Myxofibrosarcoma of the extremity and trunk

Ghazala, C; Agni, Nickil; Ragbir, Maniram; Dildey, Petra; Lee, D; Rankin, Kenneth; Beckingsale, Tom; Gerrand, CH

doi:10.1302/0301-620x.98b12.37568

Cited by 31 publications

(27 citation statements)

References 21 publications

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“…Previous imaging studies have shown that MFS has a tendency to grow in so-called ''tail sign" curvilinear extensions that arise from the main tumor 12,13 . These extensions are thought to correlate with microscopic spread of the tumor along fascial planes and have been found extending up to 3 centimeters away from macroscopically visible tumor borders 12,14,15 . Unfortunately, intraoperatieve delineation of these tumor borders and extensions…”

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confidence: 99%

Immunohistochemical selection of biomarkers for tumor-targeted image-guided surgery of myxofibrosarcoma

Gooyer

Versleijen-Jonkers²,

Hillebrandt-Roeffen³

et al. 2020

Sci Rep

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Myxofibrosarcoma(MFS) is the most common soft tissue sarcoma(STS) in elderly patients. Surgical resection remains the main treatment modality but tumor borders can be difficult to delineate with conventional clinical methods. Incomplete resections are a common problem and local recurrence remains a clinical issue. A technique that has shown great potential in improving surgical treatment of solid tumors is tumor targeted imaging and image-guided surgery with near-infrared fluorescence. To facilitate this technique, it is essential to identify a biomarker that is highly and homogenously expressed on tumor cells, while being absent on healthy non-malignant tissue. The purpose of this study was to identify suitable molecular targets for tumor-targeted imaging of myxofibrosarcoma. Ten potential molecular targets for tumor targeted imaging were investigated with immunohistochemical analysis in myxofibrosarcoma tissue (n = 34). Results were quantified according to the immunoreactive score(IRS). Moderate expression rates were found for uPAR, PDGFRa and EMA/MUC1. High expression rates of VEGF and TEM1 were seen. Strong expression was most common for TEM1 (88.2%). These results confirms that TEM1 is a suitable target for tumor-targeted imaging of myxofibrosarcoma. Keywords Image-guided surgery; Immunohistochemistry; Molecular imaging; Myxofibrosarcoma; Soft tissue sarcoma; Tumor endothelial marker 1(TEM1), Vascular endothelial growth factor (VEGF).Myxofibrosarcoma (MFS) is a histological subtype of soft tissue sarcoma (STS) formerly classified as a myxoidtype malignant fibrous histiocytoma. It has been reclassified and defined as a distinct pathological entity in the World Health Organization (WHO) criteria set in 2002 1,2 . Myxofibrosarcoma is a relatively common sarcoma in the elderly that mainly arises in the extremities 3 . Primary MFS most often presents as a subcutaneous painless, slow growing nodule, but completely infiltrative growth patterns along fascial planes without formation of an evident tumor mass have also been described 4 . The low-grade lesions have a relatively low metastatic potential but show a significant propensity for local recurrences 5-8 . These recurrences show higher-grade histology in up to 50% of all cases compared to the primary tumor 3 . Subsequently, high and intermediate grade disease exhibits a greater metastatic potential, mainly metastasizing to the lungs and lymph nodes 7,9 . Current guidelines recommend wide surgical resection combined with pre or post-operative radiotherapy as the preferred treatment 10 . Despite this strategy, positive margins after surgery occur in up to 20% of cases and reported local relapse rates range from 15 to 65% [5][6][7][8]11 .Currently clinicians rely on conventional imaging modalities such as CT and MRI to determine the location and extent of tumor burden prior to surgery. Translating these conventional imaging modalities to the surgical theatre remains challenging, forcing the surgeon to depend mainly on tactile and visual cues during the procedure. P...

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mentioning

confidence: 99%

Immunohistochemical selection of biomarkers for tumor-targeted image-guided surgery of myxofibrosarcoma

Gooyer

Versleijen-Jonkers²,

Hillebrandt-Roeffen³

et al. 2020

Sci Rep

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“…On the other hand, the lesion warranted further investigation, ideally with X-ray and MRI imaging. The biopsy should be a core needle biopsy, conducted in a reference center and evaluated by a specialized pathologist rather than in a referring institution, since in the latter a higher error rate has been detected, possibly related to the heterogeneous nature of the histology of MFS, which can lead to misdiagnosis [11,12,15]. In our patient, the preoperative biopsy was an incisional biopsy, interpreted by a general pathologist, in a referring center.…”

Section: Discussionmentioning

confidence: 98%

“…Normally MFS presents as a gradually enlarging, painless mass in soft tissues or bone, causing occasionally pain, paresthesia and edema. They frequently appear in the extremities [11,12]. Given their rarity, they may be confused with lipomas, cysts, panniculitis or osteochondral lesions [13].…”

Section: Discussionmentioning

confidence: 99%

“…No complementary imaging studies were requested since it was thought that they would have no additional value. However, an MRI could have shown a "high signal tail" phenomenon, which appears to be moderately sensitive and specific for MFS [12,13].…”

Section: Discussionmentioning

confidence: 99%

“…The usual treatment for MFS is a limb salvage excision accompanied by perioperative local radiotherapy [10,16,17]. However, MFS exhibits an aggressive loco-regional behavior, and with the limb salvage treatment local recurrence (LR) rates are high (16-31% in 3.5 years) , as well as secondary amputation for LR treatment (17-20%) [10,12,15]. Metastasis rates are also high (16-30%) [10,12,13,16,17].…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Undetected Myxofibrosarcoma in a Patient Diagnosed with Long Standing Complex Regional Pain Syndrome; A Case Report

Roldan¹,

Geertzen²,

Dijkstra³

2019

SCR

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Case presentation: A 61-year-old woman diagnosed with long standing, therapy resistant CRPS-1 fourteen years prior was referred to our center for assessment on her wish to have her limb amputated, due to recent worsening of the symptoms. A soft tissue lesion was detected on the affected limb and she was sent back to her primary hospital for further study of this lesion and exclusion of an alternative diagnosis. The biopsies were inconclusive, and no underlying causes were determined for her symptoms in her primary hospital. Thus, back at our center, elective amputation of the affected limb was carried out. Results: Post-operative histology of the amputated limb revealed a myxofibrosarcoma, a rare malignant tumor. Corresponding dissemination study, follow-up and treatment was conducted. The patient died from metastatic complications 17 months post-amputation. Conclusion: This case exemplifies how various confusing factors can lead to misdiagnosis of a malignant tumor. Low incidence of sarcomas, a previous CRPS-1 diagnosis of 14 years and inconclusive biopsies conducted in a primary hospital led to this misdiagnosis. Primary amputation was appropriate due to a dysfunctional limb, and size and local invasion degree of the tumor. The most important take away lessons in this case report are the importance of never discarding a neoplasm in presence of a growing mass even in the case of other possible causes and the fact that it merits a thorough diagnostic procedure, as well as the recommendation to conduct diagnostic tests in reference hospitals specialized in the relevant techniques.

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The effect of radiation or chemotherapy on the local recurrence, overall survival, and distant metastasis in patients with myxofibrosarcoma: A systematic review

Tibbo,

Landau,

Markowitz

et al. 2024

Journal of Surgical Oncology

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Background and ObjectivesManagement of myxofibrosarcoma (MFS) is widely debated, and there is no consensus regarding the effect of adjuvant therapies on local recurrence (LR), distant metastasis (DM), or overall survival (OS). This study aims to determine the effect of adjuvant therapies on the aforementioned outcomes in patients with MFS.MethodsWe conducted a systematic review via Medline, PubMed, CINAHL, Cochrane, Embase, and Google Scholar. Therapeutic studies that reported LR, DM, and OS following adjuvant therapies in patients with MFS were included. Statistical analysis and creation of hazard ratios were performed using Revman 5.3 (Cochrane Collaboration).ResultsWe identified 301 studies, 10 of which were included in the final analysis. Neither chemotherapy nor radiation affected the rate of LR (p = 0.73 and 0.17, respectively) or OS (p = 0.97 and 0.27, respectively). Chemotherapy was significantly associated with lower rates of DM (p = 0.03); however, there was no association between radiation and DM (p = 0.67).ConclusionThere is a lack of consensus regarding the effect of neoadjuvant and adjuvant therapies on LR, DM, and OS in patients with MFS. Our systematic review found that radiation and chemotherapy were not significantly associated with OS or LR in patients with MFS. However, we identified a significant association between chemotherapy and lower rates of DM.

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Myxofibrosarcoma of the extremity and trunk

Cited by 31 publications

References 21 publications

Immunohistochemical selection of biomarkers for tumor-targeted image-guided surgery of myxofibrosarcoma

Immunohistochemical selection of biomarkers for tumor-targeted image-guided surgery of myxofibrosarcoma

Undetected Myxofibrosarcoma in a Patient Diagnosed with Long Standing Complex Regional Pain Syndrome; A Case Report

The effect of radiation or chemotherapy on the local recurrence, overall survival, and distant metastasis in patients with myxofibrosarcoma: A systematic review

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