Metastatic Myxofibrosarcoma with Durable Response to Temozolomide Followed by Atezolizumab: A Case Report

Lambden, Jason; Kelsten, Max F.; Schulte, Brian; Abbinanti, Susan E.; Hayes, John P.; Villaflor, Victoria M.; Agulnik, Mark

doi:10.1002/onco.13728

Cited by 9 publications

(9 citation statements)

References 25 publications

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“…Yu et al [ 137 ] reported a major response to pembrolizumab in an adult patient with an undifferentiated embryonal sarcoma of the liver. In metastatic myxofibrosarcoma, partial responses have been reported with pembrolizumab [ 138 ] and atezolizumab plus temozolomide [ 139 ]. These data, together with the data from AcSé trial, suggest that ICIs may be useful for certain subtypes of rare sarcoma, especially when selected by immune biomarkers such as the PDL1 expression.…”

Section: Immunotherapy For Sarcoma: Clinical Resultsmentioning

confidence: 99%

Current Landscape of Immunotherapy for Advanced Sarcoma

Albarrán

Villamayor

Pozas

et al. 2023

Cancers

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There is substantial heterogeneity between different subtypes of sarcoma regarding their biological behavior and microenvironment, which impacts their responsiveness to immunotherapy. Alveolar soft-part sarcoma, synovial sarcoma and undifferentiated pleomorphic sarcoma show higher immunogenicity and better responses to checkpoint inhibitors. Combination strategies adding immunotherapy to chemotherapy and/or tyrosine–kinase inhibitors globally seem superior to single-agent schemes. Therapeutic vaccines and different forms of adoptive cell therapy, mainly engineered TCRs, CAR-T cells and TIL therapy, are emerging as new forms of immunotherapy for advanced solid tumors. Tumor lymphocytic infiltration and other prognostic and predictive biomarkers are under research.

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Section: Immunotherapy For Sarcoma: Clinical Resultsmentioning

confidence: 99%

Current Landscape of Immunotherapy for Advanced Sarcoma

Albarrán

Villamayor

Pozas

et al. 2023

Cancers

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“…A twosided p-value < 0.05 was considered statistically significant. Statistical analyses were performed using SPSS 28.0 and SAS 9.4. patients had received a median RT dose for the IT of 57 Gy (45-66), in a median of 30 fractions (23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33). Roughly 62% developed OST in the head and neck (n = 10) (p ⩽ 0.001), and most had high-grade tumours (93%, n = 13/14).…”

Section: Discussionmentioning

confidence: 99%

Clinico-demographic characteristics and outcomes of radiation-induced sarcomas (RIS): a CanSaRCC study

Ribeiro,

Peretz Soroka,

Bhura

et al. 2023

Ther Adv Med Oncol

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Background: Radiation-induced sarcomas (RIS) tend to have aggressive behaviour and because of their rarity, the most appropriate management for these malignancies is uncertain. Objectives: Using the Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) database, a national sarcoma registry, we aimed to investigate prognostic factors and outcomes for RIS. Design: Retrospective study of RIS patients treated from 1996 to 2021 at three Canadian centres. Methods: RIS was defined as a sarcoma arising in a previously irradiated field following a 3+ year latency period, whose histology was distinct from the initially irradiated tumour. Clinicopathologic and treatment-related information was extracted from the CanSaRCC database. Overall survival (OS) was defined as the time from RIS diagnosis to death from any cause. Response rate (RR) to neoadjuvant chemotherapy (NACT) was based on physician assessment. Time-to-event analyses were estimated using the Kaplan–Meier method, with Cox regression for multivariate analysis. We considered a two-tailed p-value of <0.05 as statistically significant. Results: One hundred seven tumours met the criteria for RIS and were divided into three subgroups: breast angiosarcoma (BAS, n = 54), osteosarcoma (OST, n = 16), and other soft-tissue sarcomas (STS, n = 37). Patients were mostly female ( n = 85, 79%), treated initially for breast carcinomas ( n = 54, 50.5%), and diagnosed with high-grade tumours ( n = 61/71, 86%). None had evidence of synchronous metastasis. Patients with OST were younger (median age: 48 years, p < 0.001), and BAS had the shortest latency interval (8 versus 18 years for OST/STS, p < 0.001). Most patients underwent surgery, 76% ( n = 76/100) R0; 24% ( n = 26) received radiation therapy, mostly ( n = 15, 57.7%) neoadjuvant. Among those receiving chemotherapy, 30 (75%) underwent NACT; among patients with documented response assessment, the RR was 68% ( n = 17/25), being even higher in the BAS population (89.5%, n = 13/17). Median OS was 53 months (95% CI 34–101), with a 5-year OS of 47.6%; larger tumour size, high histologic grade and older age were independent prognostic factors for worse OS. Conclusion: Surgery is standard, and NACT might be useful to downsize large lesions, especially in BAS patients. Raising RIS awareness is fundamental to promoting appropriate management and fostering research through multi-institutional collaborations.

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“…Myxofibrosarcoma (MFS) expresses high levels of immune microenvironment markers, and some case reports support PD-1 inhibition in myxofibrosarcoma, which is further explored in a Phase II trial (ENVASARC, NCT04480502) (20)(21)(22)(23).…”

Section: Immune Checkpoint Inhibitorsmentioning

confidence: 99%

New therapeutics for soft tissue sarcomas: Overview of current immunotherapy and future directions of soft tissue sarcomas

Seong

D’Angelo

2023

Front. Oncol.

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Soft tissue sarcoma is a rare and aggressive disease with a 40 to 50% metastasis rate. The limited efficacy of traditional approaches with surgery, radiation, and chemotherapy has prompted research in novel immunotherapy for soft tissue sarcoma. Immune checkpoint inhibitors such as anti-CTLA-4 and PD-1 therapies in STS have demonstrated histologic-specific responses. Some combinations of immunotherapy with chemotherapy, TKI, and radiation were effective. STS is considered a ‘cold’, non-inflamed tumor. Adoptive cell therapies are actively investigated in STS to enhance immune response. Genetically modified T-cell receptor therapy targeting cancer testis antigens such as NY-ESO-1 and MAGE-A4 demonstrated durable responses, especially in synovial sarcoma. Two early HER2-CAR T-cell trials have achieved stable disease in some patients. In the future, CAR-T cell therapies will find more specific targets in STS with a reliable response. Early recognition of T-cell induced cytokine release syndrome is crucial, which can be alleviated by immunosuppression such as steroids. Further understanding of the immune subtypes and biomarkers will promote the advancement of soft tissue sarcoma treatment.

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Metastatic Myxofibrosarcoma with Durable Response to Temozolomide Followed by Atezolizumab: A Case Report

Cited by 9 publications

References 25 publications

Current Landscape of Immunotherapy for Advanced Sarcoma

Current Landscape of Immunotherapy for Advanced Sarcoma

Clinico-demographic characteristics and outcomes of radiation-induced sarcomas (RIS): a CanSaRCC study

New therapeutics for soft tissue sarcomas: Overview of current immunotherapy and future directions of soft tissue sarcomas

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