Overview

NCI Definition: A malignant mesenchymal fibroblastic neoplasm affecting the soft tissue and bone. [1]

Fibrosarcomas most frequently harbor alterations in CDKN2A, TP53, CDKN2B, EP300, and NOTCH1 [2].

Most Commonly Altered Genes in Fibrosarcoma

CDKN2A Loss, CDKN2B Loss, TP53 Mutation, NOTCH1 Mutation, and NOTCH1 Missense are the most common alterations in fibrosarcoma [2].

Top Alterations in Fibrosarcoma

Significant Genes in Fibrosarcoma

MYCN +

Disease Details

Synonyms
Malignant Fibromatous Neoplasm, Fibrosarcoma (excluding infantile fibrosarcoma), FIBROSARCOMA, MALIGNANT, Fibrosarcoma - not infantile
Parent(s)
Sarcoma
Children
Soft Tissue Fibrosarcoma, Conventional Fibrosarcoma, Adult Fibrosarcoma, and Childhood Fibrosarcoma
OncoTree Name
Fibrosarcoma
OncoTree Code
FIBS

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.