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A low malignant potential soft tissue neoplasm of uncertain differentiation. It typically affects young patients, presenting as a slowly growing nodular or cystic tumor mass, most often in the subcutaneous tissues of the extremities. Occasionally, patients have systemic symptoms (anemia, fever, and weight loss). This tumor has a relatively good prognosis. A minority of patients develop local recurrences. Metastases are rare.
Features include: Histiocytoma.
CREB1 encodes cAMP responsive element binding protein 1 (327 aa). Phosphorylation-dependent transcription factor that stimulates transcription upon binding to the DNA cAMP response element (CRE), a sequence present in many viral and cellular promoters. Highest expression in Cells EBV-transformed lymphocytes (20.6 TPM) and Cervix Ectocervix (18.2 TPM).
Histiocytoma, Angiomatoid fibrous is associated with mutations in the CREB1 gene on chromosome 2.
The CREB1 protein participates in CREB1 gene expression is stimulated by MECP2 pathway.
CREB1 is classified as a druggable target (Clinically Actionable, Kinase, and Transcription Factor categories) with score 4.7.
Genetic testing for CREB1 is available. Testing is considered confirmatory for diagnosis.
Biomarker and diagnostic research for histiocytoma, Angiomatoid fibrous has been reported in the published literature.
Estimated prevalence: Unknown (Unknown prevalence).
2 clinical trials registered, 1 recruiting. Interventions under study include drug therapy and procedural interventions. Pipeline includes 1 PHASE2, 1 PHASE1. Research is primarily sponsored by academic and government institutions.
45 publications have been identified in PubMed for histiocytoma, Angiomatoid fibrous. Research spans Case Report / Case Series (60%), Review / Meta-Analysis (22%), and Basic Science / Preclinical (9%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 27 |
Data assembled from 7 of 12 sources · Last updated Sep 20, 2026, 5:37 PM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
Research summaries | 10 | 22% |
Laboratory research | 4 | 9% |
Testing and diagnosis research | 3 | 7% |
Disease patterns and progression | 1 | 2% |
Liu C (2026). [PMID: 41555109](https://pubmed.ncbi.nlm.nih.gov/41555109/). *Discov Oncol*. [Case Report / Case Series]
Zhao M (2026). [PMID: 42023520](https://pubmed.ncbi.nlm.nih.gov/42023520/). *Histopathology*. [Basic Science / Preclinical]
Sha T (2026). [PMID: 41835473](https://pubmed.ncbi.nlm.nih.gov/41835473/). *Front Cardiovasc Med*. [Case Report / Case Series]
Jing Y (2026). [PMID: 42078171](https://pubmed.ncbi.nlm.nih.gov/42078171/). *J Surg Case Rep*. [Case Report / Case Series]
Della Mura M (2026). [PMID: 42091107](https://pubmed.ncbi.nlm.nih.gov/42091107/). *Am J Dermatopathol*. [Case Report / Case Series]
Bao L (2026). [PMID: 41820040](https://pubmed.ncbi.nlm.nih.gov/41820040/). *Zhonghua Jie He He Hu Xi Za Zhi*. [Review / Meta-Analysis]
Li DH (2025). [PMID: 40032419](https://pubmed.ncbi.nlm.nih.gov/40032419/). *Zhonghua Bing Li Xue Za Zhi*. [Case Report / Case Series]
Al Khader A (2025). [PMID: 41020205](https://pubmed.ncbi.nlm.nih.gov/41020205/). *Pathol Oncol Res*. [Review / Meta-Analysis]
Sono K (2025). [PMID: 40017677](https://pubmed.ncbi.nlm.nih.gov/40017677/). *NMC Case Rep J*. [Case Report / Case Series]
Marcelis L (2025). [PMID: 40205020](https://pubmed.ncbi.nlm.nih.gov/40205020/). *Virchows Arch*. [Review / Meta-Analysis]
AI-curated news mentioning histiocytoma, Angiomatoid fibrous
Updated Sep 3, 2026
A case study highlights angiomatoid fibrous histiocytoma as a rare pulmonary artery tumor that can present with an asymptomatic heart murmur. This discovery adds to the understanding of the tumor's clinical presentation and implications.
A case report details angiomatoid fibrous histiocytoma located in the pulmonary artery, contributing to the understanding of this rare tumor. The review of literature provides insights into its clinical presentation and management.