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Solitary Fibrous Tumor

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Overview

NCI Definition: A localized neoplasm of probable fibroblastic derivation. It is characterized by the presence of round to spindle-shaped cells, hylanized stroma formation, thin-walled branching blood vessels, and thin bands of collagen. [1]

Solitary fibrous tumors most frequently harbor alterations in NAB2, STAT6, TP53, BCOR, and SETD2 [2].

Most Commonly Altered Genes in Solitary Fibrous Tumor

NAB2-STAT6 Fusion, STAT6-NAB2 Fusion, TP53 Mutation, TP53 c.217-c.1178 Missense, and TP53 Missense are the most common alterations in solitary fibrous tumor [2].

Top Alterations in Solitary Fibrous Tumor

Clinical Trials

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Disease Details

Synonyms
Localized Fibrous Mesothelioma, Soft tissue solitary fibrous tumor, Submesothelial Fibroma, Localized Fibrous Tumor, Hemangiopericytoma, SFT
Parent(s)
Fibroblastic Neoplasm
Children
Liver Solitary Fibrous Tumor, Peritoneal Solitary Fibrous Tumor, Malignant Solitary Fibrous Tumor, Pleural Solitary Fibrous Tumor, and Central Nervous System Solitary Fibrous Tumor/Hemangiopericytoma
OncoTree Name
Solitary Fibrous Tumor/Hemangiopericytoma
OncoTree Code
SFT

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 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.