Prognostic factors in clear cell sarcoma: an analysis of soft tissue sarcoma in 43 cases.

Autor: Grothues J; Department of Musculoskeletal Oncology, Sarcoma Center, 45147, Essen, Germany.; German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany., Hardes J; Department of Musculoskeletal Oncology, Sarcoma Center, 45147, Essen, Germany.; German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany., Agaimy A; Institute of Pathology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital, Erlangen, Germany., Collaud S; Lung Clinic, Department of Thoracic Surgery, Cologne Merheim Hospital, University of Witten/Herdecke, Witten, Germany., Podleska L; Department of Musculoskeletal Oncology, Sarcoma Center, 45147, Essen, Germany.; German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany., Farzalyev F; Department of Musculoskeletal Oncology, Sarcoma Center, 45147, Essen, Germany.; German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany., Engel NM; Department of Musculoskeletal Oncology, Sarcoma Center, 45147, Essen, Germany.; German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany., Hamacher R; Department of Medical Oncology and Sarcoma Center, West German Cancer Center, Medical School, University Duisburg-Essen, Essen, Germany., Fletcher B; Department of Medical Oncology and Sarcoma Center, West German Cancer Center, Medical School, University Duisburg-Essen, Essen, Germany., Pöttgen C; Department of Radiotherapy, West German Cancer Centre, University Hospital Essen, 45147, Essen, Germany., Bertram S; Institute of Pathology, University Medical Center Essen, 45147, Essen, Germany., Schildhaus HU; Discovery Life Sciences Biomarker GmbH and Pathologie Nordhessen, Germaniastr. 7, 34119, Kassel, Germany., Streitbürger A; Department of Musculoskeletal Oncology, Sarcoma Center, 45147, Essen, Germany., Bauer S; Department of Medical Oncology and Sarcoma Center, West German Cancer Center, Medical School, University Duisburg-Essen, Essen, Germany., Falkenhorst J; Department of Medical Oncology and Sarcoma Center, West German Cancer Center, Medical School, University Duisburg-Essen, Essen, Germany. johanna.falkenhorst@ume.de.
Jazyk: angličtina
Zdroj: Journal of cancer research and clinical oncology [J Cancer Res Clin Oncol] 2024 Nov 13; Vol. 150 (11), pp. 494. Date of Electronic Publication: 2024 Nov 13.
DOI: 10.1007/s00432-024-05980-3
Abstrakt: Purpose: Clear cell sarcoma (CCS) of tendons and aponeuroses and CCS-like malignant gastrointestinal neuroectodermal tumor/sarcoma (GINET) are characterized by frequent local and distant relapses, alongside with low efficacy of all systemic treatments. We aimed to collect a comprehensive dataset to identify prognostic factors and treatment outcomes.
Methods: We performed a retrospective single center analysis for diagnosed CCS and GINET on demographic, tumor, treatment and survival data.
Results: We identified 43 patients (w:25, m:18) with a median follow-up of 35mo and a 5y-OS-rate of 42%. At diagnosis the median age was 42yrs. Median tumor size was 3.6 cm (0.3-11.1 cm), and 24/26 (94%) tissues analyzed at our institute were EWSR1::ATF1-translocation-positive. Distant extremities (incl. knee or elbow) were affected in 72.5%. Of note, 79.5% received an excisional biopsy (benign histology suspected in 30.2%) leading to frequent incomplete resection. Final R0 status correlated significantly (p = 0.017) with longer survival rates compared to R + status in localized CCS (N0M0, 5-yr OS 0% vs 64%). Radiation and systemic treatment had limited antitumor effects while isolated limb perfusion was active in some patients. 18.6% of patients showed lymphatic spread and 20.9% distant metastases. Presence of initial M + was associated with a dismal survival of 1.4 years (M +) vs 7.1 years (M0; p < .001).
Conclusion: We here present one of the largest clinical cohorts of patients with CCS/GINET. Our data underscores the exceptional risk of metastatic disease even in small tumors. As systemic treatment and radiation showed limited efficacy, complete resection was the most important treatment option.
Competing Interests: Declarations Conflict of interest No other potential conflicts of interest were reported. The authors declare no competing interests.
(© 2024. The Author(s).)
Databáze: MEDLINE
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