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 Low-grade fibromyxoid sarcoma: A rare case in an unusual location

2020 ◽  
Vol 8 ◽  
pp. 2050313X2094431
Author(s):  
Diandra Perez ◽  
Ola El-Zammar ◽  
Brando Cobanov ◽  
Rana Naous
Keyword(s):  
Young Adults ◽  
Soft Tissue ◽  
Rare Case ◽  
Primary Site ◽  
Low Grade ◽  
Deep Soft Tissue ◽  
Unusual Location ◽  
Fibromyxoid Sarcoma ◽  
Intrathoracic Mass

Low-grade fibromyxoid sarcoma, also known as Evans tumor, is a low-grade sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site, with only a few cases reported in the literature. Here, we present a case of Evans tumor occurring in an unusual and rarely reported location; an intrathoracic mass arising from the diaphragmatic pleura.

Low-Grade Fibromyxoid Sarcoma and Hyalinizing Spindle Cell Tumor With Giant Rosettes Share a Common t(7;16)(q34;p11) Translocation

2003 ◽  
Vol 27 (9) ◽  
pp. 1229-1236 ◽  
Author(s):  
Robin Reid ◽  
M. V. Chandu de Silva ◽  
Lindsay Paterson ◽  
Eleanor Ryan ◽  
Cyril Fisher
Keyword(s):  
Spindle Cell ◽  
Cell Tumor ◽  
Low Grade ◽  
Spindle Cell Tumor ◽  
Fibromyxoid Sarcoma

Low-grade fibromyxoid sarcoma of the parapharyngeal space: an unusual location

2021 ◽  
Vol 14 (5) ◽  
pp. e237083
Author(s):  
Muhammad Hammad Deewani ◽  
Muhammad Hassan Danish ◽  
Muhammad Sohail Awan ◽  
Nasir Ud Din
Keyword(s):  
Head And Neck ◽  
Parapharyngeal Space ◽  
Neck Region ◽  
Low Grade ◽  
Head And Neck Region ◽  
Myxoid Stroma ◽  
Spindle Cells ◽  
Fibromyxoid Sarcoma ◽  
Established Treatment

Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon soft-tissue malignancy. LGFMS preferentially affects trunks and extremities of young adults; however, occasional cases have been reported in different sites of head and neck region including oral cavity, larynx and oropharynx. LGFMS usually exhibit areas of collagenised and myxoid stroma with appearance of spindle cells in whorling pattern. It is a challenge to diagnose it accurately as most of the time it is misdiagnosed as benign neoplastic entity of spindle cells. There have been only few isolated cases of LGFMS reported in head and neck region and LGFMS originating from the parapharyngeal space has never been reported before. We recently experienced a case of low grade fibomyxoid sarcoma in parapharyngeal space of neck. LGFMS have the propensity to locally recur and to metastasise. Due to its rarity in head and neck region, there are no well-established treatment and follow-up guidelines.

scholarly journals Low grade fibromyxoid sarcoma (Evans tumour) of the arm

2014 ◽  
Vol 47 (02) ◽  
pp. 259-262 ◽  
Author(s):  
Shalaka Indap ◽  
Manjiri Dasgupta ◽  
Nilay Chakrabarti ◽  
Abhishek Agarwal
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Distant Metastasis ◽  
Wide Resection ◽  
Soft Tissue Tumour ◽  
Low Grade ◽  
Deep Soft Tissue ◽  
The Past ◽  
Tissue Tumour ◽  
Fibromyxoid Sarcoma

ABSTRACTA low-grade fibromyxoid sarcoma is a rare soft tissue tumour that has a tendency to develop in the deep soft tissue of young adults with potential for local recurrence or distant metastasis. We describe a 40-year-old female patient with a low-grade fibromyxoid sarcoma of the shoulder that had been excised twice in the past and then had recurred after a few months. A wide resection of this tumour with flap reconstruction was performed followed by radiation to the area. The patient had no evidence of local recurrence or distant metastasis at 2 years after surgery.

Hyalinizing Spindle Cell Tumor with Giant Rosettes and Low-grade Fibromyxoid Sarcoma: An Immunohistochemical and Ultrastructural Comparative Investigation

2003 ◽  
Vol 27 (5) ◽  
pp. 349-355 ◽  
Author(s):  
Alessandro Franchi ◽  
Daniela Massi ◽  
Marco Santucci
Keyword(s):  
Spindle Cell ◽  
Comparative Investigation ◽  
Cell Tumor ◽  
Low Grade ◽  
Spindle Cell Tumor ◽  
Fibromyxoid Sarcoma

scholarly journals Clinicopathologic and molecular genetic characterization of low-grade fibromyxoid sarcoma, and cloning of a novel FUS/CREB3L1 fusion gene

2005 ◽  
Vol 85 (3) ◽  
pp. 408-415 ◽  
Author(s):  
Fredrik Mertens ◽  
Christopher D M Fletcher ◽  
Cristina R Antonescu ◽  
Jean-Michel Coindre ◽  
Maurizio Colecchia ◽  
...  
Keyword(s):  
Genetic Characterization ◽  
Fusion Gene ◽  
Molecular Genetic ◽  
Low Grade ◽  
Fibromyxoid Sarcoma ◽  
Molecular Genetic Characterization

Low-Grade Fibromyxoid Sarcoma and Hyalinizing Spindle Cell Tumor With Giant Rosettes

2000 ◽  
Vol 24 (10) ◽  
pp. 1353-1360 ◽  
Author(s):  
Andrew L. Folpe ◽  
Kathryn L. Lane ◽  
Gerson Paull ◽  
Sharon W. Weiss
Keyword(s):  
Spindle Cell ◽  
Cell Tumor ◽  
Low Grade ◽  
Spindle Cell Tumor ◽  
Fibromyxoid Sarcoma

Low-grade fibromyxoid sarcoma of intracranial origin

2008 ◽  
Vol 108 (4) ◽  
pp. 798-802 ◽  
Author(s):  
Ryuta Saito ◽  
Toshihiro Kumabe ◽  
Mika Watanabe ◽  
Hidefumi Jokura ◽  
Makoto Shibuya ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Parietal Lobe ◽  
High Rate ◽  
Surgical Removal ◽  
Low Grade ◽  
Deep Soft Tissue ◽  
Soft Tissue Neoplasm ◽  
Diffuse Infiltration ◽  
First Case ◽  
Fibromyxoid Sarcoma

✓The authors report on a 21-year-old man who presented with a low-grade fibromyxoid sarcoma primarily located in the right parietal lobe with diffuse infiltration. The low-grade fibromyxoid sarcoma is a rare sarcoma of the deep soft tissue that is characterized as an indolent but metastasizing soft-tissue neoplasm with a deceptively benign histological appearance. Only one case of intracranial origin has been previously reported in the literature. A high rate of local recurrence and eventual metastasis has been demonstrated for this tumor in deep soft tissue. Similarly, the patient in the present case suffered recurrence 6 times; he underwent treatment by surgical removal 4 times, Gamma Knife surgery twice, and local radiation therapy once during the 7-year follow-up period. The tumor is still under control without any evidence of extracranial metastasis. To the authors' knowledge, this is the first case report that discusses the clinical course of this rare disease in detail.

Solitary Fibrous Tumors in Pediatric Patients: A Rare and Potentially Overdiagnosed Neoplasm, Confirmed by STAT6 Immunohistochemistry

2017 ◽  
Vol 21 (4) ◽  
pp. 389-400 ◽  
Author(s):  
Serena Y Tan ◽  
Linda J Szymanski ◽  
Carlos Galliani ◽  
David Parham ◽  
Eduardo Zambrano
Keyword(s):  
Fusion Gene ◽  
Pediatric Population ◽  
Bone Cyst ◽  
Cell Tumor ◽  
Positive Staining ◽  
Low Grade ◽  
Adipocytic Differentiation ◽  
Fibromyxoid Sarcoma ◽  
Fibrous Tumor ◽  
Round Blue Cell Tumor

Pathological diagnosis of solitary fibrous tumor (SFT) in the pediatric population is challenging, as it occurs uncommonly in this age-group and resembles other spindle cell neoplasms. SFT contains a NAB2–STAT6 fusion gene, which can be reliably detected using STAT6 immunohistochemistry. Positive staining is highly sensitive and specific. We sought to investigate the utility of STAT6 immunohistochemistry, to show how commonly SFT was historically recognized at 3 academic pediatric institutions, to reclassify them when appropriate, and to demonstrate features of major mimics of SFT. Our series included cases with a previous diagnosis of SFT or for which SFT was among key considerations, from 3 major academic pediatric hospitals seen over the past 30 years. Of 18 tumors identified, only 3 tumors from 2 patients demonstrated positive STAT6 staining as well as the typical histology and immunophenotype seen in SFT. The remaining 15 tumors were reclassified based on morphology, additional immunohistochemistry and fluorescence in situ hybridization as desmoid-type fibromatosis (3 tumors), nerve sheath/neural tumors (3 tumors), low-grade fibromyxoid sarcoma, medallion-like dermal fibroma, poorly differentiated Sertoli cell tumor, nodular/proliferative fasciitis, calcifying fibrous tumor, aneurysmal bone cyst of soft tissue, STAT6-negative SFT with adipocytic differentiation, undifferentiated small round blue cell tumor, and scar (1 tumor each). Our study confirms that SFT is rare in the pediatric population and that it is potentially overdiagnosed. STAT6 immunohistochemistry is recommended to confirm the diagnosis of SFT in the pediatric population.

Sign in / Sign up

Export Citation Format

Share Document