scholarly journals Mediastinal Low-Grade Fibromyxoid Sarcoma With FUS-CREB3L2 Gene Fusion

Cureus ◽  
2021 ◽  
Author(s):  
Chelsey M Williams ◽  
Wei Du ◽  
William E Mangano ◽  
Lin Mei
Keyword(s):  
Gene Fusion ◽  
Low Grade ◽  
Fibromyxoid Sarcoma

scholarly journals A case of low-grade fibromyxoid sarcoma with unusual central necrosis in a 77-year-old man confirmed by FUS-CREB3L2 gene fusion

2014 ◽  
Vol 5 (12) ◽  
pp. 1123-1127 ◽  
Author(s):  
Aiko Kurisaki-Arakawa ◽  
Keisuke Akaike ◽  
Ran Tomomasa ◽  
Atsushi Arakawa ◽  
Yoshiyuki Suehara ◽  
...  
Keyword(s):  
Gene Fusion ◽  
Low Grade ◽  
Central Necrosis ◽  
Fibromyxoid Sarcoma

Primary Low-Grade Fibromyxoid Sarcoma of the Kidney in a Child with the Alternative EWSR1-CREB3L1 Gene Fusion

2014 ◽  
Vol 17 (4) ◽  
pp. 321-326 ◽  
Author(s):  
Jill C. Rubinstein ◽  
Arjun Visa ◽  
Lei Zhang ◽  
Cristina R. Antonescu ◽  
Emily R. Christison-Lagay ◽  
...  
Keyword(s):  
Gene Fusion ◽  
Low Grade ◽  
Fibromyxoid Sarcoma

scholarly journals Deeply located low-grade fibromyxoid sarcoma with FUS-CREB3L2 gene fusion in a 5-year-old boy with review of literature

2014 ◽  
Vol 9 (1) ◽  
Author(s):  
Aiko Kurisaki-Arakawa ◽  
Yoshiyuki Suehara ◽  
Atsushi Arakawa ◽  
Tatsuya Takagi ◽  
Michiko Takahashi ◽  
...  
Keyword(s):  
Gene Fusion ◽  
Low Grade ◽  
Review Of Literature ◽  
Fibromyxoid Sarcoma

Imaging findings of thoracic low-grade fibromyxoid sarcoma: report of three cases

2009 ◽  
Vol 27 (9) ◽  
pp. 375-380 ◽  
Author(s):  
Eriko Maeda ◽  
Satoshi Ohta ◽  
Takeyuki Watadani ◽  
Akiteru Goto ◽  
Atsushi Nakajima ◽  
...  
Keyword(s):  
Low Grade ◽  
Imaging Findings ◽  
Fibromyxoid Sarcoma

scholarly journals HGG-12. A CASE OF PEDIATRIC SPINAL HIGH-GRADE GLIOMA WITH NTRK1 GENE FUSION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii346-iii346
Author(s):  
Tamaki Morisako ◽  
Daisuke Umebayashi ◽  
Kazuaki Kamata ◽  
Hiroyuki Yamamoto ◽  
Takumi Yamanaka ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
Tumor Progression ◽  
Gene Fusion ◽  
High Grade Glioma ◽  
Gene Panel ◽  
Partial Resection ◽  
Low Grade ◽  
High Grade ◽  
Surgical Specimen

Abstract INTRODUCTION Tumors arising from the spinal cord are uncommon, especially high-grade tumors in pediatric patients. We report a case of high-grade glioma in the spinal cord harboring NTRK1 gene fusion, who received effective entrectinib therapy. CASE REPORT: A 5-year-old boy presented right hemiparesis and MR imaging revealed an intramedullary enhancing mass at the vertebral body level between C3 and Th1. He underwent microsurgical partial resection and the histological diagnosis was low-grade astrocytoma. After the first-line chemotherapy with vincristine and carboplatin, his right hemiparesis deteriorated and recurrent MR imaging showed growth of the tumor. He underwent microsurgical partial resection again and the histological examination was high-grade glioma with endothelial proliferation and necrosis. The chemoradiotherapy with temozolomide and focal irradiation of 50.4 Gy were given, and his neurological symptom slightly improved. One month later, he presented respiratory disturbance and required assisted ventilation with tracheostomy. MR imaging showed tumor progression invading upward to medulla oblongata. NTRK1 gene fusion was detected in the previous surgical specimen by a gene panel testing, and he received entrectinib, a potent inhibitor of tropomyosin receptor kinase (TRK). Since then, no tumor progression has been demonstrated for several months by MRI and he has been stable neurologically. CONCLUSION High-grade spinal cord tumors are rare and effective treatment strategies have not been addressed. Although the frequency of the gene fusion is very low in pediatric gliomas, identification of the driver gene aberration like in this case by a gene panel can provide potential targeted therapies for selected patients.

Low-Grade Fibromyxoid Sarcoma: A Brief Review

2006 ◽  
Vol 130 (9) ◽  
pp. 1358-1360 ◽  
Author(s):  
Stephen E. Vernon ◽  
Pablo A. Bejarano
Keyword(s):  
Spindle Cell ◽  
Fusion Gene ◽  
Cytogenetic Abnormality ◽  
Low Grade ◽  
Deep Soft Tissue ◽  
Balanced Translocation ◽  
Linear Arrangement ◽  
Myxoid Stroma ◽  
Fibromyxoid Sarcoma ◽  
The Common

Abstract Low-grade fibromyxoid sarcomas are uncommon deep soft tissue neoplasms first described by Evans in 1987. They exhibit a deceptively benign appearance, with a whorled or linear arrangement of spindle-shaped cells showing few to absent mitoses. A characteristic, but not specific, feature is the presence of areas of myxoid stroma. Recurrences are common, and late metastases have been recorded. A closely related but morphologically distinct tumor, the so-called hyalinizing spindle cell tumor with giant rosettes, has also been described; both neoplasms share the same cytogenetic abnormality, a balanced translocation resulting in a FUS/CREB3L2 fusion gene. Because of similar clinical behavior and the common cytogenetic abnormality, some authors prefer to consider both lesions as a single entity within the spectrum of low-grade sarcomas.

scholarly journals A rare case of low-grade fibromyxoid sarcoma with ossification which was radiologically detected as apparent calcification and histopathologically proven

2018 ◽  
Vol 12 ◽  
pp. 24-28
Author(s):  
Yasuaki Tsuchida ◽  
Yoshitane Tsukamoto ◽  
Hiroyuki Futani ◽  
Shunsuke Kumanishi ◽  
Takahiro Watanabe ◽  
...  
Keyword(s):  
Rare Case ◽  
Low Grade ◽  
Fibromyxoid Sarcoma

scholarly journals MRI findings of low-grade fibromyxoid sarcoma: a case report and literature review

2018 ◽  
Vol 19 (1) ◽  
Author(s):  
Yali Yue ◽  
Yongkang Liu ◽  
Lina Song ◽  
Xiao Chen ◽  
Yaohui Wang ◽  
...  
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Low Grade ◽  
Mri Findings ◽  
Fibromyxoid Sarcoma
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