Clinical Sarcoma Research最新文献

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Survival of soft tissue sarcoma patients after completing six cycles of first-line anthracycline containing treatment: an EORTC-STBSG database study. 完成六个周期一线蒽环类药物治疗后软组织肉瘤患者的生存:EORTC-STBSG数据库研究
Clinical Sarcoma Research Pub Date : 2020-09-09 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00137-5
Arie Jan Verschoor, Saskia Litière, Sandrine Marréaud, Ian Judson, Maud Toulmonde, Eva Wardelmann, Axel LeCesne, Hans Gelderblom
{"title":"Survival of soft tissue sarcoma patients after completing six cycles of first-line anthracycline containing treatment: an EORTC-STBSG database study.","authors":"Arie Jan Verschoor,&nbsp;Saskia Litière,&nbsp;Sandrine Marréaud,&nbsp;Ian Judson,&nbsp;Maud Toulmonde,&nbsp;Eva Wardelmann,&nbsp;Axel LeCesne,&nbsp;Hans Gelderblom","doi":"10.1186/s13569-020-00137-5","DOIUrl":"https://doi.org/10.1186/s13569-020-00137-5","url":null,"abstract":"<p><strong>Background: </strong>Doxorubicin based chemotherapy is standard first line treatment for patients with soft tissue sarcoma. Currently several options to improve survival after doxorubicin based chemotherapy are being studied. This study reports on survival after completing 6 cycles of doxorubicin containing first line treatment, which is important when designing studies trying to improve outcomes of first line treatment.</p><p><strong>Methods: </strong>A retrospective database analysis was performed on 2045 patients from 12 EORTC sarcoma trials (inclusion period 1980-2012) receiving first line doxorubicin based chemotherapy for advanced soft tissue sarcoma in order to establish progression free survival and overall survival after completing 6 cycles of first line doxorubicin based chemotherapy. Endpoints were overall survival and progression free survival. Factors studied were histologic subtype and type of doxorubicin chemotherapy.</p><p><strong>Results: </strong>748 of 2045 (36.6%) received at least 6 cycles and did not progress during or at the end of chemotherapy. 475 of 2045 (23.2%) of patients received exactly 6 cycles and did not progress during or at the end of chemotherapy. Median progression free survival after 6 cycles of doxorubicin based chemotherapy was 4.2 months (95% confidence interval 3.7-4.8) and median overall survival 15.7 months (14.0-17.8). Median progression free survival and overall survival from randomisation/registration were 8.7 months (95% confidence interval 8.2-9.1) and 20.1 months (95% confidence interval 18.3-22.3) respectively. Significant differences in progression free survival were found between chemotherapy regimens, but not for overall survival. These data are also reported for patients receiving 7 or more cycles of chemotherapy and for patients with 3 or more cycles of chemotherapy.</p><p><strong>Conclusion: </strong>This large retrospective study is the first to report progression free survival and overall survival after completion of 6 cycles of first line doxorubicin containing chemotherapy. These results are important when designing new studies exploring for example maintenance therapy after doxorubicin based chemotherapy.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"18"},"PeriodicalIF":0.0,"publicationDate":"2020-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00137-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38392734","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}
引用次数: 8
Anthracycline-based and gemcitabine-based chemotherapy in the adjuvant setting for stage I uterine leiomyosarcoma: a retrospective analysis at two reference centers. 以蒽环类和吉西他滨为基础的化疗辅助治疗I期子宫平滑肌肉瘤:两个参考中心的回顾性分析
Clinical Sarcoma Research Pub Date : 2020-08-28 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00139-3
Giovanni Fucà, Chiara Fabbroni, Rosanna Mancari, Sara Manglaviti, Giorgio Bogani, Elena Fumagalli, Rossella Bertulli, Carlo Morosi, Paola Collini, Francesco Raspagliesi, Nicoletta Colombo, Paolo G Casali, Roberta Sanfilippo
{"title":"Anthracycline-based and gemcitabine-based chemotherapy in the adjuvant setting for stage I uterine leiomyosarcoma: a retrospective analysis at two reference centers.","authors":"Giovanni Fucà,&nbsp;Chiara Fabbroni,&nbsp;Rosanna Mancari,&nbsp;Sara Manglaviti,&nbsp;Giorgio Bogani,&nbsp;Elena Fumagalli,&nbsp;Rossella Bertulli,&nbsp;Carlo Morosi,&nbsp;Paola Collini,&nbsp;Francesco Raspagliesi,&nbsp;Nicoletta Colombo,&nbsp;Paolo G Casali,&nbsp;Roberta Sanfilippo","doi":"10.1186/s13569-020-00139-3","DOIUrl":"https://doi.org/10.1186/s13569-020-00139-3","url":null,"abstract":"<p><strong>Background: </strong>Radically resected early uterine leiomyosarcoma (eULMS) is still marked by a poor prognosis. Adjuvant strategies investigated up to now have not been corroborated by controlled studies. We retrospectively reviewed the clinical outcome of eULMS patients treated with adjuvant anthracycline-based or gemcitabine-based chemotherapy at two Italian reference centers.</p><p><strong>Methods: </strong>In this explorative, retrospective, cohort analysis, we included all the consecutive patients with radically resected eULMS treated at two centers between 1997 and 2017.</p><p><strong>Results: </strong>A total of 109 consecutive patients were included. Sixty-six (60%) received an anthracycline-based regimen, whereas 43 (40%) received a gemcitabine-based regimen. Median disease-free survival (DFS) was 41.3 months with anthracycline-based regimens compared to 20.9 months with gemcitabine-based regimens (HR: 0.49; 95% CI: 0.30-0.80; <i>P</i> = 0.004). In the multivariable model, anthracycline-based regimens were independently associated with a better DFS. No difference in terms of overall survival was observed.</p><p><strong>Conclusions: </strong>DFS was not the same by using an anthracycline-based or a gemcitabine-based adjuvant chemotherapy for patients with radically resected eULMS. The results of our study are in line with recent prospective controlled evidence in limb and superficial trunk soft tissue sarcomas. The role of anthracycline-based adjuvant chemotherapy should still be viewed as a research issue in eULMS.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"17"},"PeriodicalIF":0.0,"publicationDate":"2020-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00139-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38431591","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}
引用次数: 5
Long lasting response with trabectedin monotherapy in relapsed metastatic mesenchymal chondrosarcoma. 单药治疗复发性转移性间充质软骨肉瘤的长期疗效。
Clinical Sarcoma Research Pub Date : 2020-08-27 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00138-4
Ghazal Tansir, Sameer Rastogi, Adarsh Barwad, Ekta Dhamija
{"title":"Long lasting response with trabectedin monotherapy in relapsed metastatic mesenchymal chondrosarcoma.","authors":"Ghazal Tansir,&nbsp;Sameer Rastogi,&nbsp;Adarsh Barwad,&nbsp;Ekta Dhamija","doi":"10.1186/s13569-020-00138-4","DOIUrl":"https://doi.org/10.1186/s13569-020-00138-4","url":null,"abstract":"<p><strong>Background: </strong>Mesenchymal chondrosarcoma is an exceedingly rare malignancy, accounting for around 5% of all patients with chondrosarcoma. It is a translocation-related sarcoma that tends to have both local and distant recurrences. Surgery is the mainstay of treatment in localised cases however treatment of advanced cases remains a challenge. The rarity of the disease precludes dedicated clinical trials and hence guidelines for its management are not well defined. The dearth in literature makes it pertinent that the cases treated with newer therapies must be reported to contribute to existing knowledge.</p><p><strong>Case presentation: </strong>We hereby report a case of a 39-year old male without any comorbidity presenting with pelvic pain and was diagnosed as mesenchymal chondrosarcoma of the pelvis. He underwent an initial curative resection followed by a disease-free interval of 7 months. Subsequently, he was treated with pulmonary metastatectomy and local debulking surgery at time of initial relapse. He was then exposed to multiple lines of palliative chemotherapy, which limited our treatment options upon subsequent disease progression. Based on recent data, the patient was given trabectedin monotherapy as fourth line chemotherapy. He tolerated the therapy well and attained a progression-free survival of 12 months, which is an impactful figure in relapsed setting in this patient population.</p><p><strong>Conclusion: </strong>This report aims to present a comprehensive review into available and newer treatment choices for mesenchymal chondrosarcoma, and to highlight trabectedin monotherapy as a possible therapeutic option for mesenchymal chondrosarcoma in the relapsed setting.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"16"},"PeriodicalIF":0.0,"publicationDate":"2020-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00138-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38421926","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}
引用次数: 2
Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary. 副瘤性皮肌炎伴原发不明的转移性平滑肌肉瘤。
Clinical Sarcoma Research Pub Date : 2020-08-25 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00140-w
Eve Merry, Alannah Smrke, Kapil Halai, Gulam Patel, Khin Thway, Robin L Jones, Charlotte Benson
{"title":"Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary.","authors":"Eve Merry,&nbsp;Alannah Smrke,&nbsp;Kapil Halai,&nbsp;Gulam Patel,&nbsp;Khin Thway,&nbsp;Robin L Jones,&nbsp;Charlotte Benson","doi":"10.1186/s13569-020-00140-w","DOIUrl":"https://doi.org/10.1186/s13569-020-00140-w","url":null,"abstract":"<p><strong>Background: </strong>Sarcomas are rare and heterogeneous tumours of mesenchymal origin, with over 100 histological subtypes. Paraneoplastic dermatomyositis has rarely been described in sarcoma. This is the first documented case of paraneoplastic dermatomyositis in a patient with metastatic leiomyosarcoma.</p><p><strong>Case presentation: </strong>A 43-year-old female diagnosed with metastatic leiomyosarcoma of unknown primary presented with a mild rash in sun-exposed areas of her face and upper chest, with no other neuromuscular symptoms. This rash resolved with systemic treatment with doxorubicin for metastatic leiomyosarcoma. Imaging assessment confirmed overall stable disease after chemotherapy completion. She presented acutely 2 months later with new onset rash in a shawl-like distribution, periorbital oedema and proximal muscle weakness. Based on the characteristic cutaneous signs and symmetrical proximal muscle weakness, abnormal electromyography and raised skeletal muscle enzymes with a positive anti-transcription intermediary factor-1 gamma antibody result, a diagnosis of paraneoplastic dermatomyositis was made. Re-evaluation of her metastatic leiomyosarcoma revealed disease progression. Second-line chemotherapy was commenced once the dermatomyositis was controlled on steroid therapy. Systemic anti-cancer therapy was again associated with mild improvement in dermatomyositis symptoms.</p><p><strong>Discussion: </strong>Paraneoplastic dermatomyositis heralded disease progression after first-line chemotherapy; however, in hindsight, subtle cutaneous features were present at sarcoma diagnosis. The temporal relationship between paraneoplastic dermatomyositis and metastatic leiomyosarcoma is key in this case, as fluctuations in dermatomyositis severity correlated with growth of metastatic disease. Understanding this relationship may provide clues for tumour progression and prompt timely initiation of anti-cancer therapy. It is important to recognise that in addition to the more common cancers associated with paraneoplastic dermatomyositis, it can also occur in rarer tumours such as leiomyosarcoma.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"15"},"PeriodicalIF":0.0,"publicationDate":"2020-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00140-w","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38325855","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}
引用次数: 2
Successful treatment of lipofibromatosis-like neural tumor of the lumbar spine with an NTRK-fusion inhibitor. ntrk融合抑制剂成功治疗腰椎脂肪纤维瘤样神经肿瘤。
Clinical Sarcoma Research Pub Date : 2020-08-06 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00136-6
Megan Dupuis, Yulei Shen, Christian Curcio, Jeanne M Meis, Wei-Lien Wang, Behrang Amini, Laurence Rhines, Jacquelyn Reuther, Angshumoy Roy, Kevin E Fisher, Anthony P Conley, J Andrew Livingston
{"title":"Successful treatment of lipofibromatosis-like neural tumor of the lumbar spine with an NTRK-fusion inhibitor.","authors":"Megan Dupuis,&nbsp;Yulei Shen,&nbsp;Christian Curcio,&nbsp;Jeanne M Meis,&nbsp;Wei-Lien Wang,&nbsp;Behrang Amini,&nbsp;Laurence Rhines,&nbsp;Jacquelyn Reuther,&nbsp;Angshumoy Roy,&nbsp;Kevin E Fisher,&nbsp;Anthony P Conley,&nbsp;J Andrew Livingston","doi":"10.1186/s13569-020-00136-6","DOIUrl":"https://doi.org/10.1186/s13569-020-00136-6","url":null,"abstract":"<p><strong>Background: </strong>Lipofibromatosis-like neural tumors (LPF-NT) are a newly identified class of rare mesenchymal neoplasms. Current standard of care therapy is surgical resection alone; there are no chemotherapies or molecular targeted therapies that have been shown to be effective in patients who are not surgical candidates due to either tumor bulk or location. Most LPF-NT harbor NTRK fusions, although the therapeutic significance of these fusions has not been previously demonstrated in this malignancy. Here, we present the first case of a patient with surgically-unresectable LPF-NT successfully treated with medical therapy, specifically the TRK fusion-protein inhibitor entrectinib.</p><p><strong>Case presentation: </strong>The patient is a 21 year old man with no co-morbidities who presented for evaluation due to intermittent abdominal pain and was found to have a mass spanning from T12-L2. Biopsy revealed a mesenchymal spindle cell neoplasm and S100 positivity pointed to possible nerve sheath origin. The sample was ultimately found to have an LMNA-NTRK1 fusion, confirming the diagnosis of LP-NFT. Unfortunately, due to the bulk and location of the tumor, surgery was felt to be exceptionally morbid and the patient was treated in a clinical trial with the NTRK inhibitor entrectinib. Surprisingly, he had such a robust clinical response that he was ultimately deemed a surgical candidate and he was successfully taken to surgery. Post-operative pathology revealed > 95% necrosis, demonstrating exceptional sensitivity to the targeted therapy. The patient remains NED and on entrectinib 12 months post-operatively.</p><p><strong>Conclusions: </strong>The exceptional treatment response of this patient suggests that NTRK fusions are true drivers of the disease. Thus, all patients should be evaluated for NTRK fusions using sensitive methodologies and treatment with TRK fusion-protein inhibitors should be considered in patients who are not candidates for oncologic resection.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"14"},"PeriodicalIF":0.0,"publicationDate":"2020-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00136-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38253552","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}
引用次数: 9
Treatment of gastrointestinal tumor (GIST) of the rectum requiring abdominoperineal resection following neoadjuvant imatinib: a cost-effectiveness analysis. 治疗胃肠道肿瘤(GIST)的直肠需要腹部会阴切除后新辅助伊马替尼:成本-效果分析。
Clinical Sarcoma Research Pub Date : 2020-08-06 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00135-7
Mohamad Farid, Johnny Ong, Claramae Chia, Grace Tan, Melissa Teo, Richard Quek, Jonathan Teh, David Matchar
{"title":"Treatment of gastrointestinal tumor (GIST) of the rectum requiring abdominoperineal resection following neoadjuvant imatinib: a cost-effectiveness analysis.","authors":"Mohamad Farid,&nbsp;Johnny Ong,&nbsp;Claramae Chia,&nbsp;Grace Tan,&nbsp;Melissa Teo,&nbsp;Richard Quek,&nbsp;Jonathan Teh,&nbsp;David Matchar","doi":"10.1186/s13569-020-00135-7","DOIUrl":"https://doi.org/10.1186/s13569-020-00135-7","url":null,"abstract":"<p><strong>Background: </strong>Neoadjuvant imatinib for gastrointestinal stromal tumors (GIST) of the rectum can reduce, but may not eliminate, risk of surgical morbidity from permanent bowel diversion. We sought to evaluate the cost-effectiveness of alternative strategies in rectal GIST patients requiring abdominoperineal resection following neoadjuvant imatinib.</p><p><strong>Methods: </strong>We developed a Markov model using a healthcare payers' perspective to estimate costs in 2017 Singapore dollars (SGD) and quality adjusted life years (QALYs) for upfront abdominoperineal resection (UAPR) versus continued imatinib until progression (CIUP) following 1 year of neoadjuvant imatinib. Transition probabilities and utilities were obtained from published data, and costs were estimated using data from the National Cancer Centre Singapore. Deterministic and probabilistic sensitivity analyses were conducted to probe model uncertainty. Incremental cost-effectiveness ratio below SGD 50,000 per QALY gained was considered cost-effective.</p><p><strong>Results: </strong>In the base case, UAPR dominates CIUP being both more effective (8.66 QALYS vs 5.43 QALYs) and less expensive (SGD 312,627 vs SGD 339,011). These estimates were most sensitive to 2 variables, utility of abdominoperineal resection and annual recurrence probability post-abdominoperineal resection. However, simultaneously varying the values of these variables to maximally favor CIUP did not render it the more cost effective strategy at willingness to pay (WTP) of SGD 50,000. In probabilistic sensitivity analysis, UAPR had probability of being cost-effective compared with CIUP greater than 95%, reaching 100% at WTP SGD 10,000.</p><p><strong>Conclusion: </strong>UAPR is more effective and less costly than CIUP for patients with rectal GIST requiring abdominoperineal resection following neoadjuvant imatinib, and is the strategy of choice in this setting.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"13"},"PeriodicalIF":0.0,"publicationDate":"2020-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00135-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38253551","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}
引用次数: 3
A case report of Kaposiform haemangioendothelioma; response with propranolol and steroids. 卡泊西样血管内皮瘤1例报告服用心得安和类固醇。
Clinical Sarcoma Research Pub Date : 2020-07-30 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00134-8
Saurav Verma, Ekta Dhamija, Adarsh Barwad, Venkatesan S Kumar, Sameer Rastogi
{"title":"A case report of Kaposiform haemangioendothelioma; response with propranolol and steroids.","authors":"Saurav Verma,&nbsp;Ekta Dhamija,&nbsp;Adarsh Barwad,&nbsp;Venkatesan S Kumar,&nbsp;Sameer Rastogi","doi":"10.1186/s13569-020-00134-8","DOIUrl":"https://doi.org/10.1186/s13569-020-00134-8","url":null,"abstract":"<p><strong>Background: </strong>Kaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone. It is a locally aggressive tumor usually seen in infants. Here we report a case of kaposiform hemagioendothelioma in a child who responded to propranolol and steroids.</p><p><strong>Case presentation: </strong>A 3-year-old male child presented with a swelling below his right knee with characteristic violet skin lesion. There was no evidence of Kasabach-Merritt phenomenon. After no improvement with several attempts at debridement and anti-tubercular treatment; a diagnosis of Kaposiform Haemangioendothelioma was reached on the basis of overall clinical picture and histology. The child was treated with propranolol and steroids and had an excellent clinical response and a near complete resolution on imaging at 5 months.</p><p><strong>Conclusions: </strong>These cases are often misdiagnosed and despite a delay in diagnosis have good outcomes with appropriate multimodality management. This case highlights the unique and typical characteristics of kaposiform haemangioendothelioma.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"12"},"PeriodicalIF":0.0,"publicationDate":"2020-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00134-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38239836","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}
引用次数: 2
Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report. 派姆单抗和帕唑帕尼治疗晚期未分化多形性肉瘤的良好和持续反应:1例报告。
Clinical Sarcoma Research Pub Date : 2020-07-09 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00133-9
Shalabh Arora, Sameer Rastogi, Shamim Ahmed Shamim, Adarsh Barwad, Maansi Sethi
{"title":"Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report.","authors":"Shalabh Arora,&nbsp;Sameer Rastogi,&nbsp;Shamim Ahmed Shamim,&nbsp;Adarsh Barwad,&nbsp;Maansi Sethi","doi":"10.1186/s13569-020-00133-9","DOIUrl":"https://doi.org/10.1186/s13569-020-00133-9","url":null,"abstract":"<p><strong>Background: </strong>Conventional cytotoxic agents and pazopanib are approved for advanced soft tissue sarcomas but have low response rates and modest survival benefits. Recently, immune checkpoint inhibitors have shown clinically meaningful activity. The combination of pazopanib and immunotherapy has shown synergism in various other malignancies but has not been fully explored in advanced soft tissue sarcomas.</p><p><strong>Case presentation: </strong>A 63 year old woman with metastatic undifferentiated pleomorphic sarcoma progressed after two lines of palliative combination chemotherapy-doxorubicin with olaratumab, and gemcitabine with docetaxel. In view of significant symptoms, she was treated with pazopanib in combination with pembrolizumab. She had remarkable radiological and clinical improvement, with a manageable toxicity profile and an ongoing response at ten months of therapy.</p><p><strong>Conclusions: </strong>Undifferentiated pleomorphic sarcoma is an immunologically active subtype of soft tissue sarcoma, which is particularly amenable to immune checkpoint inhibitors. Pazopanib with immune checkpoint inhibitors is a well-tolerated, yet hitherto underexplored combination that may offer significant clinical benefit in advanced sarcomas-this finding warrants further evaluation in clinical trials.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"10"},"PeriodicalIF":0.0,"publicationDate":"2020-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00133-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38163755","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}
引用次数: 12
Chemotherapy improves distant control in localized high-grade soft tissue sarcoma of the extremity/trunk. 化疗改善了肢体/躯干局部高级别软组织肉瘤的远程控制。
Clinical Sarcoma Research Pub Date : 2020-07-09 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00132-w
Victoria T Rizk, Arash O Naghavi, Andrew S Brohl, David M Joyce, Odion Binitie, Youngchul Kim, John P Hanna, Jennifer Swank, Ricardo J Gonzalez, Damon R Reed, Mihaela Druta
{"title":"Chemotherapy improves distant control in localized high-grade soft tissue sarcoma of the extremity/trunk.","authors":"Victoria T Rizk,&nbsp;Arash O Naghavi,&nbsp;Andrew S Brohl,&nbsp;David M Joyce,&nbsp;Odion Binitie,&nbsp;Youngchul Kim,&nbsp;John P Hanna,&nbsp;Jennifer Swank,&nbsp;Ricardo J Gonzalez,&nbsp;Damon R Reed,&nbsp;Mihaela Druta","doi":"10.1186/s13569-020-00132-w","DOIUrl":"https://doi.org/10.1186/s13569-020-00132-w","url":null,"abstract":"<p><strong>Background: </strong>Soft tissue sarcomas (STS) are rare and heterogeneous tumors making chemotherapy use controversial. Our goal was to identify a subset of patients with primary STS that benefit with the addition of chemotherapy.</p><p><strong>Methods: </strong>A retrospective chart review included intermediate to high-grade localized primary STS of the extremity/trunk, and tumor size > 5 cm. The effect of chemotherapy was evaluated for local control (LC), distant control (DC), progression free survival (PFS), and overall survival (OS).</p><p><strong>Results: </strong>In this cohort (n = 273), patients were treated with surgery (98%), radiation (81%), and chemotherapy (24.5%). With a median follow-up of 51 months, the entire cohort's 5-year LC, DC, PFS, and OS are 79.1%, 59.9%, 43.8%, and 68.7%, respectively. The addition of chemotherapy did not provide a DC benefit (p = 0.238) for the entire cohort. High-grade disease (n = 210) experienced a 5-year benefit in DC (68% vs. 54.4%, p = 0.04), which was more pronounced with MAI (Mesna, Adriamycin, Ifosfamide) based regimens (74.2%, p = 0.016), and a 5-year PFS (50.8% vs 45%, p = 0.025) and OS benefit (76.2% vs 70%, p = 0.067) vs. no chemotherapy. On multivariate analysis of the high-grade subset, chemotherapy independently predicted for a DC benefit (HR 0.48 95% CI 0.26-89, p = 0.019). The benefit of chemotherapy was more pronounced with MAI, showing a significant benefit in DC (HR 0.333 95% CI 0.145-0.767, p = 0.01) and PFS (HR 0.52 95% CI 0.28-0.99, p = 0.047).</p><p><strong>Conclusion: </strong>In patients with localized STS > 5 cm, the high-grade subset had a distant control benefit with the addition of chemotherapy, leading to improved progression free survival. This is more pronounced with the use of MAI and should be considered in patients eligible for this regimen.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"11"},"PeriodicalIF":0.0,"publicationDate":"2020-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00132-w","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38163757","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}
引用次数: 2
Real-world experience with doxorubicin and olaratumab in soft tissue sarcomas in England and Northern Ireland. 阿霉素和奥拉拉单抗在英国和北爱尔兰软组织肉瘤中的实际应用经验。
Clinical Sarcoma Research Pub Date : 2020-05-06 eCollection Date: 2020-01-01 DOI: 10.1186/s13569-020-00131-x
Spyridon Gennatas, Florence Chamberlain, Thomas Carter, Susanna Slater, Elena Cojocaru, Beth Lambourn, Anna Stansfeld, Radha Todd, Mark Verrill, Nasim Ali, Robin L Jones, Peter Simmonds, Nicola Keay, Heather McCarty, Sandra Strauss, Vassilios Karavasilis, Palma Dileo, Charlotte Benson
{"title":"Real-world experience with doxorubicin and olaratumab in soft tissue sarcomas in England and Northern Ireland.","authors":"Spyridon Gennatas,&nbsp;Florence Chamberlain,&nbsp;Thomas Carter,&nbsp;Susanna Slater,&nbsp;Elena Cojocaru,&nbsp;Beth Lambourn,&nbsp;Anna Stansfeld,&nbsp;Radha Todd,&nbsp;Mark Verrill,&nbsp;Nasim Ali,&nbsp;Robin L Jones,&nbsp;Peter Simmonds,&nbsp;Nicola Keay,&nbsp;Heather McCarty,&nbsp;Sandra Strauss,&nbsp;Vassilios Karavasilis,&nbsp;Palma Dileo,&nbsp;Charlotte Benson","doi":"10.1186/s13569-020-00131-x","DOIUrl":"https://doi.org/10.1186/s13569-020-00131-x","url":null,"abstract":"<p><strong>Background: </strong>A randomised phase II trial demonstrated that the addition of olaratumab to doxorubicin significantly increased overall survival (OS) in patients with advanced soft tissue sarcomas (STS) compared to doxorubicin alone. The recently presented phase III study of doxorubicin and olaratumab in advanced soft tissue sarcoma was discordant with this finding.</p><p><strong>Methods: </strong>We performed a retrospective analysis of adult patients with advanced-/metastatic STS treated with at least two cycles of doxorubicin and olaratumab at eight sarcoma units across England and Northern Ireland between May 2017 and March 2019.</p><p><strong>Results: </strong>172 patients were evaluable and 40 patients (23.3%) had died at the time of analysis. Median ECOG performance status (PS) was 1. Median progression free survival (PFS) was 6.8 months (95% CI 5.9-7.7 months). Leiomyosarcoma was the most common histological subtype (75 patients, 43.6%), followed by liposarcomas (19, 11.0%). The mean number of cycles was 5 (doxorubicin range 2-6; olaratumab range 2-23). Two patients (1.2%) had a complete response and 34 (19.8%) had a partial response. 79 (45.9%) had stable and 58 (33.7%) progressive disease. 57 patients (33.1%) experienced grade ≥ 3 neutropenia and 7 patients (4.1%) grade ≥ 3 febrile neutropenia. Grade ≥ 3 anaemia was seen in 21 patients (12.2%). Grade ≥ 3 non-haematological toxicities were seen in 35 patients (20.3%). A clinically significant drop in left ventricular ejection fraction was seen in 6 patients (3.5%). 48 patients (27.9%) required a dose reduction. Overall survival (OS) is pending.</p><p><strong>Conclusions: </strong>Our results are in keeping with the phase III study findings: response rate, PFS and OS were similar to those reported in the phase III ANNOUNCE trial.</p>","PeriodicalId":10684,"journal":{"name":"Clinical Sarcoma Research","volume":"10 ","pages":"9"},"PeriodicalIF":0.0,"publicationDate":"2020-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13569-020-00131-x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37920562","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}
引用次数: 5
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