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Background First described in 1977, myxofibrosarcoma is one of the most common sarcoma subtypes of the elderly. Until some years ago, myxofibrosarcoma was diagnosed as “myxoid malignant fibrous histiocytoma.” The aim of this retrospective case series analysis was to investigate prognostic factors and the clinical outcome of a cohort of patients with myxofibrosarcoma treated at a single institution. Methods We reviewed 158 patients with localized myxofibrosarcoma who underwent surgery at the Istituto Nazionale Tumori of Milan, Italy, over 15 years. Local recurrence, distant metastases, and survival were analyzed. Results One hundred twenty patients had primary tumors, while 38 patients had locally recurrent tumors. Five-year overall survival was 77%. Tumor size, grade, and margins were statistically significant predictors of survival. Five-year local recurrence and distant metastases rate were 18% and 15%, respectively. Surgical margins were the only statistically significant prognosticator of local relapses. Patients treated with radiotherapy had the same prognosis as nontreated patients, but likely they had worse local presentations. The histological grade correlated with distant recurrences but not with local relapses. The value of adjuvant chemotherapy could not be determined. Conclusions Patients with myxofibrosarcoma have a better disease-specific survival than other sarcoma subtypes, but also a higher local relapse rate. This is likely related to the peculiar local growth pattern of these tumors. Adequate surgery should be pursued, while the role of adjuvant therapies need to be investigated.

Myxofibrosarcoma presents an infiltrating growth pattern that results in a high tendency for local recurrence. Clear margin resection is challenging because of microscopic infiltration. The purpose of the present study was to analyze the overall and disease-free survival rates of patients with myxofibrosarcoma and the prognostic factors that determine both survival and disease recurrence. Among the 111 patients included in our study, the 5-year overall survival rate was 65.5%. An age of more than 65 years (hazard ratio [HR] 1.9 [95% confidence interval (CI) 1.4–5.6]; p < 0.001), a tumor size of more than 5 cm (HR 2.8 [95% CI 0.9–8.1]; p = 0.049) and the G3 tumor grade (HR 14.1 [95% CI 2.1–105.0]; p < 0.001) negatively affected overall survival. The 5-year recurrence-free survival rate was 49.4%. R1/R2-type resection (HR 2.4 [95% CI 1.0–5.6]; p = 0.048) had a detrimental effect on tumor recurrence. Clear margins had a positive impact on recurrence-free survival, but did not significantly affect overall patient survival, suggesting that other factors may play a more significant role in determining patient outcomes. A surgical margin of 2 mm was not sufficient to significantly influence the incidence of recurrence. Consequently, a wider surgical margin may be necessary to reduce the risk of myxofibrosarcoma recurrence.

Background Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone. Case presentation A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up. Conclusion Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.

Myxofibrosarcoma of the spermatic cord is a rare form of para-testicular tumor that usually presents with painless scrotal or inguinal swelling. Ultrasonography revealed a solid mass in the scrotum, suggesting a para-testicular tumor, and exploration via a high inguinal incision revealed a large para-testicular mass. Finally, a pathologic examination revealed a low-grade sarcoma favoring myxoid fibrosarcoma. Here, we present the first reported case of myxofibrosarcoma of the spermatic cord in Ethiopia in a 54-year-old male patient and reviewed the available literature on the topic.

Background A sclerosing epithelioid fibrosarcoma (SEF) is an uncommon tumor of the deep soft tissue. An SEF has been described as a low-grade tumor with high local recurrence and metastatic rates. Generally, in bone and soft tissue tumors, a resection of the biopsy route is recommended; however, there is limited evidence with respect to the dissemination of the tumor tissue during a needle biopsy. Case presentation A mass in the right pelvic cavity, with no symptoms, was observed in a 45-year-old woman during a gynecological examination. Computed tomography (CT) revealed a multilocular mass with calcification in the pelvic cavity. The magnetic resonance imaging (MRI) showed an iso-signal intensity on T1 weighted images and hypo- and iso-signal intensity on T2 weighted images. The CT-guided core needle biopsy was performed using a dorsal approach, and the biopsy diagnosis was a low-grade spindle cell tumor. The tumor was excised using an anterior approach. The tumor tissue comprised spindle cells and epithelioid cells with irregular nuclei, and the immunohistological analysis was positive for vimentin and epithelial membrane antigen, which was consistent with a diagnosis of sclerosing epithelioid fibrosarcoma. Five years after the surgery, the MRI showed a tumor recurrence in the subcutaneous tissue of the right buttock, which was consistent with the needle biopsy tract. The patient underwent a tumor excision, and the resected tumor was similar to the primary tumor. Conclusions The recurrent tumor was excised with a surgical margin, and the tumor specimen had the histological features of a sclerosing epithelioid fibrosarcoma. It was difficult to investigate the association of the core needle biopsy with the tumor recurrence because the approach of the biopsy tract is usually same as that used in a tumor excision. However, the present case indicated the tumor may recur in the biopsy tract of a soft tissue sarcoma. Surgeons should be aware of the possibility of disseminating tumor tissues in a needle biopsy.

The aim of this study was to evaluate the influence of resection margins on rates of local control in patients with myxofibrosarcoma (MFS). We performed a retrospective cohort study of 135 patients with myxofibrosarcoma, treated at the Department of Orthopaedics of the Medical University of Vienna in Austria from December 1999 to October 2023. Resection margins were analysed with Enneking’s established classification scheme as well as Union internationale contre le cancer (UICC) classification. Local recurrence (LR) was reported for 14 patients (10.37%). There was no statistically significance for the influence of width of resection margins (neither UICC classification system, nor Enneking’s) on the LR rate, except for R1-dir status in the UICC-classification ( p  = 0.028), meaning there was microscopic tumour contamination of margins or resection alongside and macroscopic residual tumour in R2a status ( p  = 0.037). There was no statistical difference of resection with wide or marginal resection margins. We can carefully suppose that width of resection margins do not seem to be crucial for local recurrence in MFS, although there is a significant risk for local recurrence in R1 resections. Further investigations on the risk factors for local recurrence are needed.

Myxofibrosarcoma(MFS) is the most common soft tissue sarcoma(STS) in elderly patients. Surgical resection remains the main treatment modality but tumor borders can be difficult to delineate with conventional clinical methods. Incomplete resections are a common problem and local recurrence remains a clinical issue. A technique that has shown great potential in improving surgical treatment of solid tumors is tumor targeted imaging and image-guided surgery with near-infrared fluorescence. To facilitate this technique, it is essential to identify a biomarker that is highly and homogenously expressed on tumor cells, while being absent on healthy non-malignant tissue. The purpose of this study was to identify suitable molecular targets for tumor-targeted imaging of myxofibrosarcoma. Ten potential molecular targets for tumor targeted imaging were investigated with immunohistochemical analysis in myxofibrosarcoma tissue (n = 34). Results were quantified according to the immunoreactive score(IRS). Moderate expression rates were found for uPAR, PDGFRa and EMA/MUC1. High expression rates of VEGF and TEM1 were seen. Strong expression was most common for TEM1 (88.2%). These results confirms that TEM1 is a suitable target for tumor-targeted imaging of myxofibrosarcoma. Keywords Image-guided surgery; Immunohistochemistry; Molecular imaging; Myxofibrosarcoma; Soft tissue sarcoma; Tumor endothelial marker 1(TEM1), Vascular endothelial growth factor (VEGF).

BackgroundThe microinvasive nature of suprafascial myxofibrosarcoma reduces the accuracy of intraoperative margin assessment, and tumor bed resections after soft-tissue reconstruction are unreliable. In 2017, we began temporizing the excised tumor bed with a wound VAC, delaying soft-tissue coverage until final negative margins were achieved. We compare the oncologic/surgical outcomes of suprafascial myxofibrosarcomas managed with VAC temporization (VT) with single-stage excision/reconstruction (SS).MethodsWe retrospectively studied suprafascial myxofibrosarcomas managed from January 1, 2000 to January 1, 2019 for patients who received neoadjuvant or adjuvant radiation and had at least 2 years of oncologic follow-up at a tertiary referral cancer center. Our primary outcome was local recurrence. Comparisons were performed by using Fisher’s exact test or Student’s t test. A p value < 0.05 was considered significant.ResultsFifty-three patients (18 VAC temporized, 35 single stage) were included. While VT patients were older (74.9 ± 10.2 vs. 63.9 ± 13.6, p = 0.003), treatment groups did not significantly differ with respect to comorbidity, tumor volume, stage and grade. VT patients had significantly fewer local recurrences (5.6% vs. 28.6% after SS, p = 0.048) and R1 resections that required an unplanned readmission for tumor bed reexcision (0% vs. 37.1% after SS, p = 0.002). VT required more total surgeries (2.8 ± 0.9 vs. 1.8 ± 0.9 for SS, p = 0.0002). Postoperative infectious and wound complications were equivalent.ConclusionsOur VAC temporization strategy had a significantly lower LR than SS treatment. While high quality multi-institutional validation is required, VT may represent a paradigm shift in the management of myxofibrosarcoma.

Background Fibrosarcoma is a rare, invasive soft tissue sarcoma that is more common in males aged 30–60 years. It accounts for 1% of all malignancies in the human body. Treatment is radical surgical removal, radiotherapy, and chemotherapy. Case presentation A 58-year-old Khorasani Persian male patient with a complaint of expansion, pain, and paresthesia in the left maxillary quadrant was diagnosed with infiltrative high-grade sarcoma. Tumoral cells were strongly diffuse positive for vimentin and negative for S100 and desmin, indicating high-grade adult fibrosarcoma. Conclusion This report presents a rare case of a fibrosarcoma causing paresthesia, pain, and expansion in the maxilla with maxillary sinus invasion, which requires early diagnosis and immediate referral.

Background Myxofibrosarcoma (MFS) is a rare, aggressive soft tissue sarcoma primarily affecting older adults. Despite curative-intent surgery and multimodal therapy, outcomes remain variable. Acute kidney injury (AKI) and sarcopenia are known complications in oncologic care, but their incidence and impact in MFS patients remain unclear. Objective To assess the incidence and consequences of AKI in adult MFS patients undergoing curative-intent surgery, and its association with survival, CT-based sarcopenia progression, and postoperative morbidity. Methods In this retrospective single-center study, 49 adults with high-grade, N0M0 MFS who underwent curative-intent resection with or without multimodal therapy were included. AKI was defined as a ≥ 0.3 mg/dL increase in serum creatinine. Sarcopenia and adipose tissue loss were assessed via serial CT morphometry scans at the L3 lumbar level. Associations between AKI and clinical outcomes (survival, function, length of hospital stay, surgical site infection) were evaluated using regression and survival analyses. Results AKI occurred in 38.8% of patients and independently predicted decreased overall survival (HR 6.73; p  = 0.0005). AKI was associated with accelerated loss of muscle tissue and visceral fat (all p  < 0.001), functional decline (ECOG, p  = 0.0078), longer hospitalization ( p  < 0.001), and increased wound infections (52.6% vs. 10.0%; p  < 0.0001). Conclusions This is the first study to identify AKI as an independent predictor of mortality and sarcopenia in surgically treated MFS patients. CT morphometry may aid in early risk stratification, while renal monitoring offers a potential target for improving outcomes through nephroprotective strategies.