scholarly journals Intracranial myxoid mesenchymal tumors with EWSR1 -CREB family gene fusions: myxoid variant of angiomatoid fibrous histiocytoma or novel entity?

2017 ◽  
Vol 28 (2) ◽  
pp. 183-191 ◽  
Author(s):  
Tejus A. Bale ◽  
Angelica Oviedo ◽  
Harry Kozakewich ◽  
Caterina Giannini ◽  
Phani K. Davineni ◽  
...  
Keyword(s):  
Fibrous Histiocytoma ◽  
Gene Fusions ◽  
Mesenchymal Tumors ◽  
Angiomatoid Fibrous Histiocytoma ◽  
Family Gene

scholarly journals Intracranial Myxoid Mesenchymal Tumor With EWSR1-ATF1 Fusion

2020 ◽  
Vol 79 (3) ◽  
pp. 347-351 ◽  
Author(s):  
Leomar Y Ballester ◽  
Jeanne M Meis ◽  
Alexander J Lazar ◽  
Sujit S Prabhu ◽  
Kimberly B Hoang ◽  
...  
Keyword(s):  
Soft Tissue Tumor ◽  
Fibrous Histiocytoma ◽  
Camp Response Element Binding ◽  
Mesenchymal Tumor ◽  
Gene Fusions ◽  
Camp Response Element ◽  
Proliferation Indices ◽  
Ewsr1 Rearrangement ◽  
Element Binding Protein ◽  
Family Gene

Abstract Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that arises primarily in the extremities of young adults. Recurrent gene fusions involving EWSR1 with members of the cAMP response element binding protein (CREB) family have been reported in a diverse group of tumors, including AFH. AFH-like lesions have been reported to occur intracranially and the reported cases show low proliferation indices, frequently have a connection with the dura, and show recurrent EWSR1 rearrangements. These tumors have been termed intracranial myxoid mesenchymal tumor with EWSR1-CREB family gene fusions. A literature search identified 11 reported cases of intracranial AFH-like lesions with an EWSR1 rearrangement. Here, we report a case of intracranial myxoid mesenchymal tumor with an EWSR1-ATF1 fusion in an adult patient, and review the existing literature on this recently described entity.

Angiomatoid Fibrous Histiocytoma: The Current Status of Pathology and Genetics

2015 ◽  
Vol 139 (5) ◽  
pp. 674-682 ◽  
Author(s):  
Khin Thway ◽  
Cyril Fisher
Keyword(s):  
Fibrous Histiocytoma ◽  
Case Reports ◽  
Correct Diagnosis ◽  
Molecular Genetic ◽  
Case Series ◽  
Current Status ◽  
Gene Fusions ◽  
Soft Tissue Neoplasm ◽  
Medical Databases ◽  
Angiomatoid Fibrous Histiocytoma

Context Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate biologic potential and uncertain differentiation, most often arising in the superficial extremities of children and young adults. While it has characteristic histologic features of nodular distributions of ovoid and spindle cells with blood-filled cystic cavities and a surrounding dense lymphoplasmacytic infiltrate, there is a significant morphologic spectrum, which coupled with its rarity and lack of specific immunoprofile can make diagnosis challenging. Angiomatoid fibrous histiocytoma is associated with 3 characteristic gene fusions, EWSR1-CREB1 and EWSR1-ATF1, which are also described in other neoplasms, and rarely FUS-ATF1. Angiomatoid fibrous histiocytoma is now recognized at an increasing number of sites and is known to display a variety of unusual histologic features. Objective To review the current status of AFH, discussing putative etiology, histopathology with variant morphology and differential diagnosis, and current genetics, including overlap with other tumors harboring EWSR1-CREB1 and EWSR1-ATF1 fusions. Data Sources Review of published literature, including case series, case reports, and review articles, in online medical databases. Conclusions The occurrence of AFH at several unusual anatomic sites and its spectrum of morphologic patterns can result in significant diagnostic difficulty, and correct diagnosis is particularly important because of its small risk of metastasis and death. This highlights the importance of diagnostic recognition, ancillary molecular genetic confirmation, and close clinical follow-up of patients with AFH. Further insight into the genetic and epigenetic changes arising secondary to the characteristic gene fusions of AFH will be integral to understanding its tumorigenic mechanisms.

Angiomatoid Fibrous Histiocytoma

2016 ◽  
Vol 78 (3) ◽  
pp. 219-220
Author(s):  
Yoshihiko Inoue ◽  
Shiho Terawaki ◽  
Kazuko Imamura ◽  
Yumiko Kubota ◽  
Masazumi Tsuneyoshi ◽  
...  
Keyword(s):  
Fibrous Histiocytoma ◽  
Angiomatoid Fibrous Histiocytoma

scholarly journals FOXO1 gene involvement in a non-rhabdomyosarcomatous neoplasm

2021 ◽  
Author(s):  
Simon Haefliger ◽  
Muriel Genevay ◽  
Michel Bihl ◽  
Romina Marone ◽  
Daniel Baumhoer ◽  
...  
Keyword(s):  
Transcription Factors ◽  
Soft Tissue ◽  
Genetic Heterogeneity ◽  
Gene Fusion ◽  
Fusion Gene ◽  
Soft Tissue Tumors ◽  
Gene Fusions ◽  
Mesenchymal Tumors ◽  
Myoepithelial Differentiation ◽  
Foxo1 Gene

AbstractMyoepithelial neoplasms of soft tissue are rare tumors with clinical, morphological, immunohistochemical, and genetic heterogeneity. The morphological spectrum of these tumors is broad, and the diagnosis often requires immunostaining to confirm myoepithelial differentiation. Rarely, tumors show a morphology that is typical for myoepithelial neoplasms, while the immunophenotype fails to confirm myoepithelial differentiation. For such lesions, the term “myoepithelioma-like” tumor was introduced. Recently, two cases of myoepithelioma-like tumors of the hands and one case of the foot were described with previously never reported OGT-FOXO gene fusions. Here, we report a 50-year-old woman, with a myoepithelial-like tumor localized in the soft tissue of the forearm and carrying a OGT-FOXO1 fusion gene. Our findings extend the spectrum of mesenchymal tumors involving members of the FOXO family of transcription factors and point to the existence of a family of soft tissue tumors that carry the gene fusion of the OGT-FOXO family.

scholarly journals Aneurysmal ("angiomatoid") fibrous histiocytoma in a child

1989 ◽  
Vol 4 (4) ◽  
pp. 159 ◽  
Author(s):  
Chong Jai Kim ◽  
Jung Hee Cho ◽  
Je Geun Chi
Keyword(s):  
Fibrous Histiocytoma ◽  
Angiomatoid Fibrous Histiocytoma
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