Low grade fibromyxoid sarcoma (Evans tumour) of the arm

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.

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

SAGE Open Medical Case Reports ◽

10.1177/2050313x20944315 ◽

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.

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The Minimalistic Malignancy- Low grade Fibromyxoid Sarcoma

Journal of Clinical and Diagnostic Pathology ◽

10.14302/issn.2689-5773.jcdp-20-3577 ◽

2020 ◽

Vol 1 (3) ◽

pp. 7-14

Author(s):

Anubha Bajaj

Keyword(s):

Soft Tissue ◽

Soft Tissue Sarcoma ◽

Distant Metastasis ◽

Soft Tissue Sarcomas ◽

Benign Neoplasm ◽

Low Grade ◽

Biological Behaviour ◽

Preclinical Stage ◽

Tumour Incidence ◽

Fibromyxoid Sarcoma

Low grade fibromyxoid sarcoma (LGFMS) is an exceptional, low grade, soft tissue sarcoma with indolent biological behaviour, extensive preclinical stage, enhanced localized tumour reoccurrence and delayed, distant metastasis. As the deceptively benign neoplasm was initially scripted by Evans in 1987, the tumefaction is nomenclated as “Evan’s tumour”. Incidence of sarcomas is nearly 1% of adult malignancies wherein low-grade fibromyxoid sarcoma represents roughly beneath <5% of soft tissue sarcomas 1.

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Low-Grade Myofibroblastic Sarcoma (LGMS) of the Extremities – A Systematic Review and Case Report

10.21203/rs.3.rs-38829/v1 ◽

2020 ◽

Author(s):

Astrid Schenker ◽

Burkhard Lehner ◽

Ewgenija Gutjhar ◽

Gunhild Mechtersheimer ◽

Leila Harhaus ◽

...

Keyword(s):

Systematic Review ◽

Local Recurrence ◽

Distant Metastasis ◽

Cochrane Library ◽

Wide Resection ◽

Clinical Aspects ◽

Low Grade ◽

Incomplete Resection ◽

Myofibroblastic Sarcoma

Abstract Background: Low-grade myofibroblastic sarcoma (LGMS) is a rare subtype of soft tissue sarcoma of intermediate grade often representing fibromatosis-like features with a rare metastasing behavior. This type of tumor has a predilection for the head and neck region, but also occurs in the extremities. Confirming the diagnosis is difficult and treatment strategies have to be chosen individually.Methods: The objective of this study was to conduct a systematic review for LGMS of the extremities. The electronic databases PubMed and the Cochrane Library were searched for eligible studies. 141 abstracts were screened on PubMed, while 10 studies were identified as eligible. Cases were summarized in terms of clinical aspects, therapeutic regimen with the primary endpoint of follow-up controls regarding local recurrence or distant metastasis. In addition, we present the rare case and surgical management of a 28-year-old male patient with residual LGMS of the thumb after initial incomplete resection. Results: 33 cases of LGMS in the extremities were identified on PubMed. Cochrane library didn’t show any results. All of them were surgically resected. Only two cases of LGMS in the hand were described in literature so far. Treatment options varied from local excision to wide resection without exact definition of the safety distance. 26 cases provided follow-up information with local recurrence in 6 cases (23 %), while 4 cases (15 %) showed distant metastasis. Conclusions: Wide resection should be the surgical aim to avoid local recurrence and distant metastases. While the excision of tumors of the thumb and hand often require closer resection margins, due to the close proximity of anatomical structures, tumor-free margins are elementary even if tissue transfer from a donor site is needed for reconstruction.

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Low-grade fibromyxoid sarcoma of intracranial origin

Journal of Neurosurgery ◽

10.3171/jns/2008/108/4/0798 ◽

2008 ◽

Vol 108 (4) ◽

pp. 798-802 ◽

Cited By ~ 19

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.

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