Angiomatoid Fibrous Histiocytoma

A variant of malignant fibrous histiocytoma that typically occurs in children and adolescents and is characterized by nodular subcutaneous growth and may recur after surgical excision. Characteristic histologic features include lobulated sheets of HISTIOCYTE - like cells associated with areas of hemorrhage and cystic pseudovascular spaces, as well as a striking cuffing of inflammatory cells, mimicking a LYMPH NODE METASTASIS. May be associated with somatic fusions of the FUS, ATF1, EWSR1, and CREB1 genes. OMIM: 612160

Also Known As:

Networked: 23 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Sarcoma (Soft Tissue Sarcoma)
3.	Carcinoma (Carcinomatosis)
4.	Malignant Fibrous Histiocytoma
5.	Clear Cell Sarcoma

Experts

1.	Fisher, Cyril: 6 articles (09/2019 - 06/2012)
2.	Thway, Khin: 6 articles (11/2016 - 02/2008)
3.	Kao, Yu-Chien: 2 articles (01/2022 - 04/2017)
4.	Yoshida, Akihiko: 2 articles (01/2022 - 01/2017)
5.	Antonescu, Cristina R: 2 articles (11/2020 - 04/2017)
6.	Sung, Yun-Shao: 2 articles (11/2020 - 04/2017)
7.	Zhang, Lei: 2 articles (11/2020 - 04/2017)
8.	Wren, Dorte: 2 articles (11/2016 - 06/2015)
9.	D'Cunha, Jonathan: 1 article (12/2022)
10.	Downey, Francis X: 1 article (12/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Angiomatoid Fibrous Histiocytoma:

1.	tocilizumab (atlizumab)FDA Link 01/01/2020 - "Tocilizumab for the treatment of paraneoplastic inflammatory syndrome associated with angiomatoid fibrous histiocytoma." 01/01/2021 - "Tocilizumab Controls Paraneoplastic Inflammatory Syndrome but Does Not Suppress Tumor Growth of Angiomatoid Fibrous Histiocytoma." 10/01/2018 - "Therapeutic response of metastatic angiomatoid fibrous histiocytoma carrying EWSR1-CREB1 fusion to the interleukin-6 receptor antibody tocilizumab."
2.	Transcription Factors (Transcription Factor)IBA 01/01/2022 - "ATF1, CREB1, and CREM, which encode the CREB family of transcription factors, are fused with EWSR1 or FUS in human neoplasms, such as angiomatoid fibrous histiocytoma. " 01/01/2022 - "Intracranial myxoid mesenchymal tumors (IMMTs) with fusions between EWSR1/FUS and CREB transcription factors have morphologic overlap with myxoid angiomatoid fibrous histiocytoma (mAFH) and myoepithelial tumor/carcinoma (MET/MEC). "
3.	Keratins (Keratin)IBA 11/01/2020 - "This entity appears to display hybrid features between angiomatoid fibrous histiocytoma (cystic growth and lymphoid cuffing) and mesothelioma (peritoneal/pleural involvement, epithelioid phenotype, and cytokeratin and WT1 co-expression)." 06/01/2023 - "EWSR1/FUS-CREB-rearranged mesenchymal neoplasms are an emerging heterogeneous group of soft tissue tumors that encompasses low-grade lesions (angiomatoid fibrous histiocytoma/AFH) and a group of predominantly intra-abdominal aggressive sarcomas with epithelioid morphology and frequent keratin expression. "
4.	Activating Transcription Factor 1IBA 01/01/2021 - "Intracranial mesenchymal tumors with FET-CREB fusions are a recently described group of neoplasms in children and young adults characterized by fusion of a FET family gene (usually EWSR1, but rarely FUS) to a CREB family transcription factor (ATF1, CREB1, or CREM), and have been variously termed intracranial angiomatoid fibrous histiocytoma or intracranial myxoid mesenchymal tumor. "
5.	Interleukin-6 Receptors (Interleukin 6 Receptor)IBA 10/01/2018 - "Therapeutic response of metastatic angiomatoid fibrous histiocytoma carrying EWSR1-CREB1 fusion to the interleukin-6 receptor antibody tocilizumab."
6.	Cyclic AMP Response Element-Binding Protein (Cyclic AMP-Responsive DNA-Binding Protein)IBA 04/01/2017 - "Recurrent gene fusions involving EWSR1 with members of the cAMP response element binding protein (CREB) family (ATF1 and CREB1) have been reported in a diverse group of tumors including angiomatoid fibrous histiocytoma (AFH), soft tissue and gastrointestinal clear cell sarcoma, primary pulmonary myxoid sarcoma, and hyalinizing clear cell carcinoma of salivary gland. "
7.	Interleukin-6 (Interleukin 6)IBA 10/01/2015 - "Paraneoplastic Syndrome of Angiomatoid Fibrous Histiocytoma May Be Caused by EWSR1-CREB1 Fusion-induced Excessive Interleukin-6 Production."
8.	Biomarkers (Surrogate Marker)IBA 09/01/2023 - "Next-generation sequencing analysis identified an EWSR1 exon9-ATF1 exon4 fusion, a diagnostic biomarker of angiomatoid fibrous histiocytoma (AFH). "
9.	Proteins (Proteins, Gene)FDA Link 09/01/2000 - "Thus, the FUS/ATF-1 gene in angiomatoid fibrous histiocytoma is predicted to code for a protein that is very similar to the chimeric EWS/ATF-1 found in clear cell sarcoma."
10.	ParaffinIBA 03/01/2005 - "Formalin-fixed, paraffin-embedded tissue from 45 soft tissue sarcomas was analysed for allelic imbalance/loss of heterozygosity (AI/LOH) of chromosome 9. The specimens consisted of 17 cases of soft tissue leiomyosarcoma (LMS), 4 cases of cutaneous LMS, 22 cases of conventional malignant fibrous histiocytoma (MFH) and 2 cases of angiomatoid fibrous histiocytoma. "

Therapies and Procedures

1.	Therapeutics 05/01/2011 - "Angiomatoid fibrous histiocytoma: staging and evaluation of response to therapy with F-18 FDG PET/CT." 01/01/2021 - "Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that rarely metastasizes but lacks effective systemic therapy once it propagates. "
2.	Radiotherapy 05/01/1997 - "The authors present a case of a patient with an angiomatoid fibrous histiocytoma of the arm, treated with radiotherapy after three postsurgical recurrences." 05/01/1997 - "Angiomatoid fibrous histiocytoma of the arm treated by radiotherapy for local recurrence--case report."
3.	Pneumonectomy (Lung Volume Reduction) 12/01/2022 - "Patient underwent transmediastinal right pneumonectomy with histopathologic diagnosis of pulmonary artery angiomatoid fibrous histiocytoma, a rare etiology mimicking large pulmonary artery embolism."
4.	Drug Therapy (Chemotherapy) 01/01/2017 - "Angiomatoid fibrous histiocytoma: A case of local recurrence and metastases to loco-regional lymph nodes that responded to chemotherapy."