scholarly journals Intracranial mesenchymal tumor with FET‐CREB fusion – a unifying diagnosis for the spectrum of intracranial myxoid mesenchymal tumors and angiomatoid fibrous histiocytoma‐like neoplasms

2020 ◽  
Author(s):  
Emily A. Sloan ◽  
Jason Chiang ◽  
Javier E. Villanueva‐Meyer ◽  
Sanda Alexandrescu ◽  
Jennifer M. Eschbacher ◽  
...  
2017 ◽  
Vol 28 (2) ◽  
pp. 183-191 ◽  
Author(s):  
Tejus A. Bale ◽  
Angelica Oviedo ◽  
Harry Kozakewich ◽  
Caterina Giannini ◽  
Phani K. Davineni ◽  
...  

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

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

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii210-ii211
Author(s):  
Benjamin Ward ◽  
Christopher Wang ◽  
Rob Macaulay ◽  
James Liu

Abstract Intracranial myxoid mesenchymal tumors (IMMT) carrying an EWSR1-CREB gene family fusion are extremely rare and have only been identified in ten relatively recent reported cases. There is some question as to whether this is a novel entity, or a myxoid variant of angiomatoid fibrous histiocytoma (AFH), given certain histopathological similarities. Despite these similarities, the increasing number of cases reported appear to demonstrate clear histological differences that indicate IMMTs are a distinct and novel entity. Previous reports have focused on histological analysis but have lacked detailed long-term clinical follow-up and recommendations regarding treatment approach. In this case, we describe a 48-year-old female who presented with a left intraventricular mass that was identified histologically as an IMMT with an EWSR1-ATF1 gene fusion. Following initial resection, the tumor demonstrated local recurrence. Repeat resection was performed followed by immediate demonstration of local, as well as distant, tumor recurrence. Subsequent histological analysis of the tumor demonstrated a myxoid mesenchymal tumor clearly distinct from AFH. Fractionated stereotactic radiation therapy was administered following the second resection and tumor control was achieved at 1 year. This case is particularly remarkable as it is only the second reported IMMT case to occur in a middle-aged adult, with all other cases occurring in children or young adults. The two middle-aged adult cases share striking similarities in clinical presentation, including a history of breast cancer. Our findings indicate that an intracranial myxoid mesenchymal tumor is a novel and rare entity that may demonstrate rapid local and distant recurrence. Given the aggressive recurrence seen with the presented case, we recommend the treatment plan to be surgical resection followed by adjuvant radiation therapy to maintain tumor control.


2013 ◽  
Vol 67 (3) ◽  
pp. 210-215 ◽  
Author(s):  
Yu-Chien Kao ◽  
Jui Lan ◽  
Hui-Chun Tai ◽  
Chien-Feng Li ◽  
Kai-Wen Liu ◽  
...  

2008 ◽  
Vol 30 (3) ◽  
pp. 242-244 ◽  
Author(s):  
Laura Martelli ◽  
Paola Collini ◽  
Cristina Meazza ◽  
Alessandra Bianchi ◽  
Filippo Salvini ◽  
...  

2004 ◽  
Vol 128 (10) ◽  
pp. 1136-1141 ◽  
Author(s):  
David M. Weinrach ◽  
Kim L. Wang ◽  
Elizabeth L. Wiley ◽  
William B. Laskin

Abstract Context.—Common fibrous histiocytoma (cFH) or dermatofibroma and dermatofibrosarcoma protuberans (DFSP) are 2 spindle cell mesenchymal tumors that are distinguished in part by their microscopic growth patterns and clinically by the greater propensity for DFSP to recur. Matrix metalloproteinases (MMPs) potentially play a role in modulating the growth patterns of cFH and DFSP by remodeling the extracellular matrix. Objective.—To evaluate the immunohistochemical (IHC) expression of MMP-1, MMP-2, MMP-9, and MMP-14 in DFSP and cFH, because (1) MMP-1, MMP-2, MMP-9, and MMP-14 are synthesized by dermal fibroblasts, the major constituent of DFSP and cFH; and (2) platelet-derived growth factor B, which is overexpressed in most examples of DFSP because of t(17;22), activates ets-1, a transcription factor that regulates molecules associated with tumor invasion and metastasis, including MMP-1, MMP-3, and MMP-9. Design.—Immunohistochemical studies were performed on archived, formalin-fixed, paraffin-embedded tissue of DFSP (n = 48) and cFH (n = 47). Results.—Significant IHC expression (>10% of tumor cells) in cFH included MMP-14 (27 [59%] of 46 tumors positive), MMP-2 (21 [47%] of 45 tumors positive), MMP-9 (9 [20%] of 45 tumors positive), and MMP-1 (6 [13%] of 46 tumors positive). No DFSPs showed significant IHC expression of any of the MMPs evaluated. However, anti– MMP-2 highlighted a rich microvascular element within deep tumor tissue present in 81% of DFSPs with a prominent subcutaneous component. Conclusion.—Our IHC results indicate that MMP-1 and MMP-9 are not up-regulated in DFSP. Convincing expression of MMP-14 in cFH suggests that this MMP may affect the growth pattern of the lesion, perhaps by activating MMP-2 expression in tumor cells. In DFSP, MMP-2 may play a role in tumor angiogenesis.


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