NCI Definition: A soft tissue tumor of uncertain lineage characterized by the presence of neoplastic spindle-shaped to round cells in a fibromyxoid stroma. Metaplastic bone formation may or may not be present. [1]

Fibromyxoid tumors most frequently harbor alterations in PTEN, WHSC1, RPTOR, RB1, and PHF1 [2].

Most Commonly Altered Genes in Fibromyxoid Tumor

PTEN Loss, WHSC1 Mutation, WHSC1 C687R, RPTOR Mutation, and RPTOR A245V are the most common alterations in fibromyxoid tumor [2].

Top Alterations in Fibromyxoid Tumor

Disease Details

Fibromyxoid Neoplasm, Fibromyxoma
Soft Tissue Tumor of Uncertain Differentiation
Nonossifying Fibromyxoid Tumor and Ossifying Fibromyxoid Tumor


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 8. 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.