Real-world outcomes of patients with locally advanced or metastatic epithelioid sarcoma

Gounder, Mrinal M.; Merriam, Priscilla; Ratan, Ravin; Patel, Shreyaskumar; Chugh, Rashmi; Villalobos, Víctor M.; Thorton, M.; Tine, Brian Andrew Van; Abdelhamid, Amr Hesham; Joshi, Prashant; Whalen, Jennifer; Yang, Jay; Rajarethinam, Anand; Duh, Mei Sheng; Bobbili, Priyanka; Cavanaugh, Cristi; Huynh, Lynn; Totev, Todor; Demetri, George D.

doi:10.1093/annonc/mdz283.020

Cited by 3 publications

(4 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The recent retrospective multicentre analysis of 74 patients by Gounder et al provided real-world data on the effectiveness of conventional chemotherapy (Gounder et al, 2021). In this real-world study, the ORR was 14.9% and 9.4% for patients who received firstand second-line treatment and beyond, respectively.…”

Section: Introductionmentioning

confidence: 82%

“…In this real-world study, the ORR was 14.9% and 9.4% for patients who received firstand second-line treatment and beyond, respectively. Of note, 51.4% of patients experienced a clinically significant adverse event during treatment (Gounder et al, 2021). This study provided a valuable benchmark regarding the activity and safety of chemotherapy schedules in advanced ES.…”

Section: Introductionmentioning

confidence: 85%

See 1 more Smart Citation

The evolving management of epithelioid sarcoma

et al. 2021

View full text Add to dashboard Cite

Section: Introductionmentioning

confidence: 82%

Section: Introductionmentioning

confidence: 85%

The evolving management of epithelioid sarcoma

et al. 2021

View full text Add to dashboard Cite

“…However, local recurrence and distant metastases remain high [27]. In the advanced setting, outcomes are poor with median progression-free survival ranging from 3 to 6 months [28,29]. INI1 (SMARCB1) is a tumor suppressor gene located on chromosome 22q11 that encodes a subunit of the SWI/SNF chromatin remodelling complex [30].…”

Section: Ezh2 Inhibitorsmentioning

confidence: 99%

Prospects for Epigenetic Targeted Therapies of Bone and Soft-Tissue Sarcomas

Wang

Elkrief

Guo

et al. 2021

Sarcoma

View full text Add to dashboard Cite

Targeted therapies have revolutionized cancer treatment. It is well established that alterations of chromatin configuration and modifications affect tumorigenesis of some, possibly most, bone and soft-tissue sarcomas. As epigenetic regulators play a major role in the development of bone and soft-tissue sarcomas, epigenetic drugs provide a novel potential avenue for rational targeted therapies for these aggressive cancers. The present review summarizes the application of epigenetic drugs for clinical utilization in bone and soft-tissue sarcomas and provides an overview of clinical trials currently evaluating epigenetic therapies in this space.

show abstract

“…For patients with unresectable or advanced ES, first-line conventional systemic therapies include anthracycline-based or gemcitabine-based regimens. Experience from clinical practice indicates that the effect of these regimens is limited, with an overall response rate (ORR) of 15-27% and median overall survival (OS) of 13-19 months (4,5). The tyrosine kinase inhibitor Pazopanib is mainly used after the failure of standard chemotherapy.…”

Section: Introductionmentioning

confidence: 99%

Response to immunotherapy in a patient with advanced epithelioid sarcoma of adrenal gland: A case report

2022

View full text Add to dashboard Cite

Epithelioid sarcoma (ES) is a rare and highly invasive soft tissue malignant tumor with uncertain histogenesis. The localization of ES in the adrenal gland is rather unusual. The present study reported a case of stage IV ES in the adrenal gland of a 28-year-old male. The tissue biopsy of adrenal and lung lesions revealed epithelioid cells. Immunohistochemistry indicated that the tumor cells were strongly positive for cytokeratin (CK)8, CK, epithelial membrane antigen (EMA), CD34 and programmed cell death protein 1 ligand (PD-L1) but negative for nuclear integrase interactor 1 expression. The next-generation sequencing technology was applied using peripheral blood, indicating a low tumor mutation burden of 4.11 mutations (Muts)/Mb and somatic mutations in SMARCB1 . After diagnosis, the patient underwent unsuccessful palliative chemotherapy and radiotherapy. However, application of immune checkpoint inhibitors (ICIs) achieved partial remission and the overall survival reached 25 months. ICI monotherapy may be a feasible treatment for patients with ES with a strong expression of PD-L1.

show abstract

Real-world outcomes of patients with locally advanced or metastatic epithelioid sarcoma

Cited by 3 publications

References 0 publications

The evolving management of epithelioid sarcoma

The evolving management of epithelioid sarcoma

Prospects for Epigenetic Targeted Therapies of Bone and Soft-Tissue Sarcomas

Response to immunotherapy in a patient with advanced epithelioid sarcoma of adrenal gland: A case report

Contact Info

Product

Resources

About