Fibrosarcoma (FIBS)

Overview

Fibrosarcoma is a malignant tumor of fibroblasts, classified under Soft Tissue Sarcoma in OncoTree (parent: SOFT_TISSUE). Adult fibrosarcoma is a diagnosis of exclusion after other spindle-cell sarcomas have been ruled out. Infantile fibrosarcoma (IFS) is a distinct entity driven by ETV6–NTRK3 fusion.

Cohorts in the corpus

• sarcoma_ucla_2024: represented in the 24-subtype UCLA sarcoma PDTO biobank (194 specimens from 126 patients, Feb 2018–May 2022). PMID:39305899

Recurrent alterations

• No specific alterations directly genotyped in the UCLA PDTO study for adult fibrosarcoma.

Subtypes

• Adult fibrosarcoma (FIBS)
• Infantile fibrosarcoma (IFS) — distinct entity with ETV6–NTRK3 fusion

Therapeutic landscape

• Patient-derived tumor organoid (PDTO) drug screening was applied across all 24 sarcoma subtypes in the UCLA cohort, including FIBS. Across the full cohort, 80.4% of specimens screened had at least one significant drug response, and 59% had at least one FDA-approved or NCCN-recommended top-five regimen. PMID:39305899

Sources

• PMID:39305899 — Al Shihabi et al. Cell Stem Cell 2024. UCLA sarcoma PDTO drug-sensitivity landscape.

This page was processed by crosslinker on 2026-05-04.