ArticleJNCI cancer spectrum2026
Treatment patterns and outcomes in advanced clear cell sarcoma and gastrointestinal neuroectodermal tumors.
Article in JNCI cancer spectrum, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundClear cell sarcoma and gastrointestinal neuroectodermal tumor are ultra-rare soft tissue sarcomas with no established standard of care for advanced disease. Data evaluating outcomes of systemic therapy remain limited. We aimed to evaluate systemic therapy in patients with metastatic clear cell sarcoma and gastrointestinal neuroectodermal tumor treated at a tertiary referral institution.
methodsThis is a retrospective, single-institution study of patients with metastatic clear cell sarcoma and gastrointestinal neuroectodermal tumor diagnosed between January 2000 and May 2025. The primary objective was to describe patterns of systemic therapy use, and secondary endpoints included overall survival (OS) and real-world progression-free survival (PFS). Survival outcomes were estimated using the Kaplan-Meier method. Hazard ratios were estimated using Cox proportional hazards models. Exploratory analyses evaluated outcomes by treatment class.
resultsA total of 66 patients were included; 55 received systemic therapy. Median follow-up was 192 months, and patients received a median of 2 treatment lines with heterogeneous sequencing. Median OS was 18 months (95% CI = 13.6 to 28.0). In first-line analyses, tyrosine kinase inhibitor-based therapy and immune checkpoint inhibitors were associated with improved OS compared with doxorubicin-based chemotherapy (tyrosine kinase inhibitor: hazard ratio [HR] = 0.33, 95% CI = 0.14 to 0.79; P = .01; immune checkpoint inhibitor: HR = 0.39, 95% CI = 0.10 to 0.94; P = .04). Median first-line real-world PFS was 3.8 months (95% CI = 2.9 to 6.3), with no statistically significant differences between treatment classes. Nondoxorubicin-based chemotherapy was associated with shorter real-world PFS compared with doxorubicin regimens (HR = 4.1, 95% CI = 1.71 to 9.83; P < .001).
conclusionsMetastatic clear cell sarcoma and gastrointestinal neuroectodermal tumor is highly aggressive with limited effective systemic options. Outcomes remain poor, highlighting the need for novel therapies and collaborative studies.
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