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

ABSTRACT:

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 e

SUBMITTER: Grothues J 

PROVIDER: S-EPMC11560989 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature