Olivia Näslund, Per Sveino Strand, Thomas Skoglund, Ole Solheim, Asgeir S Jakola
{"title":"Overview and recent advances in incidental meningioma.","authors":"Olivia Näslund, Per Sveino Strand, Thomas Skoglund, Ole Solheim, Asgeir S Jakola","doi":"10.1080/14737140.2023.2193333","DOIUrl":"https://doi.org/10.1080/14737140.2023.2193333","url":null,"abstract":"<p><strong>Introduction: </strong>Meningioma has a prevalence around 1% in the population, and with the increasing use and availability of diagnostic imaging modalities, incidental meningiomas are increasingly detected. There is no clear consensus on their management, although several guidelines suggest firsthand active monitoring if no aggravating factors emerge. However, no collective guidelines on follow-up interval exist.</p><p><strong>Areas covered: </strong>This narrative review covers the epidemiology, diagnosis, growth prediction, and management strategies of incidental meningioma.</p><p><strong>Expert opinion: </strong>Overdiagnosis and excessive follow-up are potential pitfalls in the management of incidental meningioma. An MRI after 6-12 months could be reasonable to rule out rapid growth and differential diagnoses. Using the available prognostic models, one might later suggest more active monitoring for certain patient groups harboring specific radiological features predictive of growth. However, detecting growth may not necessarily be clinically significant as all larger non-growing meningiomas have at one point been small. Too much follow-up may place an unnecessary burden on patients and the health-care system and could be a driver toward overtreatment. It must be contemplated whether growth is an acceptable primary outcome measure or if there are other factors more relevant to consider in this often benign tumor entity.</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 4","pages":"397-406"},"PeriodicalIF":3.3,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9285552","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rui Zhang, Samantha D Clark, Beibei Guo, Tianyi Zhang, Duane Jeansonne, Samithamby J Jeyaseelan, Joseph Francis, Weishan Huang
{"title":"Challenges in the combination of radiotherapy and immunotherapy for breast cancer.","authors":"Rui Zhang, Samantha D Clark, Beibei Guo, Tianyi Zhang, Duane Jeansonne, Samithamby J Jeyaseelan, Joseph Francis, Weishan Huang","doi":"10.1080/14737140.2023.2188196","DOIUrl":"10.1080/14737140.2023.2188196","url":null,"abstract":"<p><strong>Introduction: </strong>Immunotherapy (IT) is showing promise in the treatment of breast cancer, but IT alone only benefits a minority of patients. Radiotherapy (RT) is usually included in the standard of care for breast cancer patients and is traditionally considered as a local form of treatment. The emerging knowledge of RT-induced systemic immune response, and the observation that the rare abscopal effect of RT on distant cancer metastases can be augmented by IT, have increased the enthusiasm for combinatorial immunoradiotherapy (IRT) for breast cancer patients. However, IRT largely follows the traditional sole RT and IT protocols and does not consider patient specificity, although patients' responses to treatment remain heterogeneous.</p><p><strong>Areas covered: </strong>This review discusses the rationale of IRT for breast cancer, the current knowledge, challenges, and future directions.</p><p><strong>Expert opinion: </strong>The synergy between RT and the immune system has been observed but not well understood at the basic level. The optimal dosages, timing, target, and impact of biomarkers are largely unknown. There is an urgent need to design efficacious pre-clinical and clinical trials to optimize IRT for cancer patients, maximize the synergy of radiation and immune response, and explore the abscopal effect in depth, taking into account patients' personal features.</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 4","pages":"375-383"},"PeriodicalIF":3.3,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10929662/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9292750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Caroline J Violette, Ravi Agarwal, Rachel S Mandelbaum, José L González, Kurt M Hong, Lynda D Roman, Maximilan Klar, Jason D Wright, Richard J Paulson, Andreas Obermair, Koji Matsuo
{"title":"The potential role of GLP-1 receptor agonist targeting in fertility-sparing treatment in obese patients with endometrial malignant pathology: a call for research.","authors":"Caroline J Violette, Ravi Agarwal, Rachel S Mandelbaum, José L González, Kurt M Hong, Lynda D Roman, Maximilan Klar, Jason D Wright, Richard J Paulson, Andreas Obermair, Koji Matsuo","doi":"10.1080/14737140.2023.2194636","DOIUrl":"https://doi.org/10.1080/14737140.2023.2194636","url":null,"abstract":"<p><strong>Introduction: </strong>Most patients diagnosed with endometrial hyperplasia or cancer are obese. Obesity, along with polycystic ovarian syndrome (PCOS) and type-2 diabetes mellitus (T2DM), may act synergistically to increase risk of malignant endometrial pathology. Incidence of malignant endometrial pathology is increasing, particularly in reproductive aged women. In patients who desire future fertility, the levonorgestrel intrauterine device (LNG-IUD) is often utilized. If the first-line progestin therapy fails, there is not an effective second-line adjunct option. Moreover, pregnancy rates following fertility-sparing treatment are lower-than-expected in these patients.</p><p><strong>Areas covered: </strong>This clinical opinion provides a summary of recent studies exploring risk factors for the development of malignant endometrial pathology including obesity, PCOS, and T2DM. Studies assessing efficacy of fertility-sparing treatment of malignant endometrial pathology are reviewed, and a potential new adjunct treatment approach to LNG-IUD is explored.</p><p><strong>Expert opinion: </strong>There is an unmet-need for a personalized treatment approach in cases of first-line progestin treatment failure. Glucagon-like peptide 1 receptor agonists are a class of anti-diabetic agents, but may have a role in fertility-sparing treatment of obese patients with malignant endometrial pathology by reducing weight, decreasing inflammation, and decreasing insulin resistance; these changes may also improve chances of subsequent pregnancy. This hypothesis warrants further exploration.</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 4","pages":"385-395"},"PeriodicalIF":3.3,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9345350","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Timing of CAR-T therapy in follicular lymphoma.","authors":"Muhamad Alhaj Moustafa","doi":"10.1080/14737140.2023.2185222","DOIUrl":"https://doi.org/10.1080/14737140.2023.2185222","url":null,"abstract":"Low-grade follicular lymphoma (FL) is an indolent malignancy that is characterized by a relapsing and remitting course with multiple lines of therapy needed to control the disease over time [1]. In recent years, various therapeutic options have been approved by the US Food and Drug Administration (FDA) for the treatment of FL. Sequencing therapies to maximize the benefit and minimize the risks and side effects in FL is becoming more challenging with the expansion of the therapeutic armamentarium [2]. This is particularly an issue in the relapsed and refractory (R/R) setting where treatment options have wide ranges of efficacy and toxicity profiles [2]. Also, in some cases, asymptomatic relapses can be observed without the need for immediate therapeutic intervention. Thus, the timing and order of treatments for FL vary between individual patients. There are nine classes of therapeutic interventions that are currently available for the treatment of FL. These include antiCD20 monoclonal antibodies, chemotherapeutic agents, immunomodulatory agents, radioimmunotherapy, EZH2 inhibitors, PI3K inhibitors, high-dose chemotherapy followed by autologous stem cell rescue (ASCT), allogeneic hematopoietic cell transplantation, and anti-CD19 chimeric antigen receptor T-cells (CAR-T) therapy [2–11]. In addition, a new class of antibodies that has dual specificity (known as bispecific antibodies) is currently being evaluated [12]. The purpose of this editorial is to discuss the optimal timing for the use of available CAR-T products in R/R FL. There are two anti-CD19 CAR-T therapies with FDA approval in R/R FL after two or more lines of systemic therapy. Axicabtagene ciloleucel (Yescarta) which was approved on 5 March 2021, for the treatment of adult patients with R/R FL based on the phase 2 ZUMA-5 clinical trial. Tisagenlecleucel (Kymriah) is the second product that was approved on 27 May 2022, for the same indication based on the phase 2 ELARA clinical trial [10,11]. The ZUMA-5 clinical trial enrolled patients with FL and marginal zone lymphoma (MZL) who had received at least two lines of prior therapy, which had to include an antiCD20 monoclonal antibody combined with an alkylating agent. Patients were heavily pretreated, with a median of three prior lines of therapy. Enrolled patients were relatively young, with a median age of 60 years. Bulky disease was seen in 52% of the patients. Disease progression within 24 months of receiving initial chemoimmunotherapy (POD24) was seen in 55% of patient with FL included in the study [10]. POD24 is a strong indicator of shortened survival [13,14]. Among patients with FL, the objective response rate (ORR) was at 94%, with 80% achieving complete response (CR). The ORR and CR rates are among the highest reported to date in such a heavily pretreated population of R/R FL. The follow-up was 17.5 months, which is relatively short for a study of FL. Thus, the overall survival (OS) benefit is immature to evaluate. Median progress","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 4","pages":"347-349"},"PeriodicalIF":3.3,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9297390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Craig W Freyer, Mitchell E Hughes, Alison Carulli, Adam Bagg, Elizabeth Hexner
{"title":"Pemigatinib for the treatment of myeloid/lymphoid neoplasms with <i>FGFR1</i> rearrangement.","authors":"Craig W Freyer, Mitchell E Hughes, Alison Carulli, Adam Bagg, Elizabeth Hexner","doi":"10.1080/14737140.2023.2192930","DOIUrl":"https://doi.org/10.1080/14737140.2023.2192930","url":null,"abstract":"<p><strong>Introduction: </strong>Myeloid/lymphoid neoplasms with fibroblast growth factor receptor-1 (<i>FGFR1</i>) rearrangements (MLN<sup>FGFR1</sup>) are rare entities with aggressive features and poor prognosis. Presentation is heterogeneous, ranging from myeloproliferative neoplasms (with or without eosinophilia) to T-cell lymphoma and acute leukemia. Historical treatments have been guided by the presenting phenotype with induction chemotherapy frequently used. Pemigatinib is a FGFR1-3 tyrosine kinase inhibitor that has demonstrated high complete hematologic and cytogenetic response rates in MLN<sup>FGFR1</sup>.</p><p><strong>Areas covered: </strong>We discuss the pathogenesis, presentation, and historical treatments for MLN<sup>FGFR1</sup>, in addition to clinical data using pemigatinib and other targeted therapies. Discussion of the mechanism of action and adverse events is also included.</p><p><strong>Expert opinion: </strong>Pemigatinib represents a significant advance in the management of MLN<sup>FGFR1</sup>. High rates of complete hematologic and cytogenetic response have been observed. While direct comparative data are unavailable, outcomes appear favorable compared to conventional approaches. Long-term efficacy and tolerability are not yet known, and allogeneic hematopoietic stem cell transplant (alloHSCT) continues to be the treatment with the highest chance of long-term disease free survival in responding patients. Combinations of pemigatinib and chemotherapy, particularly for more aggressive phenotypes, warrant future investigation as does the use of pemigatinib maintenance following alloHSCT.</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 4","pages":"351-359"},"PeriodicalIF":3.3,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9646900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Wei Wang, Jianping Zhang, Jumei Cai, Xinmin Zhao, Fazhi Wang
{"title":"Transanal drainage tube for the prevention of anastomotic leakage in anterior resection for rectal cancer: a systematic review and meta-analysis.","authors":"Wei Wang, Jianping Zhang, Jumei Cai, Xinmin Zhao, Fazhi Wang","doi":"10.1080/14737140.2023.2179991","DOIUrl":"https://doi.org/10.1080/14737140.2023.2179991","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the ability of the transanal drainage tube (TDT) to prevent anastomotic leakage (AL) and provide clinicians with the latest evidence in this area.</p><p><strong>Materials and methods: </strong>We search for relevant studies according to a search strategy. Data extracted from the study were analyzed using RevMan 5.4 software.</p><p><strong>Results: </strong>Fourteen eligible studies were included in our meta-analysis. The results of this meta-analysis suggest that patients with TDT placement have a lower incidence of AL than those without TDT placement (6% vs. 9.1%) (RR = 0.58, 95% CI: 0.46, 0.73, <i>P</i> < 0.00001). However, pooled results from RCTs suggest that TDT does not appear to reduce the incidence of AL in patients (6.4% vs. 8%) (RR = 0.79, 95% CI 0.52, 1.18, <i>P</i> = 0.24). In addition, the results of the meta-analysis suggest that TDT appears to reduce patient reoperation rates (2.6% vs. 5.8%) (RR = 0.38, 95% CI 0.27, 0.54, <i>P</i> < 0.00001).</p><p><strong>Conclusions: </strong>The results of the RCTs suggest that TDT placement does not reduce the AL rate in patients, however, it is undeniable that the placement of TDT does provide patients with some clinical benefits (such as reduced reoperation rates).</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 4","pages":"431-442"},"PeriodicalIF":3.3,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9292311","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ibrahim Elmakaty, Basel Elsayed, Mohamed Elmarasi, Omar Kujan, Mohammed Imad Malki
{"title":"Clinicopathological and prognostic value of chemokine receptor CCR7 expression in head and neck squamous cell carcinoma: a systematic review and meta-analysis.","authors":"Ibrahim Elmakaty, Basel Elsayed, Mohamed Elmarasi, Omar Kujan, Mohammed Imad Malki","doi":"10.1080/14737140.2023.2177156","DOIUrl":"https://doi.org/10.1080/14737140.2023.2177156","url":null,"abstract":"<p><strong>Objectives: </strong>This study aimed to identify the clinicopathological characteristics and prognostic value of CC chemokine receptor 7 (CCR7) expression in patients with head and neck squamous cell carcinoma (HNSSC).</p><p><strong>Methods: </strong>The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed in this meta-analysis. Up to the 2<sup>nd</sup> of July 2022 a search was conducted using five databases: PubMed, Embase, Scopus, ProQuest, and Web of Science. The methodological standards for the epidemiological research scale were used to assess the quality of the included articles, and Stata software was used to synthesize the meta-analysis.</p><p><strong>Results: </strong>We considered 13 of the 615 studies which included 1005 HNSCC patients. High expression of CCR7 increased the pooled odds ratio (OR) of advanced stage, tumor size, metastasis and recurrence by 2.82 [95% confidence interval (CI) 1.84 to 4.33], 2.48 (95% CI 1.68, to 3.67), 3.57, 95% CI 2.25 to 5.05) and 3.93 (95% CI 2.03 to 7.64), respectively. High CCR7 reduced overall patient survival [hazard ratio 2.62 (95% CI 1.59 to 4.32)].</p><p><strong>Conclusion: </strong>This study showed that high expression of CCR7 in HNSCC tumors was significantly associated with worse clinicopathological and survival outcomes, suggesting that CCR7 and its pathway could be potential therapeutic strategies for HNSCC.</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 4","pages":"443-453"},"PeriodicalIF":3.3,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9344812","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alessandra Fozza, Fiorenza De Rose, Maria Carmen De Santis, Icro Meattini, Bruno Meduri, Elisa D'angelo, Damiano Dei, Vanessa Figlia, Eliana La Rocca, Piero Fregatti, Camilla Satragno, Liliana Belgioia, Niccolò Giaj-Levra
{"title":"Technological advancements and future perspectives in breast cancer radiation therapy.","authors":"Alessandra Fozza, Fiorenza De Rose, Maria Carmen De Santis, Icro Meattini, Bruno Meduri, Elisa D'angelo, Damiano Dei, Vanessa Figlia, Eliana La Rocca, Piero Fregatti, Camilla Satragno, Liliana Belgioia, Niccolò Giaj-Levra","doi":"10.1080/14737140.2023.2195167","DOIUrl":"https://doi.org/10.1080/14737140.2023.2195167","url":null,"abstract":"<p><strong>Introduction: </strong>Breast cancer is still one of the most common tumors worldwide and radiation therapy has a central role in the oncological pathway. Several technological options are now available with the aim to improve therapeutic index, target definition, and patient selection.</p><p><strong>Areas covered: </strong>In this review, we summarize current available technologies in the management of breast cancer. These advances can support the prescription of postoperative partial breast cancer treatment and preoperative stereotactic partial breast irradiation. Moreover, image-guided radiotherapy is crucial for high-quality radiation treatments. Additionally, the recent development of hybrid magnetic resonance linear accelerator can impact target volume outline procedure, adaptive planning and radiomics. Finally, artificial intelligence represents the new frontier in medicine, supporting clinicians in target definition, patient selection, and treatment planning.</p><p><strong>Expert opinion: </strong>In patients with breast cancer the overall level of evidence about new technologies is still low even if some advances are potentially very interesting to further development.</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 4","pages":"407-419"},"PeriodicalIF":3.3,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9661327","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"TKIs in combination with immunotherapy for hepatocellular carcinoma.","authors":"Bernardo Stefanini, Luca Ielasi, Rusi Chen, Chiara Abbati, Matteo Tonnini, Francesco Tovoli, Alessandro Granito","doi":"10.1080/14737140.2023.2181162","DOIUrl":"https://doi.org/10.1080/14737140.2023.2181162","url":null,"abstract":"<p><strong>Introduction: </strong>The treatment landscape of hepatocellular carcinoma (HCC) has significantly changed over the last 5 years with multiple options in the frontline, second line, and beyond. Tyrosine kinase inhibitors (TKIs) were the first approved systemic treatments for the advanced stage of HCC; however, thanks to the increasing knowledge and characterization of the immunological features of the tumor microenvironment, the systemic treatment of HCC has been further expanded with the immune checkpoint inhibitor (ICI) approach and the following evidence of the higher efficacy obtained with combined treatment with atezolizumab plus bevacizumab over sorafenib.</p><p><strong>Areas covered: </strong>In this review, we look at rationale, efficacy, and safety profiles of current and emerging ICI/TKI combination treatments and discuss the available results from other clinical trials using similar combinatorial therapeutic approaches.</p><p><strong>Expert opinion: </strong>Angiogenesis and immune evasion are the two key pathogenic hallmarks of HCC. While the pioneering regimen of atezolizumab/bevacizumab is consolidating as the first-line treatment of advanced HCC, it will be essential, in the near future, to determine the best second-line treatment options and how to optimize the selection of the most effective therapies. These points still need to be addressed by future studies that are largely warranted to enhance the treatment's effectiveness and ultimately to tackle down HCC lethality.</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 3","pages":"279-291"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9541416","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Laureline Wetterwald, Nicolò Riggi, Anastasios Kyriazoglou, Giovanni Dei Tos, Angelo Dei Tos, Antonia Digklia
{"title":"Clear cell sarcoma: state-of-the art and perspectives.","authors":"Laureline Wetterwald, Nicolò Riggi, Anastasios Kyriazoglou, Giovanni Dei Tos, Angelo Dei Tos, Antonia Digklia","doi":"10.1080/14737140.2023.2183846","DOIUrl":"https://doi.org/10.1080/14737140.2023.2183846","url":null,"abstract":"<p><strong>Introduction: </strong>Clear cell sarcoma (CCS) is an ultrarare soft tissue sarcoma (STS) with a poor prognosis due to its propensity to metastasize and its low chemosensitivity. The standard treatment of localized CCS consists of wide surgical excision with or without additive radiotherapy. However, unresectable CCS is generally treated with conventional systemic therapies available for treatment of STS despite the weak scientific evidence to support its use.</p><p><strong>Areas covered: </strong>In this review, we discuss the clinicopathologic characteristics of CSS, as well as the current treatment landscape and future therapeutic approaches.</p><p><strong>Expert opinion: </strong>The current treatment strategy of advanced CCSs, based on STSs regimens, shows a lack of effective options. Combination therapiesin particular, the association of immunotherapy and TKIs, represent a promising approach. Translational studies are needed in order to decipher the regulatory mechanisms involved in the oncogenesis of this ultrarare sarcoma and identify potential molecular targets.</p>","PeriodicalId":12099,"journal":{"name":"Expert Review of Anticancer Therapy","volume":"23 3","pages":"235-242"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9541432","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}