the LRG registry has improved over time (<i>p</i> = 0.0013), correlated with the increased use of TKIs in ≥ 2L settings.</p><p><strong>Conclusions: </strong>In our analysis, sunitinib and regorafenib significantly improved OS compared with patients who never received these agents. Our data also support the hypothesis that the use of KIT/PDGFRA inhibitors, including non-approved agents, has improved OS for patients with imatinib- and sunitinib-resistant GIST.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"4"},"PeriodicalIF":0.0,"publicationDate":"2019-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-019-0114-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37315365","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genomic aberrations in cell cycle genes predict progression of <i>KIT</i>-mutant gastrointestinal stromal tumors (GISTs).","authors":"Michael C Heinrich, Janice Patterson, Carol Beadling, Yuexiang Wang, Maria Debiec-Rychter, Barbara Dewaele, Christopher L Corless, Anette Duensing, Chandrajit P Raut, Brian Rubin, Tamas Ordog, Matt van de Rijn, Jerry Call, Thomas Mühlenberg, Jonathan A Fletcher, Sebastian Bauer","doi":"10.1186/s13569-019-0112-7","DOIUrl":"10.1186/s13569-019-0112-7","url":null,"abstract":"<p><strong>Background: </strong>Activating mutations of the receptor tyrosine kinase <i>KIT</i> are early events in the development of most gastrointestinal stromal tumors (GISTs). Although GISTs generally remain dependent on oncogenic <i>KIT</i> during tumor progression, <i>KIT</i> mutations alone are insufficient to induce malignant behavior. This is evidenced by <i>KIT</i>-mutant micro-GISTs, which are present in up to one-third of normal individuals, but virtually never progress to malignancy.</p><p><strong>Methods: </strong>We performed whole exome sequencing on 29 tumors obtained from 21 patients with high grade or metastatic <i>KIT</i>-mutant GIST (discovery set). We further validated the frequency and potential prognostic significance of aberrations in <i>CDKN2A/B, RB1,</i> and <i>TP53</i> in an independent series of 71 patients with primary GIST (validation set).</p><p><strong>Results: </strong>Using whole exome sequencing we found significant enrichment of genomic aberrations in cell cycle-associated genes (Fisher's Exact p = 0.001), most commonly affecting <i>CDKN2A/B, RB1, and TP53</i> in our discovery set. We found a low mutational tumor burden in these 29 advanced GIST samples, a finding with significant implications for the development of immunotherapy for GIST. In addition, we found mutation of spliceosome genes in a minority of cases, implicating dysregulation of splicing as a potential cancer promoting mechanism in GIST. We next assessed the prognostic significance of <i>CDKN2A, RB1</i> or <i>TP53</i> mutation/copy loss in an independent cohort of 71 patients with primary GIST. Genetic events (mutation, deletion, and/or LOH) involving at least one of the three genes examined were found in 17% of the very low-risk, 36% of the low-risk, 42% of the intermediate risk, 67% of the high-risk/low mitotic-count, and in 86% of the high-risk/high mitotic-count group. The presence of cell cycle-related events was associated with a significantly shorter relapse-free survival (median 67 months versus not reached; <i>p</i> < 0.0001) and overall survival (Log Rank, <i>p </i>= 0.042).</p><p><strong>Conclusion: </strong>Our results demonstrate that genomic events targeting cell cycle-related genes are associated with GIST progression to malignant disease. Based on this data, we propose a model for molecular pathogenesis of malignant GIST.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"3"},"PeriodicalIF":0.0,"publicationDate":"2019-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399846/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37053101","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genome-wide methylation profiling and copy number analysis in atypical fibroxanthomas and pleomorphic dermal sarcomas indicate a similar molecular phenotype.","authors":"Christian Koelsche,&nbsp;Damian Stichel,&nbsp;Klaus G Griewank,&nbsp;Daniel Schrimpf,&nbsp;David E Reuss,&nbsp;Melanie Bewerunge-Hudler,&nbsp;Christian Vokuhl,&nbsp;Winand N M Dinjens,&nbsp;Iver Petersen,&nbsp;Michel Mittelbronn,&nbsp;Adrian Cuevas-Bourdier,&nbsp;Rolf Buslei,&nbsp;Stefan M Pfister,&nbsp;Uta Flucke,&nbsp;Gunhild Mechtersheimer,&nbsp;Thomas Mentzel,&nbsp;Andreas von Deimling","doi":"10.1186/s13569-019-0113-6","DOIUrl":"https://doi.org/10.1186/s13569-019-0113-6","url":null,"abstract":"<p><strong>Background: </strong>Atypical fibroxanthomas (AFX) and pleomorphic dermal sarcomas (PDS) are lesions of the skin with overlapping histologic features and unspecific molecular traits. PDS behaves aggressive compared to AFX. Thus, a precise delineation, although challenging in some instances, is relevant.</p><p><strong>Methods: </strong>We examined the value of DNA-methylation profiling and copy number analysis for separating these tumors. DNA-methylation data were generated from 17 AFX and 15 PDS using the Illumina EPIC array. These were compared with DNA-methylation data generated from 196 tumors encompassing potential histologic mimics like cutaneous squamous carcinomas (cSCC; n = 19), basal cell carcinomas (n = 10), melanoma metastases originating from the skin (n = 11), leiomyosarcomas (n = 11), angiosarcomas of the skin and soft tissue (n = 11), malignant peripheral nerve sheath tumors (n = 19), dermatofibrosarcomas protuberans (n = 13), extraskeletal myxoid chondrosarcomas (n = 9), myxoid liposarcomas (n = 14), schwannomas (n = 10), neurofibromas (n = 21), alveolar (n = 19) and embryonal (n = 17) rhabdomyosarcomas as well as undifferentiated pleomorphic sarcomas (n = 12).</p><p><strong>Results: </strong>DNA-methylation profiling did not separate AFX from PDS. The DNA-methylation profiles of the other cases, however, were distinct from AFX/PDS. They reliably assigned to subtype-specific DNA-methylation clusters, although overlap occurred between some AFX/PDS and cSCC. Copy number profiling revealed alterations in a similar frequency and distribution between AFX and PDS. They involved losses of 9p (22/32) and 13q (25/32). Gains frequently involved 8q (8/32). Notably, a homozygous deletion of <i>CDKN2A</i> was more frequent in PDS (6/15) than in AFX (2/17), whereas amplifications were non-recurrent and overall rare (5/32).</p><p><strong>Conclusions: </strong>Our findings support the concept that AFX and PDS belong to a common tumor spectrum. We could demonstrate the diagnostic value of DNA-methylation profiling to delineating AFX/PDS from potential mimics. However, the assessment of certain histologic features remains crucial for separating PDS from AFX.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"2"},"PeriodicalIF":0.0,"publicationDate":"2019-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-019-0113-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37002717","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term cure of soft tissue sarcoma with pegylated-liposomal doxorubicin after doxorubicin and ifosfamide failure.","authors":"Malvi Savani,&nbsp;Paari Murugan,&nbsp;Keith M Skubitz","doi":"10.1186/s13569-018-0111-0","DOIUrl":"https://doi.org/10.1186/s13569-018-0111-0","url":null,"abstract":"<p><strong>Background: </strong>Doxorubicin is one of the most active drugs available for the treatment of sarcoma. Pegylated-liposomal doxorubicin (PLD) is a formulation of doxorubicin in which the doxorubicin is encapsulated in liposomes coated with methoxypoly (ethylene glycol); this formulation results in decreased uptake by the reticuloendothelial system, higher concentrations of drug in tumor, and less toxicity, including reduced cardiotoxicity, nausea, alopecia, and myelosuppression. No premedication is necessary. While PLD has a better toxicity profile than free doxorubicin, there is no consensus on the relative efficacy of PLD and free doxorubicin in sarcoma.</p><p><strong>Case presentation: </strong>In this report, we describe a patient with high-grade metastatic soft tissue sarcoma with rapid recurrence after adjuvant treatment with free doxorubicin, cisplatin, ifosfamide, and dacarbazine. Second-line treatment with PLD resulted in long-term disease remission during a 20-year follow-up period. Mucositis and hand-foot syndrome were controlled by adjustment of dose and treatment interval.</p><p><strong>Conclusions: </strong>This case illustrates the curative potential of PLD after failure of free doxorubicin and the absence of long term cardiotoxicity with PLD. As with all drugs, individual adjustment of dose and treatment interval is important.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"9 ","pages":"1"},"PeriodicalIF":0.0,"publicationDate":"2019-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-018-0111-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36860565","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}