Materiały źródłowe

• #1 Myxofibrosarcoma: Prognosis, Treatment & Staging

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

  Myxofibrosarcoma is more common in adults over 50 and slightly more common in males than females. […] Other factors that can increase your risk of malignant soft tissue tumors, including MFS, include: Genetics: Some genetic conditions may increase your risk of soft tissue sarcomas. Environmental factors: Certain chemicals, such as arsenic and herbicides, can raise your risk. Radiation therapy: A history of radiation therapy may increase your risk of certain cancers, such as myxofibrosarcoma. […] Experts dont know the cause of myxofibrosarcoma. Like all types of cancer, it develops when a change happens in your cells DNA. This change tells your cells to multiply. As your cells multiply, they form an irregular mass called a tumor.

• #2 Myxofibrosarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/myxofibrosarcoma/

  Myxofibrosarcoma is one of the most common sarcomas of elderly patients, with a slight male predominance. Although the overall age range is wide, these neoplasms mainly affect patients in the sixth to eighth decades of life, whereas they only exceptionally rarely arise in patients aged < 30 years. [...] Etiology: Unknown.

• #3 Myxofibrosarcoma | Sarcoma UK

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

  Myxofibrosarcoma doesn’t have a known cause. Researchers are still trying to learn more about what causes it. […] It’s not clear how well chemotherapy works for people with myxofibrosarcoma. Some types of chemotherapy have shown to work well while others have not.

• #4 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) […] Etiology: Unknown […] Increased risk of development in Werner syndrome […] Association with Muir-Torre syndrome (Hered Cancer Clin Pract 2021;19:34).

• #5 Myxofibrosarcoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/myxofibrosarcoma/

  Myxofibrosarcoma is a type of cancer that begins in the connective tissue. […] The cause of myxofibrosarcoma often isn’t known. This cancer starts as a growth of cells in the connective tissue. […] Myxofibrosarcoma happens when cells develop changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. […] In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells. The cancer cells can form a lump that may grow into healthy body tissue.

• #6 Mayo Clinic Health Library – Myxofibrosarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20357210

  The cause of myxofibrosarcoma often isn’t known. This cancer starts as a growth of cells in the connective tissue. […] Myxofibrosarcoma happens when cells develop changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. […] In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells. The cancer cells can form a lump that may grow into healthy body tissue.

• #7

  https://www.sydneysarcomaunit.com.au/myxofibrosarcoma

  Myxofibrosarcoma development is still poorly understood. It results due to DNA mutations in connective tissue cells leading to uncontrolled growth and formation of mutated cells, resulting in tumour formation. […] The use of radiotherapy for these tumours is common. Radiotherapy (either before or after surgery) is useful in the management of myxofibrosarcomas. It has been shown to reduce the chance of the tumour re-occurring locally after surgical resection. […] Chemotherapy is reserved for select cases, studies have shown benefit from chemotherapy following surgical resection for larger myxofibrosarcomas.

• #8 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. […] Currently, the molecular pathogenesis of MFS is still poorly understood and its genomic profile exhibits a complex karyotype with a number of aberrations including amplifications, deletions and loss of function. […] From a molecular point of view, they exhibit highly complex karyotypes with a number of different genomic aberrations. […] At present no specific molecular pathology markers are available for the standard differential diagnosis, which is based on the analysis of cytomorphologic findings. […] Recently, genomic and transcriptomic profiling have shed light on the molecular alterations of this disease through the identification of new targets with promising results.

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

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

  However, at present there are no specific immunohistochemical markers for the standard differential diagnosis of MFS since they are generally characterized by highly complex karyotypes and show many of the aberrations observed in other STS. […] Some amplifications have been reported in chromosomes 5p (rapamycin-insensitive binding partner of mTOR, CDH9, LIFR) and 1p/1q (PI4KB, ETV3, MCL1), as well as deletions of tumor-suppressor genes (CDKN2A/B, TP53) and loss-of-function mutations in NF1 and PTEN. […] In particular, adult STSs show low somatic mutation burdens while harboring many more copy-number alterations than most other cancer types. […] The presence of TP53 alteration and KRAS amplification was also significantly associated with poorer overall survival. […] Myxofibrosarcoma is characterized by the highest rate of recurrence among all STS, ranging from 20-60% after 5 years, depending on the case series.

• #10 Modeling Myxofibrosarcoma: Where Do We Stand and What Is Missing?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10650645/

  Myxofibrosarcoma (MFS) is a malignant soft tissue sarcoma (STS) that originates in the body’s connective tissues. It is characterized by the presence of myxoid (gel-like) and fibrous components and typically affects patients after the fifth decade of life. […] MFS has several peculiar features. The tumor is lobulated and heterogeneous. There is variability in cellular content, with a tendency to become more cellularized and of higher grade over time as a result of local recurrence. […] Specific cytogenetic alterations have not been found so far, but MFS exhibits complex structural and numerical aberrations indicative of genetic instability developed in a multistep tumor progression. […] Furthermore, the same patient can have intratumoral mutational heterogeneity. […] In patients with localized disease, primary treatment is represented by surgical resection of the tumor, which can be combined with radiotherapy delivered either in the neoadjuvant or adjuvant setting.

• #11 Myxofibrosarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Myxofibrosarcoma

  Causes Unknown […] Most cases of MFS have tumor cells that contain complex chromosome and/or gene abnormalities including ring chromosomes, double minutes, chromosomes with deletions of part of their genetic material, and chromosome translocations. […] Tumor cell mutations or deletions in the NF1 gene occur ~10% of MFS cases while mutations in the CDKN2A/CDKN2B and amplifications in the CDK6, CCND1, and MDM2 genes occur in rare MFS cases. […] The chromosome/gene abnormalities have not yet been defined in the epithelioid variant of MFS.

• #12 Myxofibrosarcoma – DoveMed

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

  Myxofibrosarcoma (MFS) forms a group of malignant soft tissue tumors that are commonly observed in older adults. It is also referred to as Myxoid Malignant Fibrous Histiocytoma. […] The cause of Myxofibrosarcoma occurrence is unknown and no risk factors have been reported. […] Currently, the specific risk factors for Myxofibrosarcoma are yet to be identified. […] The exact cause and mechanism of formation of Myxofibrosarcoma is unknown. […] Some reports indicate that they may occur due to genetic defects. […] A minority of the tumors (about 1 in 10 tumors) display gene mutations and deletions similar to those seen in neurofibromatosis type 1 (NF1). […] NF1 is a genetic disorder affecting the skin and other body systems. Research is being undertaken to determine the significance of such similar mutations.

• #13 Aetiology And Risk Factors For Myxofibrosarcoma – Klarity Health Library

  https://my.klarity.health/aetiology-and-risk-factors-for-myxofibrosarcoma/

  Myxofibrosarcoma (MFS) is a subset of malignant soft tissue tumours seen mainly in elderly patients. […] Currently, there is no known exact cause of myxofibrosarcoma. […] While most people who get soft tissue sarcoma have no known risk factors, several things make one person to be at a higher chance of developing soft tissue sarcoma than another. […] Age and sex remain the most expressive risk factors for myxofibrosarcoma; the disease affects mostly adults above 50 years and is seen more in males. […] Individuals who have received high-dose radiation to treat other malignancies, such as breast or cervical cancer, have a slightly increased risk of developing soft tissue sarcoma. […] Chronic lymphoedema following radiation to lymph nodes or surgical removal of lymph nodes is an added risk.

• #14 What Is Myxofibrosarcoma? – Klarity Health Library

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

  The precise origins of most soft tissue sarcomas remain unclear. […] The initiation of soft tissue sarcoma occurs when there are modifications in the DNA of connective tissue cells. DNA carries the instructions guiding cellular activities. These changes convert the connective tissue cells into cancerous cells. This alteration instructs the cancer cells to proliferate and generate more cells. […] Certain factors are associated with an elevated risk of developing sarcoma, including: […] Genetic conditions that heighten the risk encompass hereditary retinoblastoma, Li-Fraumeni syndrome, familial adenomatous polyposis, neurofibromatosis, tuberous sclerosis, and Werner syndrome. […] Exposure to specific chemicals can increase susceptibility to soft tissue sarcomas. Notable among these chemicals are herbicides, arsenic, and dioxin.

• #14 What Is Myxofibrosarcoma? – Klarity Health Library

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

  Individuals who have undergone radiation therapy for different types of cancers have a higher likelihood of developing soft tissue sarcomas as a consequence of the radiation treatment. […] Specific genetic conditions can heighten the susceptibility to developing soft tissue sarcomas. […] Exposure to certain chemicals, such as arsenic and herbicides, is associated with an increased risk. […] A history of undergoing radiation therapy can amplify the likelihood of developing particular cancers, including myxofibrosarcoma.

• #15 Risk Factors for Soft Tissue Sarcomas | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/causes-risks-prevention/risk-factors.html

  Radiation exposure accounts for less than 5% of sarcomas. But patients might develop sarcomas from radiation given to treat other cancers, like breast cancer or lymphoma. The sarcoma often starts in the part of the body that was treated with radiation. The average time between the radiation treatments and the diagnosis of a sarcoma is about 10 years. […] Family cancer syndromes are disorders caused by gene defects (mutations) that people are born with (often inherited from a parent) that are linked to a high risk of getting certain cancers. Some family cancer syndromes increase a person’s risk of developing soft tissue sarcomas. […] Neurofibromatosis is also known as von Recklinghausen disease. It usually runs in families and causes many benign (not cancer) tumors that form in nerves under the skin and in other parts of the body (These are called neurofibromas.) It’s caused by a defect (mutation) in genes called NF1 and NF2. About 5% of people with neurofibromatosis will develop a sarcoma in a neurofibroma.

• #16 Risk Factors for Soft Tissue Sarcomas | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/causes-risks-prevention/risk-factors.html

  Li-Fraumeni syndrome is caused by inherited defects in the TP53 gene. People affected by this syndrome have a high risk of cancer, such as breast cancer, brain tumors, leukemia, and sarcomas. Still, only 10 to 20 out of 100 people with Li-Fraumeni syndrome will develop a soft tissue sarcoma. […] Children with this gene defect also have a higher risk of developing bone or soft tissue sarcomas, especially if the retinoblastoma was treated with radiation. […] Werner syndrome is caused by defects in the RECQL2 gene. Children with this syndrome have problems like those seen in the elderly. They also have an increased risk of cancer, including soft tissue sarcomas. […] Gorlin syndrome is also called nevoid basal cell carcinoma syndrome (NBCCS). It’s caused by defects in the PTCH1 gene. People with this syndrome have a high risk of developing many basal cell skin cancers. They also have an increased risk of fibrosarcoma and rhabdomyosarcoma.

• #17 Risk Factors for Soft Tissue Sarcomas | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/causes-risks-prevention/risk-factors.html

  People with tuberous sclerosis have an increased risk of rhabdomyosarcoma. […] Exposure to vinyl chloride (a chemical used in making plastics) is a risk factor for developing sarcomas of the liver, but it hasn’t been proven to cause soft tissue sarcomas. Arsenic has also been linked to a type of liver sarcoma but not soft tissue sarcoma. Exposure to dioxin and to herbicides that contain phenoxyacetic acid at high doses may also be risk factors, but this isn’t known for certain.

• #18 Myxofibrosarcoma: Causes, Symptoms, and Treatment Options

  https://sehathub.com/myxofibrosarcoma

  The exact cause of MFS is unknown. Like all other types of cancer, it can develop with specific mutations or modifications in the cellular material. A change happens in the DNA of the connective tissue cells. This change commands your cells to multiply irregularly and grow abnormally without dying, causing them to accumulate and form a tumor mass. […] Some factors can increase the risk of MFS. The risk factors include: […] Environmental Factors Some specific environmental factors like chemicals (herbicides, arsenic) can increase the risk of developing MFS. […] Genetic Factors Mutations of certain genes can raise the risk of STS. […] Treatment-Related Factors A history of radiation therapy can increase the risk of developing MFS in individuals. […] Age Older age is also a risk factor for developing MFS. […] Family History People with a family history of STS or any other cancer are more inclined to develop MFS.

• #19 Myxofibrosarcoma – Vejthani Hospital | JCI Accredited International Hospital in Bangkok, Thailand.

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

  Myxofibrosarcomas cause is unknown, but it, like other forms of cancer, occurs due to a mutation in the DNA of cells, prompting them to replicate. This replication leads to the formation of an abnormal mass known as a tumor. […] Several factors, including MFS, can raise the risk of developing malignant soft tissue tumors. […] Certain hereditary disorders may increase the risk of soft tissue sarcoma. […] Exposure to herbicides and arsenic are two pollutants that could potentially increase the risk. […] Previous radiation therapy may also increase the risk of developing myxofibrosarcoma.

• #20 Idiopathic and radiation-induced myxofibrosarcoma in the head and neck—case report and literature review | Chinese Neurosurgical Journal | Full Text

  https://cnjournal.biomedcentral.com/articles/10.1186/s41016-021-00267-9

  Myxofibrosarcoma (MFS), especially radiation-Induced MFS (RIMFS) in the head and neck, is an extremely rare malignant fibroblastic tumor. […] MFS is a rare soft tissue sarcoma that can arise sporadically or be induced by radiation, representing approximately 5% of all sarcomas. […] Radiation-induced sarcomas (RIS) are increasingly seen in long-term survivors of head and neck tumors, with an estimated risk of up to 0.3%. Common histologic subtypes of RIS parallel their idiopathic counterparts and mainly include osteosarcoma, chondrosarcoma, malignant fibrous histiocytoma, and fibrosarcoma. […] Radiation-induced MFS is very rare; only 3 cases have been reported until now. The diagnosis of RIS requires the following criteria: (1) history of radiotherapy; (2) asymptomatic latency period of several years (conventionally, 4 years); (3) occurrence of sarcoma within a previously irradiated field; and (4) histological confirmation of the sarcomatous nature of the post-irradiated lesion. […] Our case met all the criteria for RIS, including the development of myxofibrosarcoma within the radiation field, a 10-year latent period, and a different histopathological type.

• #21 Myxofibrosarcoma – Symptoms, Treatment & Support – Without a Ribbon

  https://withoutaribbon.org/myxofibrosarcoma-symptoms-treatment-support/

  Myxofibrosarcoma is a rare, slowly growing aggressive tumor that occurs in the soft tissues of the body. […] There is no well-known cause, but a prior history of trauma or trauma is a risk factor for the development of a tumor in the affected area in the future.

• #22 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

  Low incidence of sarcomas, a previous CRPS-1 diagnosis of 14 years and inconclusive biopsies led to this misdiagnosis. […] The association between CRPS-I and malignancies is unclear. Sarcomas are thought to arise de novo, but it has been suggested that chronic irritation, inflammation and lymphedema may induce development of a malignancy. […] It seems unlikely that the FMS was the initial trigger for development of CRPS-I, since the first CRPS-I symptoms dated from 14 years before the diagnosis of the FMS, which usually exhibits an aggressive loco-regional behavior with rapid growth.

• #23 FIBROSARCOMA – MYXOFIBROSARCOMA

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

  Fibrosarcoma can develop spontaneously, but they can also be caused by burns or radiation. […] Chemotherapy is not routinely used in patients with fibrosarcoma. Chemotherapy is used for large and deep-seated tumors, especially in the presence of metastases.

• #24 Fibrosarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1257520-overview

  No definite cause of fibrosarcoma is known, though genetic mutations may play a role. Research has shown that many sarcomas are associated with such mutations. The more common genetic defects include allele loss, point mutations, and chromosome translocations. […] Several inherited syndromes are associated with sarcomas. For example, patients with multiple neurofibromas may have a 10% lifetime risk of developing a neurosarcoma or a fibrosarcoma. […] The occurrence of fibrosarcoma in conjunction with metallic implants used for fracture fixation or joint reconstruction has been reported, albeit very rarely. The cause of this transformation is unknown. […] Preexisting lesions from which secondary fibrosarcoma may arise include bone infarcts and lesions associated with fibrous dysplasia, chronic osteomyelitis, and Paget disease, as well as previously irradiated areas of bone. This form of fibrosarcoma is very aggressive and is associated with a much poorer outcome than that associated with primary fibrosarcoma of bone.

• #25 Modeling Myxofibrosarcoma: Where Do We Stand and What Is Missing?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10650645/

  MFS has a peculiar infiltrative pattern, especially in superficial forms, which tend to grow into the contiguous soft tissues along normal planes, such as the fascial planes and vascular planes at microscopic and macroscopic levels. […] Because of the propensity to infiltrate surrounding tissues, MFS is surgically removed with wide-margin resections. Such surgeries create large defects that require reconstruction surgery using free or rotational flaps and skin grafts. […] The rate of local recurrence (LR) is particularly high compared to other STS. […] In 40-60% of the patients, the tumor recurs more than once; therefore, the patient is subjected to several local surgeries, with great discomfort for the patients and an increased cost for the health system. […] Furthermore, each time the tumor recurs, it can show an increase in the histological grade, leading to decreased response to radio- and chemotherapy.

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

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

  Noteworthy, from 15-38% of MFS local recurrences evolve to higher histological grade disease with increased metastatic potential. […] The overall incidence of distant metastases is estimated to be between 20% and 25%. […] In 2015, Emori et al. performed immunohistochemical staining on specimens from 37 MFS patients showing that CD109 expression—a TGF- co-receptor whose frequency was approximately 10%—was associated with poor prognosis. […] In 2017, De Vita et al. performed CD109 analysis on three high-grade MFS showing its role as a promising marker for the identification of more aggressive high-grade MFS and a potential therapeutic target. […] More recently, Sugiura et al. evaluated the prognostic value of CD34 expression status in 192 patients with MFSs and UPS. […] The authors also suggest two possible mechanisms, with one being related to CD34-positive stromal fibroblastic/fibrocytic cells (CD34+SFCs), which synthesize and remodel the extracellular matrix thereby contributing to fibrosis and tumor stroma formation.

• #27 Modeling Myxofibrosarcoma: Where Do We Stand and What Is Missing?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10650645/

  Additionally, these metastases further impact on overall survival. […] Potential prognostic predictor factors are grade, appropriate margins, primary unplanned resection at another facility, age, size, and depth. […] Thus, for MFS patients, the current therapeutic options are inadequate, and there is a constant effort to develop innovative therapeutic strategies. […] In both cases, preclinical models can be extremely useful to identify innovative therapeutic strategies. […] MFS cell lines have been proven to be sensitive to classical drugs such as trabectidin, epirubicin, cisplatin, and doxorubicin. […] Altogether, these results highlight the importance of immortalized MFS cells grown in monolayer cultures in preliminary drug screening, and promising drugs should be further tested in more complex preclinical models to speed up the translation of these results toward the clinical application and to increase the spectrum of therapeutic agents that can be used to control MFS growth. […] MFS in vivo models constitute fundamental tools to test the efficacy of therapeutic strategies to control a tumor’s growth locally. […] For example, to our knowledge, no zebrafish models have been developed to visualize the migration and invasion of MFS cells.

• #28 Analysis of clinical factors impacting recurrence in myxofibrosarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-024-53606-y

  Myxofibrosarcoma (MFS) is a malignant fibroblastic/myofibroblastic neoplasm with a prominent myxoid area. It has the clinical features of frequent local recurrence (LR) and occasional distant metastasis. […] The significant factors identified by univariate analysis were subsequently analyzed via multivariate analysis. […] The probability of LR in patients with MFS was significantly greater in association with unplanned operations, positive margins, low serum CK levels or superficial tumor depth. […] Diagnosis and treatment at qualified regular medical centers can reduce the local recurrence rate of MFS. […] The incidence of metastatic disease in patients with MFS has been documented in the literature, with reported rates ranging between 15 and 38%. Factors such as histological grade and tumor size have been proposed as potential determinants of metastatic risk.

• #29 Primary cardiac myxofibrosarcoma: case report, literature review and pooled analysis | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4434-2

  Primary cardiac myxofibrosarcoma is a very rare cardiac malignancy. The prognosis of these tumors is poor and is much influenced by the surgical possibilities. Statistical analyses revealed that primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Tumors 40 mm in size or with high-grade had significantly worse prognosis, which should be early diagnosed and treated with rational surgery. […] Primary cardiac myxofibrosarcoma is a malignant tumor of heart. Statistical analyses revealed that primary cardiac myxofibrosarcoma were more likely to present with local recurrences and dismal metastases. Tumors 40 mm in size or with high-grade had significantly worse prognosis.

• #30 Analysis of clinical factors impacting recurrence in myxofibrosarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-024-53606-y

  Numerous studies to date have identified factors such as surgical margins and location at diagnosis as significant predictors of LR. […] In contrast to the findings of previous studies, which described superficial tumors as having no substantial impact on recurrence, our study revealed that superficial tumors were associated with a heightened probability of recurrence. […] Myxofibrosarcomas are categorized into two distinct subtypes: superficial and deep. […] A planned operation was defined as a procedure that was thoroughly evaluated at our center, planned, and executed according to the results of comprehensive imaging examinations. […] In this study, we found that the likelihood of recurrence in patients who underwent planned surgery was significantly lower than that in patients who underwent unplanned surgery.

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

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

  Tumor-related mortality and morbidity, and the risk of distant metastasis development, are related to the MFS histological grade. […] The main differential diagnosis is represented by but not limited to malignant peripheral nerve sheath tumors, myxoma, low-grade fibromyxoid sarcoma, pleiomorphic leiomyosarcomas, pleomorphic rhabdomyosarcomas, spindle cell lipoma, pleomorphic or dedifferentiated liposarcoma, melanoma, undifferentiated carcinoma, undifferentiated sarcoma, and nodular fasciitis. […] Studies have shown that MFS, irrespective of surgical margin resection status, location, size, depth, or grade, leads to LRs in 22–79% of cases. […] It appears that systemic inflammation (high level of serum C-reactive protein and high neutrophil–lymphocyte ratio) in the presence of high-grade MFS of the extremities leads to a worse prognosis.

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

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

  The other hypothesis is that CD34 suppresses tumor development, in which case it could be used for the development of novel therapeutic agents. […] Interestingly, an online specialized tool named Online consensus Survival analysis for MyxoFibroSarcoma (OSmfs) has been developed to evaluate the prognostic value of genes in MFS, utilizing gene expression profiling and clinical follow-up data of MFS cases from three independent cohorts. […] In particular, it has been shown that metastasis-related signatures of UPS and MFS are mainly related to forebrain development and fatty acid degradation. […] However, a major limitation of cell line-derived mouse xenografts is that they do not maintain the complexity of tumor microenvironment of the original lesion, which can only be obtained with heterotransplanted tumors. […] Despite the fact that many efforts have been put into sarcoma research in recent years, the advances made in unraveling the pathogenesis and development of MFS have not led to a substantial improvement of the clinical outcome.

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