# Bromodomain and extra-terminal motif (BET) inhibitors in Solitary fibrous tumor (SFT)

> **NIH CA R01** · UNIVERSITY OF TEXAS DALLAS · 2026 · $399,996

## Abstract

Project Summary
 Solitary fibrous tumor (SFT), historically also called hemangiopericytoma (HPC), is a soft-tissue sarcoma
occurring in adults and infants. This nonhereditary cancer is a result of an environmental intrachromosomal
gene fusion between NAB2 and STAT6 on chromosome 12, which fuses the activation domain of STAT6 with
the repression domain of NAB2. Consequently, instead of NAB2 repressing early growth response (EGR-1)
target genes, the fusion protein activates them leading to tumorigenesis. There are at least 6 distinct fusion
variants that account for pathologic variation and tumor aggressiveness in SFTs. Anatomically, these blood
vessel-derived tumors can occur anywhere; however, most occur within the meninges of the head. Intracranial
SFTs have a high rate of local recurrence (65%), metastases (33%) and overall survival less than 10 years.
Either surgery or radiation is the first line of treatment against this cancer, however for many this becomes
challenging as the cancer can travel to inoperable areas or reoccur in locations already irradiated.
 Currently there is no approved chemotherapy regimen for SFTs. Anti-angiogenic drugs developed to
treat other cancers have been used on SFTs with limited success. None of the chemotherapies enables
complete remission, with the best response being a partial response or stable disease for several months. The
average survival of patients on the current chemotherapies is 2 years.
 Using the CRISPR-based genome editing, we established SFT cell models which closely match the
endogenous NAB2-STAT6 genetic characteristics. In addition, we prepared several primary SFT cell lines and
a PDX animal model from resected patient tissue samples that faithfully recapitulates phenotypes of malignant
SFTs. Using these SFT models, we performed a high-throughput assay, which identified the BET inhibitor
Mivebresib as a promising candidate against SFTs. Here, we propose the following three aims: (a) perform
comprehensive phenotypi

## Key facts

- **NIH application ID:** 11306032
- **Project number:** 5R01CA283330-03
- **Recipient organization:** UNIVERSITY OF TEXAS DALLAS
- **Principal Investigator:** Leonidas  Bleris; JAVIER  MARTIN-BROTO
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** CA
- **Fiscal year:** 2026
- **Award amount:** $399,996
- **Award type:** 5
- **Project period:** 2024-04-01T00:00:00 → 2029-03-31T00:00:00

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/11306032

## Citation

> US National Institutes of Health, RePORTER application 11306032, Bromodomain and extra-terminal motif (BET) inhibitors in Solitary fibrous tumor (SFT) (5R01CA283330-03). Retrieved via AI Analytics 2026-05-20 from https://api.ai-analytics.org/grant/nih/11306032. Licensed CC0.

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