Materiały źródłowe

• #1 Epidemiology and survival factors of appendicular myxofibrosarcoma: a SEER-retrospective study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10954266/

  The low incidence of myxofibrosarcoma (MFS) makes high power studies difficult to perform. […] The purpose of this study was to characterize a large cohort of patients with MFS and evaluate epidemiologic and survival factors when compared to all STS. […] Overall survival was greater for STS than MFS (79% vs. 67%). […] Patients with MFS had a higher average age at diagnosis than STS (62 vs. 56), and older age was strongly associated with decreased survivorship for MFS (HR = 7.94). […] The incidence of MFS and STS increased over the 15-year period, with MFS increasing at a greater rate than STS (1.25% vs. 2.59%). […] Monitoring changes in demographic and survival trends for rare STS subtypes like MFS is important to improve diagnostic algorithms and treatment options. […] Myxofibrosarcoma (MFS) is a rare malignant soft tissue sarcoma (STS) subset of the heterogenous group of fibrohistiocytic tumors with an estimated prevalence of 0.1/100,000/years.

• #2 Epidemiology and survival factors of appendicular myxofibrosarcoma: a SEER-retrospective study | Dahl | Reports of Practical Oncology and Radiotherapy

  https://journals.viamedica.pl/rpor/article/view/97733

  Myxofibrosarcoma (MFS) is a rare malignant soft tissue sarcoma (STS) subset of the heterogenous group of fibrohistiocytic tumors with an estimated prevalence of 0.1/100,000/years. […] To date there have been no large-scale population-based studies comparing outcomes, survival factors, and prognostic factors of MFS to all STS. […] Our study confirmed that surgical excision greatly increases survival. […] Our results also showed that the sensitivity of MFS to radiotherapy is unclear, given that high-risk tumors will typically receive (neo)adjuvant therapy in addition to resection. […] Our results also indicated that survival rates for STS and MFS are increasing over time, with MFS survival increasing at a higher rate. […] This analysis of the SEER database contributes to the breadth and scope of prognostic data available for clinical decision making in patients diagnosed with MFS.

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

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

  Myxofibrosarcoma (MFS), one of the undifferentiated pleomorphic sarcomas (20% of adult sarcomas), is known as an aggressive soft tissue sarcoma (STS) frequently located in subcutaneous and deep tissue areas. The incidence is about 0.1 per 100.000 per year (versus 2–5 per 100.00/year for all soft tissue sarcomas). MFS commonly affects people in the sixth to eighth decades of life, having a slight predisposition in male patients. Although MFSs are the most common sarcomas, data about the incidence in relation to age group are scarce, with studies showing that cases under 20 years are extremely rare. Patient age has a reported median of between 42 and 66 years. The lower extremity is usually involved, followed by the upper extremity (combined, 77%), the trunk (12%), and the neck (3%), with other locations being rarely implicated. It typically shows distant metastasis to the lungs, and rarely to the bones, lymph nodes, or liver. MFSs located in soft tissues, mainly in the extremities, have a higher rate of local recurrence but a lower rate of metastasis compared with other sarcomas.

• #4 Myxofibrosarcoma | SpringerLink

  http://link.springer.com/10.1007/978-3-319-28845-1_5434-1

  Myxofibrosarcoma is a rare soft tissue sarcoma with an estimated incidence of 0.1/100.000/year (Stiller et al. 2013). Nevertheless, it is one of the most common sarcomas in adulthood. […] Myxofibrosarcoma affects adults, mostly elderly patients (peak of incidence in the seventh decade). However, cases occur in young adults, but almost never in children (Mentzel et al. 1996; Merck et al. 1983). […] There is a slight male predominance (Mentzel et al. 1996; Merck et al. 1983). […] About two-thirds of myxofibrosarcomas develop within the dermis and subcutis while the remaining one-third in deep (subfascial) soft tissues. Most myxofibrosarcomas affect lower extremities (thigh, buttock, leg, and knee), followed by upper extremities (arm, shoulder, and forearm), trunk, and seldom…

• #5 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. [...] Myxofibrosarcoma is a malignant fibroblastic neoplasm characterized by diverse cellularity, myxoid stroma, and curvilinear vasculature, commonly affecting elderly patients.

• #6 Myxofibrosarcoma

  http://www.thedoctorsdoctor.com/diseases/myxofibrosarcoma.htm

  Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients. […] Myxofibrosarcoma (MFS) is one of the most common soft tissue sarcomas of elderly patients and has a predilection for the limbs. […] Myxofibrosarcoma is a malignant tumor with distinctive histologic features and is believed to be derived from fibroblasts. […] Myxofibrosarcoma is one of the most frequent soft tissue tumours in elderly patients, mostly arising in the extremities. […] Local recurrence in 33 cases (54%) […] 13/60 patients developed metastases […] 13 tumor-related deaths occurred […] Short interval to first local recurrence was associated with poor clinical outcome.

• #7 Myxofibrosarcoma | Basicmedical Key

  https://basicmedicalkey.com/myxofibrosarcoma/

  Myxofibrosarcoma represents a spectrum of malignant fibroblastic neoplasms with variably myxoid stroma and characteristic elongated curvilinear vessels. […] One of most common sarcomas in elderly patients. […] 2/3 of cases arise in dermal/subcutaneous tissues. […] Local, often repeated, recurrences in up to 50-60% of cases (unrelated to histologic grade). […] Intermediate- and high-grade malignant neoplasms may develop metastases in 30-35% of cases. […] Incidence: One of most common sarcomas in elderly patients. […] Affects mainly patients in 6th to 8th decade. […] Exceptionally rare in patients 20 years old. […] Slight male predominance. […] 2/3 of cases arise in dermal/subcutaneous tissues. […] Local, often repeated, recurrences in up to 50-60% of cases (unrelated to histologic grade). […] Intermediate- and high-grade MFS may develop metastases in 30-35% of cases. […] Overall 5-year survival is 60-70%.

• #8 Myxofibrosarcoma of The Abdominal Wall at Moewardi Hospital, Surakarta: A Rare Case Report | Soewoto | Indonesian Journal of Cancer

  https://www.indonesianjournalofcancer.or.id/e-journal/index.php/ijoc/article/view/1018

  Myxofibrosarcoma (MFS) is a fibroblast-derived sarcoma, which accounts for approximately 510% of all soft tissue malignant tumors. The mean age in patients with MFS is between the fifth and seventh decades. Around 77% of MFS cases occur in the upper extremities. Other areas of the body including the trunk (12%), retroperitoneum or mediastinum (8%), abdominal wall, and heart have also been reported. […] Recognizing the histopathological features of MFS and applying the appropriate immunohistochemical examinations are crucial in establishing the correct diagnosis. This case provides a diagnosis and treatment experience of MFS that occurs in the abdominal wall.

• #9 Myxofibrosarcoma | Oncohema Key

  https://oncohemakey.com/myxofibrosarcoma/

  Myxofibrosarcoma is a unique subtype of soft tissue sarcoma with a locally infiltrative behavior and a predilection for local recurrence. […] This represents approximately 5% of soft tissue sarcoma diagnoses. […] Series of MFS and myxoid MFH studies describe these tumors as having a significant propensity for local recurrence, while exhibiting an overall better prognosis when compared with other complex karyotype sarcomas such as leiomyosarcoma. […] Given its relatively recent recognition as a distinct pathologic entity, the clinical behavior and outcomes for patients with MFS are uncertain, and there are no randomized trials to guide treatment protocols. […] Most cases of MFS demonstrate a highly complex karyotype, often with triploid or tetraploid alterations. […] MFS usually affects older patients. […] The most common sites of presentation are the extremities (77%), with a predilection for the lower extremities. […] MFS is uncommon in the abdominal cavity and retroperitoneum. […] One of the major challenges of myxofibrosarcoma is to define the boundaries of the tumor.

• #10 Epidemiology and survival factors of appendicular myxofibrosarcoma: a SEER-retrospective study | Dahl | Reports of Practical Oncology and Radiotherapy

  https://journals.viamedica.pl/rpor/article/view/97733

  The low incidence of myxofibrosarcoma (MFS) makes high power studies difficult to perform. […] The purpose of this study was to characterize a large cohort of patients with MFS and evaluate epidemiologic and survival factors when compared to all STS. […] We performed a retrospective review of the Surveillance, Epidemiology, and End Results (SEER) database from 2000 to 2015 to identify 1,440 patients diagnosed with MFS and 12,324 with STS. […] Overall survival was greater for STS than MFS (79% vs. 67%). […] The incidence of MFS and STS increased over the 15-year period, with MFS increasing at a greater rate than STS (1.25% vs. 2.59%). […] Monitoring changes in demographic and survival trends for rare STS subtypes like MFS is important to improve diagnostic algorithms and treatment options.

• #11 Epidemiology and survival factors of appendicular myxofibrosarcoma: a SEER-retrospective study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10954266/

  Current clinical management for MFS is dependent on characteristics of the malignancy, including depth, size, grade, and episodes of previous recurrence. […] Identifying prognostic characteristics is vital for accurate clinical decision making, especially considering the variation of STSs in clinical behavior and treatment. […] To date there have been no large-scale population-based studies comparing outcomes, survival factors, and prognostic factors of MFS to all STS. […] Our study confirmed that high grade tumors, higher stages, and presence of metastasis at diagnosis were all strongly associated with decreased survival. […] Our results also showed that the sensitivity of MFS to radiotherapy is unclear, given that high-risk tumors will typically receive (neo)adjuvant therapy in addition to resection. […] This could indicate that diagnostic accuracy of MFS is improving, as they have historically been a diagnostic challenge both clinically and pathologically. […] This could also be associated with greater awareness of the condition, earlier detection and surgical excision, or new treatment options.

• #12 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) […] Affects elderly in the sixth to eighth decades of life (Cancer 2012;118:518) […] Rare under 30 years […] Slight male predominance […] Dermal / subcutaneous location: > 50% of cases […] Deep / fascial in 33% of cases.

• #13 Myxofibrosarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/myxofibrosarcoma/symptoms-causes/syc-20577507

  Myxofibrosarcoma happens most often in adults ages 50 to 70. It is rare in people under age 30. […] The risk of myxofibrosarcoma is higher in adults. […] There is no way to prevent myxofibrosarcoma.

• #14 Myxofibrosarcoma: Prognosis, Treatment & Staging

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

  Myxofibrosarcoma is more likely to return after treatment than other types of soft tissue sarcomas. Follow-up imaging increases your chances of treating MFS early. […] Myxofibrosarcoma is more common in adults over 50 and slightly more common in males than females. […] Myxofibrosarcoma is rare. Its one of the most common types of soft tissue sarcomas. However, soft tissue sarcomas make up only about 2% of all cancer diagnoses. […] Myxofibrosarcoma is more likely to come back after treatment than other types of soft tissue sarcomas. Up to 1 in 2 people have myxofibrosarcoma return within five years of treatment. […] After myxofibrosarcoma treatment, you’ll 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. […] Myxofibrosarcoma has better survival rates than some other types of soft tissue sarcomas. In one study, most people with myxofibrosarcoma lived five years or longer after treatment.

• #15 What Is Myxofibrosarcoma? – Klarity Health Library

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

  Myxofibrosarcoma (MFS) is categorized as a type of soft tissue sarcoma cancer that originates in the body’s connective tissues. […] Given its aggressive behaviour, timely diagnosis, treatment, and ongoing monitoring are vital in effectively managing myxofibrosarcoma. […] Myxofibrosarcoma is prevalent among individuals aged over 50 and is slightly more frequent in those designated male at birth (DMAB) compared to those designated female at birth (DFAB). […] Myxofibrosarcoma is considered a rare condition despite being among the more prevalent forms of soft tissue sarcomas. However, it’s important to note that soft tissue sarcomas constitute merely around 2% of the entire spectrum of cancer diagnoses. […] Myxofibrosarcoma displays a higher tendency to recur post-treatment compared to other variants of soft tissue sarcomas. It’s estimated that up to 50% of individuals with myxofibrosarcoma experience a recurrence within five years of treatment. […] However, myxofibrosarcoma has a higher likelihood of recurrence after treatment compared to other soft tissue sarcoma types. Regular consultations with your healthcare provider heighten the possibility of timely detection and intervention if myxofibrosarcoma reoccurs.

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

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

  Myxofibrosarcoma is more common in the elderly, with an average age of 50 to 70 years. It is slightly more prevalent in males than in females. […] Myxofibrosarcoma is a rare, slowly growing aggressive tumor that occurs in the soft tissues of the body. It tends to recur, but on very rare occasions it spreads to other parts of the body where it originated from.

• #17 Myxofibrosarcoma – Wikipedia

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

  Myxofibrosarcoma (MFS), although a rare type of tumor, is one of the most common soft tissue sarcomas, i.e. cancerous tumors, that develop in the soft tissues of elderly individuals. […] In one large study, MFS was diagnosed in individuals 21 to 96 years old (median age, 66 years old). […] Most studies have diagnosed MFS slightly more often in men than women but one large study conducted in France found it to be 50% more common in men. […] Recurrences of the MFS at the site of surgery have developed in 16% to 57% of patients with a significant proportion (25%52%) recurring multiple times. […] In one study, metastatic disease was detected in 23% of patients and occurred at a median of 10 months (range, 277 months) after resection of the primary tumor. […] The recommended treatment for individuals presenting with localized MFS tumors is radical surgical resection.

• #18 Expanded molecular profiling of myxofibrosarcoma reveals potentially actionable targets | Modern Pathology

  https://www.nature.com/articles/modpathol201794

  The present study was designed to gain further insights into the molecular landscape of myxofibrosarcoma by targeted re-sequencing of known cancer driver hotspot mutations and the analysis of genome-wide somatic copy number alterations. […] The median age at presentation was 65 years (ranging from 29 to 94 years) with the majority of patients being female (18 female and 7 male patients, female to male ratio of 2.6 to 1). […] In eight patients the tumors were morphologically heterogeneous and demonstrated both G1 and G3 tumor areas. […] Three out of twenty-five patients developed a tumor recurrence, and seven patients developed metastases. […] The median follow-up time for the remaining patients ranged between 9 and 160 months (median 66 months). […] Herein we performed molecular profiling in myxofibrosarcomas to unveil clinically actionable targets and to gain further insights in mechanisms involved in tumor progression.

• #19 Myxofibrosarcoma of the mandible: a case report and review of the literature | BMC Oral Health | Full Text

  https://bmcoralhealth.biomedcentral.com/articles/10.1186/s12903-020-01094-7

  Myxofibrosarcoma (MFS) is a soft tissue sarcoma that commonly occurs in late adult life. […] The mean age in patients with MFS is between the fifth and seventh decades. […] Although some studies show a slight male predominance, the current evidence suggests no significant gender predilection. […] About 77% of MFS cases occur in the extremities with a predilection for the upper extremities. […] The incidence of MFS in the head and neck region is quite rare. […] MFS occurring intra-osseously in the head and neck region is exceedingly rare. […] To the best of our knowledge, only two cases of intraosseous MFS were reported in the maxilla and four cases reported in the mandible. […] MFS is associated with a relatively high rate of local recurrence or distant metastasis. […] Accurate preoperative diagnosis and proper treatment strategies are critical in managing patients with MFS.

• #20 A Case of Myxofibrosarcoma in the Maxillary Sinus

  https://www.kjorl.org/journal/view.php?number=8782

  Myxofibrosarcoma (MFS) is a rare subtype of soft tissue sarcoma, most commonly occurring in the lower extremities and rarely in the head and neck region. […] To our knowledge, there have been no reported cases of MFS originating from the maxillary sinus in Korea. […] Since the first report of MFS in the maxillary sinus in 1982, only 17 cases have been reported in nine studies outside Korea. […] In Korea, three cases of MFS originating from the false vocal cord, mandible, and cheek in head and neck lesions have been reported. […] MFS is observed in a wide age range from adolescence to old age, with the highest incidence detected in people in their 70s. […] It most commonly occurs in the lower extremities (77%), followed by the trunk (12%) and retroperitoneum/mediastinum (8%), but it rarely occurs in head and neck lesions.

• #21 Myxofibrosarcoma of the sinus piriformis: case report and literature review | World Journal of Surgical Oncology | Full Text

  https://wjso.biomedcentral.com/articles/10.1186/1477-7819-10-245

  Myxofibrosarcoma is a common sarcoma in the extremities of older people, but is rare in the head and neck region. […] Myxofibrosarcoma rarely occurs in the head and neck regions, and only a few corresponding cases in these areas have been reported, with 3% to 10% involvement, including the larynx, esophagus, sphenoid sinus, mandible, maxillary sinus, parotid, orbit, and infratemporal space. […] Only 18 cases have been described in the head and neck so far, our case being the second in the hypopharynx. […] Low-grade MFS is considered to have low malignancy, and rarely shows distant metastasis, implying a good short-term prognosis. The overall 5-year survival rate is 60% to 70%. […] However, the local recurrence rate of the low-grade type is as high (50% to 60%) as that of the high-grade type.

• #22 Epidemiologic Survey of Myxofibrosarcoma Using Data from the Bone and Soft Tissue Tumor Registry in Japan – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36437410/

  Myxofibrosarcoma is a common sarcoma among older patients, with locally infiltrative behavior and a predilection for local postoperative recurrence. […] This study investigated the clinical overview and prognosis of myxofibrosarcoma to determine the optimal treatment. […] This retrospective study analyzed the records of 349 patients with myxofibrosarcoma in the nationwide Bone and Soft Tissue Tumor Registry in Japan from 2006 to 2015. […] The overall survival rates were 93.1% at 2 years and 84.3% at 5 years. […] The study identified four significant prognostic factors for survival: tumor size, depth, compartment status, and location. […] The overall survival rate was 69.3% at 5 years for the patients with prognostic scores of 6 or higher. Conversely, the patients with prognostic scores of 2 or lower had a survival rate of 95.6% at 5 years.

• #23 Post-operative surveillance in soft tissue sarcoma: using tumorspecific recurrence patterns to direct approach – Zaidi – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/20903/html

  Myxofibrosarcoma: Although these tumors overall have a fair prognosis with 5-year OS rates between 75% and 77%, these tumors tend to be locally aggressive and infiltrative and thus can have local recurrences quite often. The 5-year local RFS rate for myxofibrosarcoma is 69-86% and the distant metastasis free survival rate is 68-83%. Distant metastases most commonly occur in the lung in patients with myxofibrosarcoma.

• #24 Overall Survival of Patients with Myxofibrosarcomas: An Epidemiological Study

  https://www.mdpi.com/2072-6694/14/5/1102

  Distant metastases rates reported in the literature range between 15% and 38%. […] We aimed to expand the limited amount of epidemiological data on MFS by describing the patient and tumour characteristics and clinical outcomes from a large Dutch cohort of MFS patients diagnosed between 2002 and 2019, using data from the Netherlands Cancer Registry (NCR). […] The overall median survival of patients with MFS was 155 months, with a five-year survival rate of 67.7%. […] Despite all advances in diagnostic opportunities, radiotherapy techniques and systemic treatments, we could not demonstrate an improved overall survival of MFS patients over the past 20 years. […] A way to improve clinical outcomes of MFS patients might be centralization of care. […] In conclusion, in this largest MFS cohort so far, no improvement in OS was found and high local recurrence rates are confirmed.

• #25 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. Robust epidemiological data on MFS in China are lacking. […] 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. […] 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. […] The median follow-up time for patients diagnosed with MFS was 52 months, with a corresponding five-year survival rate of 75%. This five-year OS rate aligns with the previously reported range of 61% to 77%.

• #26 Myxofibrosarcoma – Wikipedia

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

  Historically, about 10% of patients treated with radical surgery developed recurrences at the surgical site and a significant number of these patients developed metastatic disease. […] In one review of 109 individuals with MFS: overall survival for the entire group was 80% at 3 years and 76% at 5 years; local recurrence-free survival was 95% at 3 years and 88% at 5 years; median survival following local recurrence was 68 months; distant metastasis-free survival was 78% at 3 and 77% at 5 years; and 18 of 25 patients (72%) died of metastatic disease during a median follow-up time of 42 months for the overall review period of study. […] In three large studies, overall 5 year disease-specific survival times (i.e. the percentage of patients surviving 5 years excluding death due to any other causes than MFS from this count) were 51%, 73%, and 96%.

• #27 Overall Survival of Patients with Myxofibrosarcomas: An Epidemiological Study

  https://www.mdpi.com/2072-6694/14/5/1102

  Myxofibrosarcoma (MFS) is a rare soft tissue sarcoma type with high local recurrence and amputation rates. Robust epidemiological data on overall survival of patients with MFS and prognostic factors are lacking due to the rareness of the disease. […] Myxofibrosarcoma (MFS) is a rare mesenchymal soft tissue sarcoma type, with a high local recurrence (LR) rate. Robust epidemiological data on MFS are lacking. […] MFS accounts for 5% of all soft tissue sarcomas (STS) with STS showing an annual incidence of approximately 800 cases in the Netherlands. […] To date, known prognostic factors for OS in MFS include older age, tumour size, histological grade, and surgical margins. […] Compared to other STS, MFS is clinically known to have high local recurrence (LR) rates, ranging between 16% and 61% compared to around 10% in other types of STS.

• #28 Epidemiologic Survey of Myxofibrosarcoma Using Data from the Bone and Soft Tissue Tumor Registry in Japan – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36437410/

  Among myxofibrosarcomas, those larger than 5 cm, deep-seated, invaded into the external compartment, or in axial body parts were associated with a significantly worse prognosis. […] Previous staging classification systems are impractical for prognosis prediction. Therefore, new classifications are needed.

• #29 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

  The multivariate analysis showed that the surgical margin [hazard ratio (HR) =3.635, P0.001] and tumor size (HR =1.889, P=0.037) were risk factors for local recurrence. In addition, the surgical margin (HR =4.475, P=0.001) and presence of CD44 (HR =3.406, P=0.005) were risk factors for distant metastasis. […] The 3-year recurrence rate was 29.2%, and 33 cases had metastasis. […] The Kaplan-Meier survival analysis showed that the surgical margin (2=22.228, P0.001) and tumor size (2=6.697, P=0.010) were associated with recurrence. The surgical margin (2=12.353, P0.001) and CD44 expression (2=5.227, P=0.022) were associated with metastasis. […] The results of this study showed that the tumor size was an independent factor influencing the 3-year recurrence rate. The larger the tumor, the more extensive its invasion to surrounding tissues will be. Therefore, R0 resection is difficult to achieve. Therefore, the larger the tumor, the higher the recurrence rate.

• #30 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. […] MFS constituted the most common high-grade sarcoma (G2/G3, 79.4%) in our cohort and was generally located in a lower limb (73.6%). […] Tumour grade and metastasis were significant prognostic factors of survival (log-rank test p=0.041 and p=0.00007). […] 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). […] To the best of our knowledge, this is the most up-to-date report providing a comprehensive epidemiological and prognostic evaluation of MFS in a large population treated at a specialized centre.

• #31 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. […] 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. […] The factors influencing the recurrence, metastasis and survival of MFS have attracted attention. […] In conclusion, there are fewer factors for assessing disease progression in MFS.

• #32 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

  A positive surgical margin is an important cause of recurrence and metastasis in patients with MFS of the trunk and extremities. Extended resection is the first-choice therapy for patients with MFS. For those with a positive incision margin, appropriate adjuvant therapy may reduce the mortality rate caused by the recurrence and metastasis of tumors.

• #33 A Case of Myxofibrosarcoma in the Maxillary Sinus

  https://www.kjorl.org/journal/view.php?number=8782

  The clinical manifestation of MFS in the sinonasal tract resembles that of other soft tissue neoplasms, which includes symptoms such as epistaxis, nasal obstruction, rhinorrhea, and nonspecific nasal discomfort. […] MFS exhibits hypointense signals on T1-weighted images and markedly hyperintense signals on T2-weighted images. […] It is difficult to diagnose MFS in the sinonasal region before receiving a pathological confirmation because of the nonspecific nature of radiographic findings and the absence of distinctive symptoms. […] In contrast to various other soft tissue sarcomas, MFS is distinguished by its notable propensity for heightened local recurrence rates, which range between 16% and 61%. […] The reported rates of distant metastases in the literature vary between 15% and 38%.

• #34 Myxofibrosarcoma of the mandible: a case report and review of the literature | BMC Oral Health | Full Text

  https://bmcoralhealth.biomedcentral.com/articles/10.1186/s12903-020-01094-7

  The local recurrence rate for MFS does not correlate well with its histopathological grade. […] The local recurrence rate of MFS is about 16-57%. […] Distant metastasis is rarely seen in low-grade MFS. […] The rate of metastasis can be as high as 20 to 35% in intermediate and high-grade MFS. […] Patients with MFS need long-term follow-up to monitor for tumor recurrence. […] Long-term follow up for tumor surveillance is advisable in all cases.

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

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

  The LR rate within the studied population was 48%, and multiple recurrences have been recorded in a substantial number of patients. […] 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. […] The overall recurrence rate among patients who underwent postoperative radiotherapy was 30%, a figure that is notably lower than the aggregate recurrence rate. […] Myxofibrosarcomas are categorized into two distinct subtypes: superficial and deep. Superficial lesions are defined as those situated within the dermal or subcutaneous layer, while deep lesions are characterized as either intramuscular or subfascial. […] 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 contrast, an unplanned operation refers to a procedure performed at another hospital without detailed evaluation, followed by referral to our facility for further intervention. […] The study’s small sample size constitutes an important limitation. This constraint is largely attributable to the relative rarity of this particular tumor subtype.

• #36 Rapid Multiorgan Dissemination of Low-Grade Myxofibrosarcoma : A Case Report

  https://search.emarefa.net/en/detail/BIM-462497-rapid-multiorgan-dissemination-of-low-grade-myxofibrosarcoma

  Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly people. […] It is characterized by a high frequency of local recurrence due to an infiltrative growth property. […] In contrast, the overall risk of distant metastases is generally low. […] This makes the prognosis for the patients with myxofibrosarcoma definitely good. […] The patient died 8 months after the diagnosis of remote metastases due to rapid tumor progression.

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

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

  The detected metastases were located mainly in the lung (n=12) and the lymph nodes (n=2). […] The rate of development of metastases varies in the literature from 9.5-27.5%. […] In our cohort, the rate of metastasis was equivalent to the highest reported in the literature; therefore, we confirmed the findings of Tsuchie et al., who also reported a comparable rate of metastases. […] The survival rate was highly dependent on the tumour grade, the achievement of negative margins during surgery, and metastasis.

• #38 Myxofibrosarcoma of the sinus piriformis: case report and literature review | World Journal of Surgical Oncology | Full Text

  https://wjso.biomedcentral.com/articles/10.1186/1477-7819-10-245

  Therefore, these patients should be placed under careful and long-term follow-up. […] Histopathological examination is the gold standard for offering a definitive diagnosis, and the prognosis is accurate after complete resection and careful surveillance. Local recurrence may occur generally with progression of the tumor stage and risk of later metastasis.

• #39 Undifferentiated Pleomorphic Sarcoma/Myxofibrosarcoma/Other Fibrosarcomas | Oncohema Key

  https://oncohemakey.com/undifferentiated-pleomorphic-sarcomamyxofibrosarcomaother-fibrosarcomas/

  Undifferentiated pleomorphic sarcoma (UPS) encompasses malignant neoplasms of mesenchymal origin, which demonstrate cellular pleomorphism and which lack evidence of cellular differentiation. […] Overall, UPS constitutes 10% of adult STSs, with an incidence of around one new case per 100,000 per year and represents one of the most common STS subtypes diagnosed in older adults. […] Essential goals of follow-up surveillance include early identification of recurrences, identification of treatment-related complications, and patient reassurance. After the definitive treatment for localized disease is completed, the patients will require periodic visits and scans (CT or MRI), because patient recurrence risk never returns to zero. […] The follow-up interval may vary depending on the institution. There is limited data regarding the best strategy for surveillance. […] The National Comprehensive Cancer Network (NCCN) guidelines outline the importance of a prudent follow-up schedule, tailored to the recurrence risk. Higher grade and larger tumors may require a more intensive follow-up.

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

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

  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. […] Patients with MFS should be treated in specialized centers, with an interdisciplinary tumor board evaluation before surgery being essential to increase survival. 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. Resection and en bloc removal of the mass, followed by local tissue reconstruction (muscle flaps, skin grafts), are practiced for large tumors. It is recommended to aim for a resection margin of at least 1 cm, or, even better, a 2 cm width, to reduce the risk of local recurrence. MFS grade 2 and grade 3 tumors with positive surgical margins, and tumors originating in visceral organs (kidney, lungs), require surgery and adjuvant therapies.

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

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

  Studies have shown that MFS, irrespective of surgical margin resection status, location, size, depth, or grade, leads to local recurrences in 22–79% of cases. This appears to be related to the infiltrative growth pattern, i.e., tumoral cells spreading along fascial and vascular planes. A study including 75 cases of myxofibrosarcoma showed that the depth of the primary lesion is not related to the incidence of local recurrence. However, deeply located masses are usually larger, high-grade, and infiltrative, and associated with a worse prognosis. […] 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. The current standard of care involves performing a proper imaging diagnosis and histopathological analysis and ensuring tumor-free surgical margins after local excision. For extensive cases, a multidisciplinary team approach is essential to ensure a high curative rate, with proper local tissue reconstruction to provide the maximum functionality and quality of life and a low local recurrence rate.

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