Materiały źródłowe

• #1 Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9244941/

  Myxofibrosarcoma (MFS) is a common entity of adult soft tissue sarcomas (STS) characterized by a predilection of the extremities and a high local recurrence rate. The diagnosis is challenging due to the unavailability of specific immunohistochemical markers and is based on the analysis of cytomorphologic features. The cornerstone of treatment for localized disease is surgical resection, possibly in combination with radiotherapy, while in metastatic MFS the standard systemic treatment is based on anthracycline chemotherapy, targeted therapy such as TKIs and CDKIs or immunotherapy. Diagnosis mainly relies on histopathological analysis (IHC) and imaging (MRI), however in recent years also NGS proved helpful in detecting distinct molecular alterations. Currently, histological MFS grading relies on the general FNCLCC grading system for STS, which assigns grades 1 to 3 depending on tumor differentiation, mitotic count and tumor necrosis. However, while it is accurate to predict the probability of distant metastases (DM) and overall survival (OS), it does not predict local recurrence (LR). The presence of TP53 alteration and KRAS amplification was also significantly associated with poorer overall survival. Diagnosis is still currently based on the analysis of cytomorphologic appearance comprising the presence of a myxoid background, nuclear atypia, pseudolipoblasts and curvilinear blood vessels. Preoperative MRI can be useful in order to identify high risk of local recurrence (LR), particularly in the presence of features such as high myxoid matrix content, high grade of contrast enhancement and presence of an infiltrative pattern (tail sign). The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern.

• #2 Myxofibrosarcoma: A Diagnostic Pitfall

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3719110/

  Myxofibrosarcoma (MFS) is a variant of the group of malignant fibrous histiocytomas. It is one of the most aggressive types of soft tissue neoplasms. The clinical presentation is not pathognomonic and the histological aspects are highly heterogenous, frequently delaying the diagnosis or leading to misdiagnosis. Complementary histochemical and immunohistochemical stainings are mandatory to achieve the diagnosis of MFS. […] The myxoid type, also known as myxofibrosarcoma (MFS), is the second most frequent subtype, representing approximately 20% of cases and is characterized by an association of myxoid areas and cellular areas in variable proportions. […] As illustrated in this case, not only the clinical diagnosis but also the histopathologic identification can be very difficult. […] Final diagnosis relies on a large series of histochemical and immunohistochemical staining.

• #3 Myxofibrosarcoma: Prognosis, Treatment & Staging

  https://my.clevelandclinic.org/health/diseases/22563-myxofibrosarcoma

  Myxofibrosarcoma may be challenging to diagnose because it can appear similar to other types of growths. […] To find out if you have MFS or another type of growth, your healthcare provider may perform: Imaging scans, such as a CT scan or MRI, look at whether the growth extends to your deep muscle tissues. […] Needle or open biopsy takes a small sample of the growth to examine in a lab. […] Chest X-ray checks if cancer has spread to your lungs. If MFS spreads (metastasizes), its most likely to go to your lungs. […] Your healthcare provider surgically removes the tumor and a small amount of surrounding tissue. […] You may have radiation therapy before or after surgery to shrink the tumor or lower the risk of it coming back. […] Healthcare providers are less likely to use chemotherapy for myxofibrosarcoma than for other types of cancer.

• #4 Myxofibrosarcoma | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/myxofibrosarcoma/

  Myxofibrosarcoma is a type of soft tissue sarcoma, which is a type of cancer. […] A specialist will diagnose myxofibrosarcoma with tests. You might have: a physical examination looking at and feeling your lump, a scan taking pictures of the inside of the body. Scans might include an ultrasound, x-ray, CT, MRI or PET scans, a biopsy taking and testing a sample of the tumour. A doctor will look to see if the biopsy looks like myxofibrosarcoma. […] There are an average of 178 cases of myxofibrosarcoma diagnosed every year in England. […] Myxofibrosarcoma makes up 4.5% of all soft tissue sarcomas.

• #5 Biology and Management of High-Grade Myxofibrosarcoma: State of the Art and Future Perspectives

  https://www.mdpi.com/2075-4418/13/19/3022

  Myxofibrosarcoma (MFS) is one of the most common adult soft tissue sarcomas, typically arising in the extremities. The diagnosis of MFS is challenging due to a lack of well-characterized immunohistochemical markers. High-grade MFS displays highly complex karyotypes with multiple copy number alterations. This review highlights the clinical, radiological, histological, cytogenetic, and molecular genetic features of high-grade MFS. […] The standard treatment for localized MFS is surgical resection. The systemic treatment options for advanced disease are limited. […] On MRI, the lesions are typically nodular or lobular in shape and display low to intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. […] The tail sign is not only valuable for suggesting the diagnosis of MFS, but its recognition is also essential in preoperative planning.

• #6 Giant Myxofibrosarcoma in the Lower Limb: An Overview of Diagnostic and Clinical Management

  https://www.mdpi.com/2075-4418/14/12/1298

  The purpose of this review is to emphasize the current standard of care for patients with MFS, starting from clinical presentation, diagnosis, and classification, and to highlight the surgical management and the main adjuvant therapies, along with the tumor-associated morbidity and mortality, to improve the outcome and increase the survival of this subgroup of patients. […] MRI is preferred for preoperative evaluation, planning, and grading. MFS has specific MRI features like the tail sign (the infiltrative growth pattern), typical nodular or lobular lesions, hemorrhage, and necrosis (in high-grade tumors). […] For our case, after the computed tomography examination with high suspicion of MFS with distant metastases, MRI analysis, along with surgical biopsy and histopathological examination, allowed us to confirm the diagnosis and better understand the invasiveness of the tumor.

• #6 Giant Myxofibrosarcoma in the Lower Limb: An Overview of Diagnostic and Clinical Management

  https://www.mdpi.com/2075-4418/14/12/1298

  Myxofibrosarcoma (MFS), an aggressive soft tissue sarcoma, is one of the undifferentiated pleomorphic sarcomas; it has a low incidence, affecting people in the sixth to eighth decades of life. […] Computed tomography examination, followed by magnetic resonance imaging and surgical biopsy with histopathological examination, confirmed the diagnosis and the presence of lung and inguinal lymph node metastases. […] Magnetic resonance imaging (MRI) is the imaging modality of choice in establishing the MFS diagnosis. […] The standard of care is represented by surgical resection with negative margins before diagnostic confirmation through a biopsy, followed by radiotherapy or chemotherapy for intermediate- and high-grade sarcoma types or rapidly growing cases. […] We performed a literature review based on the case of a female patient presenting a painful, giant, ulcerated myxofibrosarcoma of the left thigh, with a rapid-growing pattern and metastatic spread to the local lymph nodes and lung, which required rapid surgical resection followed by local reconstruction.

• #7 Myxofibrosarcoma – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/myxofibrosarcoma/

  Myxofibrosarcoma (MFS) is a type of cancer that typically appears as a slow-growing, painless lump on one of your legs or arms. […] MFS is not as well-known or understood as many other types of cancer, partly because the features distinguishing it from another soft tissue sarcoma came into focus only recently. […] Diagnosing an MFS can be a challenge. […] Even with advanced imaging, it can be difficult to accurately assess the true extent of the cancer. […] Tests to assess a suspicious-looking growth that may be an MFS include: Magnetic resonance imaging. Core needle biopsy. […] If these tests find an MFS or another type of soft tissue sarcoma, a chest X-ray is typically the next step. Its purpose is to determine whether the cancer has spread (metastasized) to your lungs, the most likely site for MFS metastasis.

• #8 Biology and Management of High-Grade Myxofibrosarcoma: State of the Art and Future Perspectives

  https://www.mdpi.com/2075-4418/13/19/3022

  Positron emission tomography/computed tomography (PET/CT) is not considered a standard imaging for staging and restaging STS, but it may exhibit a higher sensitivity in detecting distant metastases compared to conventional imaging in the initial stages. […] High-grade MFS is associated with very complex karyotypes lacking specific chromosomal aberrations. […] The progression in grade is accompanied by an increase in cytogenetic alterations. […] In general, high-grade MFS shows a higher amount of somatic copy number alterations than low-grade MFS. […] Surgical resection is the mainstay of treatment for localized MFS, although the use of RT or systemic therapies in conjunction with surgery may be considered in selected patients. […] The management of advanced MFS is challenging. Novel therapeutic approaches using immune-oncology and molecular targeted agents may lead to a substantial improvement in the outcomes of patients with this devastating disease.

• #9 FIBROSARCOMA – MYXOFIBROSARCOMA

  https://www.seyitaligumustas.com/en/fibrosarcoma-myxofibrosarcoma

  Patients diagnosed with fibrosarcoma will undergo a CT scan, whole-body MRI or PET-CT to detect metastases (most commonly to the lungs). […] Patients diagnosed with fibrosarcoma should be followed up for many years at regular intervals for recurrence and metastasis, which means spread to other parts of the body.

• #10 Undetected Myxofibrosarcoma in a Patient Diagnosed with Long Standing Complex Regional Pain Syndrome; A Case Report | Surgical Case Reports | Science Repository | Open Access

  https://www.sciencerepository.org/undetected-myxofibrosarcoma-in-a-patient-diagnosed-with-long-standing-complex-regional-pain-syndrome-a-case-report_SCR-2019-6-108

  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. […] 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. […] Given their rarity, they may be confused with lipomas, cysts, panniculitis or osteochondral lesions. […] 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 Myxofibrosarcoma pathology

  https://dermnetnz.org/topics/myxofibrosarcoma-pathology

  Myxofibrosarcoma is an uncommon soft tissue tumour, typically presenting in the lower limb girdle of the elderly. It is a malignant tumour with high risk of local recurrence. […] In general immunohistochemistry is unhelpful in myxofibrosarcoma, but is positive for vimentin, and sometimes weakly positive for smooth muscle actin and CD34. […] Epithelioid: Cellular areas morphologically epithelioid. Behave as high grade, and are prone to increased local recurrence and risk of metastasis. […] Malignant fibrous histiocytoma: This entity behaves like, and likely is synonymous with, high grade myxofibrosarcoma.

• #12 Final Diagnosis — Case 747

  https://path.upmc.edu/cases/case747/dx.html

  High grade sarcoma with features of myxofibrosarcoma. […] The differential diagnosis of myxoid lesions includes some benign entities-such as nodular fasciitis, myxoma, neurofibroma, neurothekeoma, and schwannoma-and malignant tumors such as myxoid liposarcoma, myxofibrosarcoma, and low-grade fibromyxoid sarcoma. […] In the present case however, the tumor was unquestionably malignant and the primary differential was between myxoid liposarcoma and myxofibrosarcoma. […] Myxoid liposarcoma and myxofibrosarcoma share many clinical and histological features making the differential diagnosis more difficult in some cases. […] In myxofibrosarcoma there can be high grade areas that also show nearly solid growth with little to no myxoid material between cells. […] A useful feature to aid in differentiation of these two tumors is the presence of the t(12;16)(q13;p11) translocation resulting in fusion of DDIT3 and FUS in more than 90% of myxoid liposarcomas.

• #13 Final Diagnosis — Case 747

  https://path.upmc.edu/cases/case747/dx.html

  In contrast, no characteristic molecular abnormality has been found in myxofibrosarcomas which usually show a highly complex karyotype. […] The presence of curvilinear vessels and a lack of lipoblasts favored a diagnosis of myxofibrosarcoma and this was confirmed by the lack of a DDIT3 translocation as determined by FISH.

• #14 Pathology Outlines – Myxofibrosarcoma

  https://www.pathologyoutlines.com/topic/softtissuemyxofibrosarcoma.html

  Myxofibrosarcoma includes a range of malignant fibroblastic tumors with myxoid stroma, variable pleomorphism and characteristic curvilinear vasculature (Surg Oncol Clin N Am 2016;25:775) […] Diagnosis is based primarily on histologic findings in correlation with age, site and clinical features […] Any undifferentiated pleomorphic sarcoma arising in an extremity of an elderly patient requires thorough sampling to find low grade areas of myxofibrosarcoma.

• #15 Diagnosis and Discussion — Case 1034

  https://path.upmc.edu/divisions/neuropath/bpath/cases/case410/dx.html

  High grade intracerebral myxofibrosarcoma. […] Myxofibrosarcoma is a common malignant fibroblastic neoplasm with variably myxoid stroma and represents one of the most common soft tissue sarcoma types in elderly patients. […] To the best of our knowledge eight cases of intracranial myxofibrosarcoma have been published until now in the English literature. […] Herein, we report the ninth case of a primary intracranial myxofibrosarcoma. […] The presence of prominent, elongated, thin-walled blood vessels with perivascular aggregates of neoplastic cells is considered a characteristic histological finding, especially in low-grade myxofibrosarcomas. […] World Health Organization (WHO) recommends a three-tier grading system (low-, intermediate- and high-grade tumors). […] The final diagnosis is based mainly on morphological, immunohistochemical and even molecular findings.

• #16 Myxofibrosarcoma | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/myxofibrosarcoma

  Tests and procedures used to diagnose myxofibrosarcoma include: […] Myxofibrosarcoma can be hard to diagnose because it can look like other types of cancer and other conditions that aren’t cancer. Careful imaging and lab tests can help your healthcare team make the correct diagnosis.

• #17 Myxofibrosarcoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/myxofibrosarcoma?content_id=CON-20357210

  Myxofibrosarcoma can be hard to diagnose because it can look like other types of cancer and other conditions that aren’t cancer. Careful imaging and lab tests can help your healthcare team make the correct diagnosis. […] Tests and procedures used to diagnose myxofibrosarcoma include: […] A biopsy procedure often involves using a needle to take some of the suspicious tissue for testing. The tissue goes to a lab where tests can show if you have cancer and what type. […] Myxofibrosarcoma treatment often involves surgery to remove the cancer. Other treatments include radiation therapy and chemotherapy. […] Myxofibrosarcoma can come back after surgery. This might be more likely if the cancer grows large or if lab tests show the cells are growing quickly. Your healthcare team may create a treatment plan with the goal of getting rid of all the cancer cells. […] Which treatment is best for you will depend on your cancer, such as the size and location of the myxofibrosarcoma.

• #18 Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model | Modern Pathology

  https://www.nature.com/articles/3800550

  Myxofibrosarcoma is one of the most frequent soft tissue tumours in elderly patients, mostly arising in the extremities. […] The differential diagnosis contains several other (benign) myxoid soft tissue tumours. […] Cytogenetic data on myxofibrosarcoma are scarce with only few isolated cases described in the literature. […] Local recurrences showed increase in grade compared to their primary lesions. […] Interestingly, these recurrences showed more complex cytogenetic aberrations. […] Increase in grade seems to parallel increase in cytogenetic aberrations and malignant potential. […] Based on these findings, we suggest that tumorigenesis of myxofibrosarcoma is mainly a multistep genetic process, probably ruled by genetic instability caused by targeted checkpoint genes. […] The differential diagnoses of myxofibrosarcoma include other myxoid tumours often hard to distinguish from each other, since differences are often very subtle.

• #19 Pattern of Recurrence of Myxofibrosarcoma is not Associated with Pattern at Presentation or Rate of Delayed Diagnosis

  https://brieflands.com/articles/ijradiology-13469

  The tendency of myxofibrosarcoma (MFS) to have a non-nodular appearance on magnetic resonance imaging (MRI) may present a challenge in detection of local recurrence. Appropriate index of suspicion of expected imaging appearance can alleviate this challenge. […] Imaging appearance at baseline does not reliably predict imaging appearance at recurrence. Therefore, a high index of suspicion for the infiltrative pattern is required in assessment of post-operative MRIs in patients with MFS. […] Our central hypotheses are that 1) The infiltrative pattern at recurrence is associated with a higher rate of delayed diagnosis than the focal or mixed patterns, and 2) The imaging appearance at recurrence can be predicted based on the appearance at presentation. […] The first objective of our study was to determine if our impression of the difficulty in interpreting these studies was supported by evidence of an increase in misdiagnosis rate associated with the infiltrative pattern of recurrence. The second objective of our study was to determine if the imaging pattern at recurrence was associated with the imaging pattern at presentation.

• #20 Myxofibrosarcoma of the scalp with difficult preoperative diagnosis: A case report and review of the literature

  https://www.wjgnet.com/2307-8960/full/v8/i11/2350.htm

  Myxofibrosarcoma (MFS) is a malignant fibroblastic tumor that tends to occur in the lower and upper extremities. The reported incidence of head and neck MFSs is extremely rare. […] A 46-year-old male with a neoplasm in the scalp was hospitalized and diagnosed with an MFS (highly malignant with massive necrotic lesions) based on histologic and immunohistochemistry evaluations. […] The magnetic resonance imaging manifestations did not demonstrate the tail sign mentioned in several studies, which resulted in a great challenge to establish an imaging diagnosis. […] The definitive diagnosis of MFS is based on immunohistologic features. […] Given the special anatomic location and the high malignant potential of this rare tumor, combined surgical and adjuvant radiotherapy should be considered to avoid local recurrence and distant metastasis.

• #21 Pattern of Recurrence of Myxofibrosarcoma is not Associated with Pattern at Presentation or Rate of Delayed Diagnosis

  https://brieflands.com/articles/ijradiology-13469

  Regarding objective 1, our results showed a trend for association between the infiltrative pattern and a delay in diagnosis of local recurrence. […] Therefore, a high index of suspicion is warranted for areas of linear signal abnormality on post-operative MRIs regardless of the imaging appearance at presentation.

• #22 :: JKSR :: Journal of the Korean Society of Radiology

  https://jksronline.org/DOIx.php?id=10.3348/jksr.2022.0005

  Myxofibrosarcoma is one of the most common soft tissue sarcomas in elderly patients. It often occurs in the extremities, trunk, and retroperitoneum. However, it is rarely observed in the mediastinum, and only a few cases have been reported in the literature. […] Herein, we present the imaging findings, with an emphasis on the MRI results, of a surgically confirmed anterior mediastinal myxofibrosarcoma in a 66-year-old male. […] To the best of our knowledge, there are only six cases of mediastinal myxofibrosarcoma reported in the literature. Of these, only three cases described MRI findings, and no diffusion-weighted imaging (DWI) with MRI was performed. […] In addition, we obtained DWI with MRI for tissue characterization. To our knowledge, this is the first report of DWI findings in mediastinal myxofibrosarcoma. […] DWI can be a useful sequence for tumor characterization when chest MRI is performed to evaluate mediastinal tumors. In addition, when an aggressive anterior mediastinal mass reveals a myxoid component on chest MRI, radiologists could suggest the possibility of myxofibrosarcoma.

• #23 Primary Myxofibrosarcoma of the Breast: Case Report and Review

  https://clinmedjournals.org/articles/ijpcr/international-journal-of-pathology-and-clinical-research-ijpcr-5-093.php?jid=ijpcr

  Soft tissue sarcomas of the breast are very rare tumors and account for less than 1% of all primary malignant breast tumors. […] We are reporting a diagnostic challenge of a case of primary breast myxofibrosarcoma and review literature on the best available evidence on diagnosis and management of primary soft tissue sarcomas of the breast. […] Core or excisional biopsy of the breast can differentiate this histology from other primary breast neoplasms. […] The optimal treatment is not well defined for primary sarcoma of the breast, and current recommendations are extrapolated from other STS data. […] Complete surgical resection with negative margins is strongly recommended. […] The role of chemotherapy in non-metastatic disease is not well documented. […] Adjuvant radiotherapy should be recommended to improve local control in cases in which the tumors are large (≥ 5 cm) or in tumors with positive surgical margins as extrapolated from data on other STS.

• #24 Giant Myxofibrosarcoma in the Lower Limb: An Overview of Diagnostic and Clinical Management

  https://www.mdpi.com/2075-4418/14/12/1298

  Patients with MFS should be treated in specialized centers, with an interdisciplinary tumor board evaluation before surgery being essential to increase survival, as in our case. […] Negative surgical margin resection (wide radical resection) is the standard of care for MFS, although it can be difficult considering the multidirectional spreading pattern of this tumor along the fascial planes. […] In many cases of intermediate- and high-grade MFS, adjuvant chemotherapy and radiotherapy are usually required to prevent recurrence and improve outcomes, but this topic remains controversial. […] Immunotherapy has been used for patients with MFS, especially for advanced cases, high-grade MFS, and those with distant metastases, showing the benefits of combination therapies by improving tumor response. […] Myxofibrosarcomas, especially those with deep infiltration, remain a challenge in daily practice due to their unpredictable clinical evolution; therefore, they should be treated in specialized centers by multidisciplinary teams.

• #25 Myxofibrosarcoma | Norton Children’s

  https://nortonchildrens.com/services/cancer/conditions/sarcoma/myxofibrosarcoma/

  Myxofibrosarcoma, also called MFS for short, is a rare cancer that forms in connective tissue. It typically appears as a small, painless lump under the skin of an arm or leg. It occurs in children but is most common in older adults. […] As myxofibrosarcoma grows, the lump can become painful and swollen. Like many childhood and adolescent cancers, myxofibrosarcoma can begin with vague symptoms that can be easily overlooked or attributed to other causes. Consulting with a pediatric cancer specialist offers the best opportunity for early diagnosis and successful treatment. […] Myxofibrosarcoma has the best chance of being cured when it’s treated by pediatric cancer specialists. Young adults may benefit from treatment at a children’s hospital instead of an adult-service hospital because of the care team’s expertise specifically in childhood diseases.

• #26

  https://winshipcancer.emory.edu/cancer-types-and-treatments/sarcoma/diagnosis.php

  An accurate sarcoma diagnosis is critical to achieving the best outcomes. […] The first step in making a sarcoma diagnosis is a thorough physical examination, followed by imaging. […] A critical component in accurately diagnosing a sarcoma is having an expert sarcoma pathologist review the biopsy tissue.

• #27 SciELO Brazil – Myxofibrosarcoma – Case report Myxofibrosarcoma – Case report

  https://www.scielo.br/j/abd/a/sPjk3wwXDQRgVMvJY7Pn9Rm/

  Myxofibrosarcoma is recognized as a malignant neoplasm of fibroblastic origin with increased prevalence in the elderly, presenting as nodules or tumors that may extend to the dermis and skeletal muscle, preferably in the lower limbs. […] The diagnosis of myxofibrosarcoma is hystopathological and tumors are classified as low, intermediate and high grade. […] It is important to note that skin biopsy, when taken superficially, may show misleading features, since the shallow portions of the tumor show benign characteristics, whereas deeper samples show histomorphological features of malignancy. […] Due to the low specificity of immunohistochemistry in myxofibrosarcoma, the results should always be interpreted with caution. […] Therapy consists of surgical resection with a safety margin of at least 2 cm.

• #28 Myxofibrosarcoma – DoveMed

  https://www.dovemed.com/diseases-conditions/myxofibrosarcoma

  The prognosis depends on a combination of factors including: Age of the individual, Grade of the tumor: A higher grade means a greater chance of metastasis and a poorer prognosis, Tumor stage at detection, Tumor size and location, Individuals response to therapy. […] Tumors that are small in size and at easily accessible locations generally have a good prognosis on complete surgical excision and removal. […] If the tumor recurs within a year of surgical removal, then it is an indicator of poor prognosis with increased mortality rates. […] According to studies, the key indicators of outcome include tumor size and grade, and the presence of infiltrating margins. […] Also, low-grade tumors may turn into high-grade tumors following treatment (removal), which can be associated with metastasis and consequently poorer outcomes.

• #29 Diagnosis and Discussion — Case 1034

  https://path.upmc.edu/divisions/neuropath/bpath/cases/case410/dx.html

  In summary, we present a case of a myxofibrosarcoma arising in an unusual location. Pathologists, radiologists and clinicians should be aware of the possibility to encounter in their daily praxis intracranial sarcomas, which may resemble clinically and radiologically more common intracranial tumors, such as glioblastomas. It is therefore of outmost importance the combination of morphological, immunohistochemical and in some cases even molecular studies in the establishment of the final diagnosis.

• #30 Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model | Modern Pathology

  https://www.nature.com/articles/3800550

  Therefore, the existence of a specific (cyto) genetic aberration could serve as a welcome diagnostic marker and a helpful tool to distinguish these subtypes. […] However, cytogenetic data on myxofibrosarcoma are scarce, with only a few isolated cases described in literature showing complex, but nonspecific cytogenetic aberrations. […] Here, we investigated the clinicopathologic and cytogenetic data of 32 myxofibrosarcoma in order to find tumour and/or grade specific cytogenetic aberrations. […] We showed that local recurrences occurred in all grades, with increase in grade upon recurrence, which is in accordance to the literature. […] We hypothesised that in the same lesion, increase in grade goes together with increase in cytogenetic aberrations. […] Recurrences showed increase in grade and more complex aberrations than the primary tumours. […] These complex aberrations suggest additional chromosomal events, corresponding with an increase in histological grade and, subsequently, increase in malignant potential.

• #31 Prognostic Factors and Outcomes for Patients With Myxofibrosarcoma: A 13-Year Retrospective Evaluation | Anticancer Research

  https://ar.iiarjournals.org/content/39/6/2985

  Myxofibrosarcoma (MFS) represents approximately 20% of all soft-tissue sarcomas, especially in elderly patients in their sixth to eighth decades of life. […] The aim of this study was to evaluate the prognostic factors affecting survival of patients with MFS, taking into account gender, tumour grade, state of the resection margin, local recurrence, use of radiotherapy, presence of metastases and blood levels of haemoglobin and C-reactive protein in a retrospective, single-centre analysis with a minimum follow-up period of 60 months (range=60-156 months). […] Tumour grade and metastasis were significant prognostic factors of survival (log-rank test p=0.041 and p=0.00007). […] The tumour grade and metastasis of MFS are independently associated with disease-specific survival, whereas negative surgical margins, local recurrence and blood levels of C-reactive protein and haemoglobin were not significant prognostic factors.

• #32 Prognostic Factors and Outcomes for Patients With Myxofibrosarcoma: A 13-Year Retrospective Evaluation | Anticancer Research

  https://ar.iiarjournals.org/content/39/6/2985

  The survival rate was significantly affected by the tumour grade and presence of metastases (Figures 3, 5 and 9). Patients with metastases had significantly worse overall survival than patients without metastases (hazard ratio=2.97, p=0.005) and than patients with G3 MFS (hazard ratio=1.56, p=0.024). […] The survival rate was highly dependent on the tumour grade, the achievement of negative margins during surgery, and metastasis. […] We calculated a 5-year survival rate of 78.3% for patients with negative margins. […] Local recurrence occurred independently of the state of the margins; in fact, most patients with local recurrence had histologically negative margins. […] We detected a worse 5-year survival rate in the group with local recurrence than in that without local recurrence, although the difference between these groups was not significant. […] We found similar percentages of 60.0% and 77.3%, respectively. Furthermore, we demonstrated an apparent but nonsignificant association between elevated CRP serum level and poor survival (p=0.084).

• #33 Analysis of prognostic factors in 171 patients with myxofibrosarcoma of the trunk and extremities: a cohort study

  https://atm.amegroups.org/article/view/77255/html

  Myxofibrosarcoma (MFS) of the trunk and extremities has unique clinical features. However, it is not clear which indicators are the influencing factors of recurrence, metastasis, and survival of trunk and limb MFS. The aim of the present study was to analyze clinical features and prognosis of trunk and limb MFS. […] The 3-year recurrence, 3-year metastasis, and 5-year survival rates were 29.2%, 19.3%, and 93.6%, respectively. Kaplan-Meier survival analysis showed that the surgical margin and tumor size were associated with recurrence. The surgical margin and CD44 expression were associated with metastasis. The multivariate analysis showed that the surgical margin and tumor size were risk factors for local recurrence. In addition, the surgical margin and presence of CD44 were risk factors for distant metastasis. […] A negative surgical margin can be reduced effectively the rate of recurrence and metastasis in patients with MFS of the trunk and limbs. In addition, CD44 may be used to assess the metastatic risk of patients with MFS.

• #34 Myxofibrosarcoma: Prognosis, Treatment & Staging

  https://my.clevelandclinic.org/health/diseases/22563-myxofibrosarcoma

  Myxofibrosarcoma treatment depends on several factors, including: Grade: Surgery is usually the only treatment needed for a low-grade myxofibrosarcoma. […] If you have a high-grade myxofibrosarcoma, you may have surgery along with other treatments such as radiation therapy. […] Healthcare providers use staging to determine how much cancer has spread. […] In general, higher-stage myxofibrosarcoma is more likely to require surgery along with other treatments. […] If the tumor is smaller than 2 inches (about 5 centimeters), its more likely that surgery will be your only treatment. […] You may need additional treatment, such as radiation therapy, for a larger tumor. […] A tumor that extends to your deeper tissues, such as between your muscles, may require surgery and radiation therapy.

• #35 Myxofibrosarcoma: A Diagnostic Pitfall

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3719110/

  Large resection represents the first choice of treatment. […] Due to the high recurrence and metastatic rate of MFH, adjuvant radiation therapy is recommended to treat eventual occult local micro metastases. […] The follow-up of MFS patients should be very thorough to manage properly eventual local recurrences. […] A large series of histochemical and immunohistochemical stainings is recommended. Extensive surgical excision with adjuvant radiotherapy presents the optimal therapeutic option.

• #36 Myxofibrosarcoma | Oncohema Key

  https://oncohemakey.com/myxofibrosarcoma/

  Myxofibrosarcoma is a unique subtype of soft tissue sarcoma with a locally infiltrative behavior. High-quality MRI imaging is critical for preoperative planning. […] Patients presenting with a diagnosis of myxofibrosarcoma should undergo high-quality T1- and T2-weighted MRI with pre- and postgadolinium imaging to accurately define the extent of the disease. […] Wide surgical resection with a 2 cm soft tissue margin encompassing the entire area of increased signal on T2-weighted MRI is required, which can require complex vascular and plastic surgery reconstruction. […] Myxofibrosarcoma can be graded under the FNCLCC (Fdration Nationale des Centres de Lutte Contre le Cancer) system. […] One of the major challenges of myxofibrosarcoma is to define the boundaries of the tumor. As with other soft tissue sarcomas of the extremity, MRI is the diagnostic modality of choice.

• #37 Myxofibrosarcoma: Prognosis, Treatment & Staging

  https://my.clevelandclinic.org/health/diseases/22563-myxofibrosarcoma

  No. Removing your arm or leg (amputation) used to be a common treatment for myxofibrosarcoma. […] Today, healthcare providers do everything they can to avoid amputation. […] After myxofibrosarcoma treatment, youll have regular follow-up imaging with your healthcare provider. […] If the tumor does return, regularly seeing your healthcare provider increases the chances of finding and treating myxofibrosarcoma early.

• #38 What Is Myxofibrosarcoma? – Klarity Health Library

  https://my.klarity.health/what-is-myxofibrosarcoma/

  The treatment plan will be tailored to your situation, aiming to effectively address the cancer while minimising potential side effects. […] Myxofibrosarcoma displays a higher tendency to recur post-treatment compared to other variants of soft tissue sarcomas. […] Regular consultations with your healthcare provider heighten the possibility of timely detection and intervention if myxofibrosarcoma reoccurs.

• #39 Malignant Soft Tissue Myxofibrosarcoma | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-319-65106-4_7

  Myxofibrosarcoma is an aggressive soft tissue neoplasm that is classified as a type of malignant fibrohistiocytic tumor. The combination of PET and MR imaging is helpful in determining the extent of local infiltration, defining surgical margins, and detection of small satellite lesions. PET/MRI is also useful in monitoring patients response to local and systemic therapy.

• #40 Primary Myxofibrosarcoma of the Breast: Case Report and Review

  https://clinmedjournals.org/articles/ijpcr/international-journal-of-pathology-and-clinical-research-ijpcr-5-093.php?jid=ijpcr

  A primary myxofibrosarcoma of the breast is a subtype of soft tissue sarcoma. The key features of this tumor are spindle cell proliferation with a moderate degree of nuclear pleomorphism, myxoid stroma and curvilinear blood vessels. Primary breast sarcomas constitute a specific clinicopathologic entity that carry different prognosis and, therefore should be differentiated from cystosarcoma phyllodes and metaplastic carcinomas. Central pathology review and multidisciplinary approach should be considered in management of such cases.

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