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Introduction: Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare form of bone and soft tissue sarcoma. It occurs mainly in the deep soft tissue of the lower extremities, with few cases reported in the head and neck region. Tumors involving the oral and maxillofacial region (OMFR) and intraosseous examples are rare., Case Presentation: We present a 52-year-old male with a radiolucent lesion at the apex of the left mandibular second molar tooth with the clinical impression of a periapical granuloma. The patient has a history of SEF of the skull, which was treated by chemotherapy and radiation. Histopathologic examination revealed islands of highly cellular, basophilic bone and osteoid surrounded by loose fibrous stroma, which contains large lobules and islands of round to oval cells with distinct cell borders and faintly granular eosinophilic cytoplasm. Tumor cells were strongly positive for MUC4 and INI-1. Based on these findings, a diagnosis of high-grade malignancy consistent with SEF was made, and correlation with the primary lesion was recommended., Conclusion: SEF is a rare tumor seen in OMFR, and therefore, it should be included in the differential diagnosis of any high-grade malignancy. Immunohistochemical stain for MUC-4 evaluation is important for the diagnosis of SEF., Competing Interests: Declaration of interests None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Competing Interests: Declaration of competing interest The authors have no financial conflicts to disclose.

Background: Myxofibrosarcoma is a myxoid soft tissue sarcoma showing T2 high intensity on magnetic resonance imaging. However, myxofibrosarcoma is a heterogeneous sarcoma with both myxoid and cellular portions. Magnetic resonance imaging findings were obtained MRI findings for comparison with histological and Ki-67 immunohistochemical features, in different portions of one myxofibrosarcoma., Case Presentation: Magnetic resonance imaging observations were compared with gross pathological and microscopic findings of a myxofibrosarcoma from a 50-year-old Japanese female. The Ki-67 labeling indices of different portions of the tumor, that is, the myxoid, cellular, and histologically confirmed infiltrative margin portions (pathological tail sign), were compared. The T2 low intensity area was more cellular than the T2 high intensity area, while the cellular portion had a significantly higher Ki-67 index than the myxoid portion (p = 0.0313). The portions with the pathological tail sign had a significantly higher Ki-67 labeling index than those without this sign (p = 0.0313)., Conclusions: More cellular portions of a myxofibrosarcoma correspond to more areas of the tumor showing aggressive features. Furthermore, our data also support the hypothesis of high aggressiveness being associated with the pathological tail sign in myxofibrosarcoma. To our knowledge, this is the first case report to describe comparisons among the imaging findings, histological features, and Ki-67 immunohistochemistry results for different portions of one myxofibrosarcoma., (© 2024. The Author(s).)

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., (© 2024. The Author(s).)

A 70-year-old male presented with diplopia and painless proptosis of the left eye for 5 months. Examination showed 6 mm of axial proptosis and restriction of supraduction, abduction and adduction, and mild limitation of infraduction of the left eye. Magnetic resonance imaging demonstrated a large, moderately well-circumscribed intraconal mass in the left lateral orbit, and excisional biopsy was performed. Histopathologic features of mixed fibrous and myxoid areas in a whorl-like pattern and immunohistochemical staining for MUC4 confirmed the diagnosis of low-grade fibromyxoid sarcoma (LGFMS). Next-generation sequencing revealed genetic fusion of EWSR1-CREB3L1. LGFMS is an extremely rare neoplasm with only two prior documented cases of orbital involvement. Here, we report the third case of orbital LGFMS.

Purpose: Radiolabeled graphene oxide (GO) nanosheets has been one of the most extensively studied nanoplatform for in vivo radioisotope delivery. Herein, we describe the functionalization of the surface of GO nanosheets with Fe 3 O 4 magnetic nanoparticles, cysteine amino acid as an interface ligand, and cadmium telluride quantum dots., Materials and Methods: To enable In vivo PET imaging, the GO@Fe 3 O 4 -cys-CdTe QDs were labeled with 68 Ga to yield [ 68 Ga] Ga-Go@ Fe 3 O 4 -Cys-CdTe QDs. Furthermore, serum stability tests were performed and the biological behavior of the nanocomposite was evaluated in rats bearing fibrosarcoma tumor., Results: Liver, blood and tumor were the most accumulated sites at 1 h after the injection, and the radiolabeled nanocomposite as a PET/MRI imaging agent showed fast depletion from body and acceptable tumor uptake., Conclusion: Magnetic (Fe 3 O 4 ) and fluorescent components (CdTe QDs) along with a positron-emitting radionuclide will help to design a multimodal imaging system (PET/MRI/OI) which will offer the advantages of combined imaging techniques and further possible used in localized radionuclide therapy. Overall, [ 68 Ga] Ga-GO@Fe 3 O 4 -cys-CdTe QDs nanocomposite shows great promise as a radiolabeled imaging agent owing to high accumulation in tumor region., (© 2024. The Author(s) under exclusive licence to The Japanese Society of Nuclear Medicine.)

Rationale and Objectives: To investigate whether intravoxel incoherent motion (IVIM) and diffusion kurtosis imaging (DKI) parameters correlate with hypoxia biomarkers, namely hypoxia inducible factor-1ɑ (HIF-1ɑ), carbonic anhydrase IX (CAIX), and pimonidazole (PIMO), in fibrosarcoma (FS) murine models., Materials and Methods: A model of 30 FS nude mice was established. All mice underwent magnetic resonance imaging (MRI) scans after which the IVIM (standard apparent diffusion coefficient [standard ADC], pure diffusion coefficient [D], pseudo-diffusion coefficient [D*], and perfusion fraction [f]) and DKI parameters (mean diffusion [MD], mean kurtosis [MK]) were obtained. Based on an MRI-pathology controlled method, correlations between each MRI parameter and hypoxia biomarkers were assessed by Pearson or Spearman tests. An independent sample t-test or Wilcoxon's rank sum test, and receiver operating characteristic curves were used to identify whether MRI parameters could differentiate between high and low expressions of hypoxia biomarkers., Results: The IVIM/DKI parameters showed varying degrees of correlation with HIF-1α, CAIX, and PIMO expression. Among them, the D, f, and MK values could confirm HIF-1α expression, while D, f, and MK values could assess CAIX expression. Finally, standard D and MK values could evaluate PIMO expression levels., Conclusion: IVIM and DKI parameters can be used to reflect hypoxic biomarkers of FS and have the potential to detect tumor hypoxia., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Shaowu Wang reports financial support was provided by The National Natural Science Foundation of China., (Copyright © 2024 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Sclerosing epithelioid fibrosarcoma (SEF) occurring as a primary bone tumor is exceptionally uncommon. Even more rare are cases of SEF that show morphologic overlap with low-grade fibromyxoid sarcoma (LGFMS). Such hybrid lesions arising within the bone have only rarely been reported in the literature. Due to their variegated histomorphology and non-specific radiologic features, these tumors may pose diagnostic difficulties. Herein we describe three molecularly confirmed primary bone cases of sclerosing epithelioid fibrosarcoma that demonstrated prominent areas showing the features of LGFMS and with areas resembling so-called hyalinizing spindle cell tumor with giant rosettes (HSCTGR). Two patients were female and one was male aged 26, 47, and 16, respectively. The tumors occurred in the femoral head, clavicle, and temporal bone. Imaging studies demonstrated relatively well-circumscribed radiolucent bone lesions with enhancement on MRI. Cortical breakthrough and soft tissue extension were present in one case. Histologically the tumors all demonstrated hyalinized areas with SEF-like morphology as well as spindled and myxoid areas with LGFMS-like morphology. Two cases demonstrated focal areas with rosette-like architecture as seen in HSCTGR. The tumors were all positive for MUC4 by immunohistochemistry and cytogenetics, fluorescence in-situ hybridization, and next-generation sequencing studies identified EWSR1 gene rearrangements confirming the diagnosis in all three cases.Hybrid SEF is exceedingly rare as a primary bone tumor and can be difficult to distinguish from other low-grade spindled and epithelioid lesions of bone. MUC4 positivity and identification of underlying EWSR1 gene rearrangements help support this diagnosis and exclude other tumor types., (© 2023. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Background: Dermatofibrosarcoma Protuberans (DFSP) is a rare soft tissue sarcoma, accounting for approximately 1% of all tumors; however, DFSP of the breast is extremely rare. Moreover, DFSP generally has a low malignant potential and is characterized by a high rate of local recurrence along with a small but definite risk of metastasis. The risk of metastasis is higher in fibrosarcomatous transformation in DFSP than in ordinary DFSP., Case Report: We have, herein, reported a case of a 61-year-old male patient with fibrosarcomatous transformation in DFSP. Preoperative Dynamic Contrastenhanced Magnetic Resonance Imaging (DCE-MRI) of the breast revealed an oval-shaped mass with heterogeneous internal enhancement, a large vessel embedded within, and a washout curve pattern on kinetic curve analysis. The mass exhibited a hyperintense signal on Diffusion-weighted Imaging (DWI), with a low apparent diffusion coefficient value. Histologically, the bland spindle tumor cells were arranged in a storiform pattern. Areas with the highest histological grade demonstrated increased cellularity, cytological atypia, and mitotic activity. Immunohistochemically, Ki-67 and p53 were highly expressed., Conclusion: Recognizing the risk and accurately diagnosing fibrosarcomatous transformation in male breast DFSP are critical for improving prognosis and establishing appropriate treatment and follow-up plans. This emphasizes the significance of combining immunohistopathological features with DCE-MRI and DWI to assist clinicians in the early and accurate diagnosis of sarcomas arising from male breast DFSP.

We encountered a rare case of low-grade fibromyxoid sarcoma, which is generally known as Evans tumor, with massive calcification originating from the lung. The patient was a 22-year-old man with Duchenne muscular dystrophy who was referred for a detailed investigation of an intrathoracic tumor with massive calcification. Although our preoperative diagnosis was a solitary fibrous tumor originating from the mediastinum or diaphragm, intraoperative thoracoscopy revealed the tumor arising from the left lower lobe without adhesion to the other organs. Considering his medical history, we aimed to preserve lung function and chose wedge resection, which completely removed the tumor. Based on the pathological findings, the tumor was diagnosed as low-grade fibromyxoid sarcoma with massive calcification originating from the lung. Although extremely rare, this tumor should be considered as a differential diagnosis for a solitary lung mass with massive calcification in young adults.

We present the long-term follow-up of a case of periorbital congenital infantile fibrosarcoma (CIFS) treated with chemotherapy and surgery. The tumor was detected on a routine prenatal ultrasound at 30 weeks of gestation and diagnosed via an orbital biopsy day 9 postnatal age. The patient underwent chemotherapy and surgical debulking within the first 3 months of life and has maintained complete tumor remission for 7 years. The case highlights that early recognition and prompt treatment of periorbital CIFS can lead to complete long-term remission of this uncommon malignancy.

The need for complete resection of chest wall tumors creates a huge challenge in terms of reconstructing the complex dynamics of the thorax. We are reporting a case of a low-grade fibromyxoid sarcoma (LGFMS) diagnosed in a young male, where the complete resection of the mass, sternum and parcially the pericardium was performed. Subsequently, a composite porous high-density polyethylene StarPore® prosthesis of the sternum and costal arches was used and the latissimus dorsi muscle free flap with skin graft was implanted over the sternum.

Rational and Objective: To explore the correlations of histogram parameters from diffusion-weighted imaging (DWI) and intravoxel incoherent motion (IVIM) with the heterogeneous features in a nude mouse model of fibrosarcoma., Materials and Methods: A total of 44 fibrosarcoma xenograft models were established by inoculating HT-1080 cells on the right thigh of mice and subjected tumors to DWI and IVIM imaging with 3.0 T MRI. Whole-tumor histogram parameters were calculated on apparent diffusion coefficient (ADC), pure diffusion coefficient (D), pseudo-diffusion coefficient (D*), and perfusion fraction (f). Heterogeneous features, including necrosis rate, cell density, Ki-67 labeling index (LI), and microvascular density (MVD) were measured. Intraclass correlation coefficients (ICC), Pearson or Spearman correlation tests, and receiver operating characteristics (ROC) were performed., Results: The 90th percentile, skewness and kurtosis of ADC and D histograms showed correlations with necrosis rate, and the highest correlation coefficient was found for D 90th (r = 0.485). ADC and D histogram parameters showed correlations with cell density and Ki-67 LI; D 90th showed the highest correlation coefficient with cell density (r = -0.504); and D median showed the most significant correlation with Ki-67 LI (r = -0.525). D* skewness , D* kurtosis , D* 90th , f mean , and f median showed correlations with MVD. ADC 90th , ADC skewness , ADC kurtosis , D 90th , and D skewness showed significant differences between the low necrosis and high necrosis groups, and the combination model showed the best diagnostic ability (AUC = 0.882), with 97% sensitivity, and 72.7% specificity., Conclusion: Whole-tumor histogram parameters of DWI and IVIM were correlated with heterogeneous features in nude murine models of fibrosarcoma., (Copyright © 2022 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Myxofibrosarcoma is one of the most rare tumors of the heart. Surgical resection is the principal method of treatment; however, in some cases, radical removal of the tumor is not possible. Here, we present a case of heart transplant in a patient who experienced recurrent myxofibrosarcoma of the left atrium and examine the morphological features of this tumor. A 40-year-old female patient presented for treatment for heart failure of a high functional class. An examination revealed a tumor in the left atrial cavity, which was subsequently surgically removed. Morphological examination revealed an inflammatory myofibroblastic tumor. After 2 years, the patient's tumor recurred. The tumor was removed, and a morphological study again diagnosed myxofibrosarcoma of the heart. A year later, recurrence was again diagnosed. Instrumental examination determined that the tumor had incurred into the mitral valve and possibly spread to the myocardium. Tumor resection was not possible, and the need to perform a heart transplant was determined. The given case contributes to the practical conclusion that heart transplant contributes to an increase in the life expectancy for patients with inoperable cases of cardiac sarcoma.

Fibroblastic and myofibroblastic tumors are a variable group of neoplasms ranging from benign to malignant. These lesions may affect patients of any age group but are more frequently encountered in the pediatric population. Patient clinical presentation depends on the location, growth pattern, adjacent soft-tissue involvement, and pathologic behavior of these neoplasms. In the 2020 update to the World Health Organization (WHO) classification system, these tumors are classified on the basis of their distinct biologic behavior, histomorphologic characteristics, and molecular profiles into four tumor categories: (a) benign (eg, fibrous hamartoma of infancy, nodular fasciitis, proliferative fasciitis, fibroma of the tendon sheath, calcifying aponeurotic fibroma); (b) intermediate, locally aggressive (eg, desmoid fibromatosis); (c) intermediate, rarely metastasizing (eg, dermatofibrosarcoma protuberans, myxoinflammatory fibroblastic sarcoma, low-grade myofibroblastic sarcoma, infantile fibrosarcoma); and (d) malignant (eg, sclerosing epithelioid fibrosarcomas; low-grade fibromyxoid sarcoma; myxofibrosarcoma; fibrosarcoma, not otherwise specified). Detection of various components of solid tumors at imaging can help in prediction of the presence of corresponding histopathologic variations, thus influencing diagnosis, prognosis, and treatment planning. For example, lesions with a greater myxoid matrix or necrotic components tend to show higher signal intensity on T2-weighted MR images, whereas lesions with hypercellularity and dense internal collagen content display low signal intensity. In addition, understanding the radiologic-pathologic correlation of soft-tissue tumors can help to increase the accuracy of percutaneous biopsy and allow unnecessary interventions to be avoided. © RSNA, 2023 Quiz questions for this article are available in the supplemental material.

Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue tumour subtype, and those arising from the spine are even rarer. To the best of our knowledge, only 3 cases with the spine as the primary site of SEF have been previously reported. We report a 61-year-old female who presented with backache and bilateral leg numbness for 3 years, worsened over the last three months. Pathological fracture of the L1 vertebra was detected, and soft tissue density in the spinal canal and left vertebral body margin was also seen on contrast CT. She underwent tumour resection via a posterior approach, decompression, bone grafting, fusion, and internal fixation. Histology confirmed the diagnosis of SEF.

Background: Infantile fibrosarcoma is a rare pediatric soft tissue tumor and usually appears in children before one year of age. Distal extremities constitute the most frequently affected locations, and other tissues such as the trunk, head and neck, gut, sacrococcygeal region, and viscera are uncommon sites., Case Presentation: We describe a rare case of infantile fibrosarcoma arising from the perineum. First, a cystic mass was detected using prenatal ultrasonography, and then an echo was changed in serial ultrasound examinations. A solid cystic lesion was found at term; a hypoechoic lesion occurred in the back. The tumor became so large that massive bleeding occurred, which then underwent surgical resection. Pathological examination confirmed infantile fibrosarcoma., Conclusion: Our report demonstrates not all ultrasonographic findings in cases of infantile fibrosarcoma exhibit a solid mass during the initial examination - an early-stage lesion may reveal a cystic echo. Infantile fibrosarcoma has a good prognosis and surgery constitute the main treatment, with adjuvant chemotherapy being received if necessary., (© 2023. The Author(s).)

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., (© 2023. The Author(s).)

Background: Intraoperative molecular imaging (IMI)-guided resections have been shown to improve oncologic outcomes for patients undergoing surgery for solid malignancies. The technology utilizes fluorescent tracers targeting cancer cells without the use of any ionizing radiation. However, currently available targeted IMI tracers are effective only for tumors with a highly specific receptor expression profile, and there is an unmet need for IMI tracers to label a broader range of tumor types. Here, we describe the development and testing of a novel tracer (CR)-S0456) targeted to the sodium multivitamin transporter (SMVT)., Methods: Preclinical models of fibrosarcoma (HT-1080), lung (A549), breast (4T1), and renal cancers (HEK-293 T) in vitro and in vivo were used for assessment of (CR)-S0456 specific tumor labeling via sodium-mediated SMVT uptake in dipotassium phosphate or choline chloride-containing media buffer. Additionally, pharmacologic inhibition of multiple intracellular coenzyme-R obligate signaling pathways, including holocarboxylase synthetase (sulconazole nitrate), PI3K/AKT/mTOR (omipalisib), and calmodulin-dependent phosphatase (calmidazolium), were investigated to assess (CR)-S0456 uptake kinetics. Human fibrosarcoma-bearing xenografts in athymic nude mice were used for tumor and metabolic-specific labeling. Novel NIR needle confocal laser endomicroscopic (nCLE) intratumoral sampling was performed to demonstrate single-cell specific labeling by CR-S0456., Results: CR-S0456 localization in vitro correlated with highly proliferative cell lines (MTT) and doubling time (p < 0.05) with the highest microscopic fluorescence detected in aggressive human fibrosarcomas (HT-1080). Coenzyme-R-specific localization was demonstrated to be SMVT-specific after competitive inhibition of internal localization with excess administration of pantothenic acid. Inhibiting the activity of SMVT by affecting sodium ion hemostasis prevented the complete uptake of CR-S0456. In vivo validation demonstrated (CR)-S0456 localization to xenograft models with accurate identification of primary tumors as well as margin assessment down to 1 mm 3 tumor volume. Systemic treatment of xenograft-bearing mice with a dual PI3K/mTOR inhibitor suppressed intratumoral cell signaling and (CR)-S0456 uptake via a reduction in SMVT expression. Novel analysis of in vivo intratumoral cytologic fluorescence using near-infrared confocal laser endomicroscopy demonstrated the absence of coenzyme-R-mediated NIR fluorescence but not fibroblast activation protein (FAP)-conjugated fluorochrome, indicating specific intracellular inhibition of coenzyme-R obligate pathways., Conclusion: These findings suggest that a SMVT-targeted NIR contrast agent can be a suitable tracer for imaging a wide range of malignancies as well as evaluating metabolic response to systemic therapies, similar to PET imaging with immune checkpoint inhibitors., (© 2022. The Author(s), under exclusive licence to World Molecular Imaging Society.)

Ameloblastic fibrosarcoma (AFS) is the most common odontogenic sarcoma, but the incidence is relatively low, and its molecular biology is poorly understood. We experienced a young female patient with a rapidly growing soft tissue tumor of the left maxilla, which eventually occupied the left side of the oral cavity. Histologically, the tumor mainly consisted of a proliferation of atypical spindle to polygonal cells without any specific differentiation, but a small number of benign odontogenic epithelial foci mainly in the tumor periphery were also noted; thus, a diagnosis of AFS was made. We performed whole-exome sequencing (WES) on the tumor to investigate its molecular features and identify therapeutic options. We found that the tumor harbored EGFR exon 20 insertions and MDM2 amplification; the former may be a target for newly developed tyrosine kinase inhibitors in case of recurrence. To the best of our knowledge, this is the first case of AFS for which WES was performed and with EGFR mutation. Our case provides new genetic information on AFS and suggests that comprehensive genetic analysis can clarify the molecular biology in rare cancers, potentially leading to the proposal of therapeutic strategies., Competing Interests: Declaration of Competing Interest No conflicts of interest to disclose, (Copyright © 2021. Published by Elsevier B.V.)

Aim: To highlight the imaging findings in a case series of histologically confirmed infantile fibrosarcoma (IF) and identify any features specific to this entity., Materials and Methods: Retrospective identification was undertaken of patients with histologically confirmed IF from the electronic patient databases of two institutions between 1 January 2010 and 1 May 2021. Available pre-treatment imaging, histopathological reports, and clinical records were reviewed., Results: Eighteen patients with IF met the inclusion criteria. There were 10 male and eight female patients with a mean age at presentation of 3 weeks. All patients had the t (12; 15) chromosomal translocation. Eleven (61%) tumours were located in the extremities, three were in the craniofacial region, two were intrathoracic, one abdominal and one paraspinal. A single patient had extensive metastases. The tumours were generally isointense to skeletal muscle on T1-weighted sequences and hyperintense on T2 with heterogeneous enhancement and high cellularity seen as diffusion restriction. Fifteen of the 18 lesions were evaluated on ultrasound and appeared as heterogeneous, hypervascular solid or mixed solid/cystic masses, mimicking benign vascular lesions in two cases., Conclusion: The present two-centre, retrospective study of the largest case series described thus far demonstrates that IF is always highly cellular on magnetic resonance imaging but has no other specific imaging features. It should be considered in the differential diagnosis of any enlarging soft-tissue, solid mass arising in the limbs or neck at birth or in infancy., (Copyright © 2022 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

The ultrasound appearance of myxofibrosarcoma is highly variable corresponding to its variable and at times heterogeneous histopathologic appearance. Myxofibrosarcomas may mimic a benign process and the infiltrative tumor margins may be difficult to precisely delineate on ultrasound imaging. These tumor characteristics pose a diagnostic challenge on ultrasound evaluation. The radiologist should be aware of the variable morphologic presentation and infiltrative nature of myxofibrosarcoma and the limitations of ultrasound in the initial diagnosis, biopsy guidance, and post-surgical follow-up of this tumor., (© 2021. ISS.)

Myxofibrosarcoma (MFS) is a highly aggressive malignancy with complex karyotypes and a postoperative recurrence tendency, owing to its strong invasiveness. Although systemic chemotherapy is considered in patients with unresectable MFS, the efficacy of conventional chemotherapy is hitherto unclear. Recently, drug screening analysis using a large number of tumor cell lines has been attempted to discover novel therapeutic candidate drugs for common cancers. However, the number of MFS cell lines is extremely small because of its low incidence-this hinders the conduction of screening studies and slows down the development of therapeutic drugs. To overcome this problem, we established a novel MFS cell line, NCC-MFS5-C1, which was shown to harbor typical MFS genetic abnormalities and thus had useful properties for in vitro studies. We conducted the largest integrated screening analysis of 210 drugs using NCC-MFS5-C1 cells along with four MFS cell lines, which we previously reported. Bortezomib (a proteasome inhibitor) and romidepsin (a histone deacetylase inhibitor) showed stronger antitumor effects than the standard drug, doxorubicin. Therefore, the NCC-MFS5-C1 cell line can potentially contribute to elucidating MFS pathogenesis and developing a novel MFS treatment.

We report the first case of a patient with myxofibrosarcoma (MFS) who presented acutely with a rib fracture and developed a rapidly expanding loculated hemothorax after chest trauma. The patient was taken to the operating room for evacuation of hemothorax, and samples and biopsy specimens were taken for cytologic and pathologic examination. Final report with immunohistochemical staining showed a high-grade MFS. After the procedure, there was clinical and radiological improvement, and the patient was followed up as an outpatient. Myxofibrosarcoma is a very rare and aggressive connective tissue neoplasm with variable presentations. Surgical resection is the preferred treatment. Prompt diagnosis and adequate management of these tumors are important to reduce their high local recurrence and distant metastasis rates. Therefore, it is important to be aware of its common and uncommon presentations., (Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

Rationale and Objectives: Myxofibrosarcoma (MFS) is a common soft tissue sarcoma in the elderly patients with both clinical and magnetic resonance imaging (MRI) peculiar features: very high recurrence rate, relatively low risk of distant metastases. On MRI it shows an infiltrative pattern ("tail sign") and high myxoid matrix content with water-like appearance on fluid-sensitive sequences. Due to these unusual characteristics, we propose a specific MRI grading system to stratify the risk of local recurrence (LR) and offer other prognostic information., Materials and Methods: Two expert radiologists retrospectively and blindly reviewed preoperative MRI of 150 patients affected by MFS of the extremities treated at a single Institution. Myxoid matrix component and contrast enhancement of the tumor were evaluated and graded with a semiquantitative method. The presence of an infiltrative pattern, the depth of the tumor (deep and/or superficial) and tumor sizes were also recorded. MRI features were analyzed separately and correlated to LR risk, sarcoma specific survival and distant metastases rate. Then, according to the statistical significance of the correlation between MRI features and prognosis a 3-grade scoring system was proposed and evaluated to assess the risk of LR., Results: Mean age was 66.1 ± 14.4 years; mean follow-up was 16 ± 28.3 months. The MRI features most associated with higher risk of LR resulted to be: lesion sizes (both volume and maximum diameter with a cut-off of 20 cm - p = 0.01), the "tail sign" (p = 0.045), and high myxoid matrix content with MRI water-like appearance (p = 0.0493). Ninety-four patients (94 of 150- 62.7%) were grade 1, 33 (22.0%) grade 2, and 23 (15.3%) grade 3. Interobserver agreement was substantial with K= 0.779 (95%CI 0.685-0.874). Higher grades of MRI grading system proposed were significantly associated with an increased LR risk, hazard ratio = 2.031 (95%CI 1.366-3.019; p < 0.001)., Conclusion: This is the largest series evaluating MRI features as prognostic factors for MFS. The MRI grading system proposed is significantly able to stratify the risk of LR in MFS of the extremities. The system is applicable to all the standard MRI studies protocols, might help in surgical planning, and may offer prognostic information., (Copyright © 2020 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Background: Myxofibrosarcoma (MFS) is a rare soft tissue sarcoma with a high recurrence rate and a low risk of distant metastasis. It occurs mainly in the extremities of elderly men. Head and neck MFS is extremely rare. Surgery is the cornerstone of treatment. The role of radiotherapy (RT) and chemotherapy (CHT) on MFS is still debated., Case Presentation: A 67-year-old Caucasian man presented to our sarcoma referral center (SRC) with a history of MFS of the neck excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis. After a multidisciplinary discussion, preoperative RT was administered with a total dose of 50 Gy followed by wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred during the 24-month postoperative follow-up., Conclusions: The case described suggests the importance of planned combined treatments with both RT and surgery for high-grade soft tissue sarcoma. RT seems to be promising within this specific histotype. Close follow-up is advisable in all cases. Further studies are needed to confirm if the observed efficacy of combined treatments results in a prolonged time of disease-free survival and overall survival., (© 2021. The Author(s).)

Two adult neutered male dogs were presented for evaluation of firm, painless masses arising within muscle: an 8-year-old German wirehaired pointer dog with an accessory tricipital growth, and a 3-year-old German shepherd dog with a gracilis muscle growth. Magnetic resonance imaging (MRI) characteristics suggested malignant behavior, with a central fluid-like portion with a hyperenhancing lining, a nidus of disorganized tissue, and an extensive reactive zone, whereas histopathology was consistent with low-grade fibrosarcoma. This report describes histologically low-grade, yet biologically high-grade intramuscular fibrosarcoma, in which MRI provided detailed information on tumor behavior and assisted with biopsy and surgical planning., (Copyright and/or publishing rights held by the Canadian Veterinary Medical Association.)

Low grade fibromyxoid sarcoma (LGFMS) is an uncommon variant of fibrosarcoma with high risk of local recurrence, immense metastatic potential and frequently protracted period between tumour presentation and metastasis. This unusual malignancy rarely affects the region of the head and neck which makes cases of laryngeal LGFMS extremely infrequent. To date, LGFMS of the larynx has been scatteredly mentioned in the literature. Neither incidence nor causes and risk factors for laryngeal LGFMS have been clarified so far. To the authors' knowledge, this is the first case report that discusses the clinical course, imaging diagnosis, histopathological evaluation and surgical approach to radiation-induced laryngeal LGFMS.We present a case of a 70-year-old man who developed a LGFMS after previous radiotherapy (RT) for squamous cell carcinoma (SCC) of the larynx. The latency period between the time of radiation exposure and the diagnosis of LGFMS was twenty-seven months. After re-confirming the diagnosis with second biopsy and extensive imaging evaluation the patient was subjected to an open partial resection of the larynx. Owing to the rarity of the tumour, there is no established protocol with follow-up recommendations.This case highlights the importance of considering the RT history of the patient in order to monitor radiotherapy-related complications, including the occurrence of LGFMS., (This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Purpose: Preclinical radiation replicating clinical intensity modulated radiation therapy (IMRT) techniques can provide data translatable to clinical practice. For this work, treatment plans were created for oxygen-guided dose-painting in small animals using inverse-planned IMRT. Spatially varying beam intensities were achieved using 3-dimensional (3D)-printed compensators., Methods and Materials: Optimized beam fluence from arbitrary gantry angles was determined using a verified model of the XRAD225Cx treatment beam. Compensators were 3D-printed with varied thickness to provide desired attenuation using copper/polylactic-acid. Spatial resolution capabilities were investigated using printed test-patterns. Following American Association of Physicists in Medicine TG119, a 5-beam IMRT plan was created for a miniaturized (∼1/8th scale) C-shape target. Electron paramagnetic resonance imaging of murine tumor oxygenation guided simultaneous integrated boost (SIB) plans conformally treating tumor to a base dose (Rx 1 ) with boost (Rx 2 ) based on tumor oxygenation. The 3D-printed compensator intensity modulation accuracy and precision was evaluated by individually delivering each field to a phantom containing radiochromic film and subsequent per-field gamma analysis. The methodology was validated end-to-end with composite delivery (incorporating 3D-printed tungsten/polylactic-acid beam trimmers to reduce out-of-field leakage) of the oxygen-guided SIB plan to a phantom containing film and subsequent gamma analysis., Results: Resolution test-patterns demonstrate practical printer resolution of ∼0.7 mm, corresponding to 1.0 mm bixels at the isocenter. The miniaturized C-shape plan provides planning target volume coverage (V 95% = 95%) with organ sparing (organs at risk D max < 50%). The SIB plan to hypoxic tumor demonstrates the utility of this approach (hypoxic tumor V 95%,Rx2 = 91.6%, normoxic tumor V 95%,Rx1 = 95.7%, normal tissue V 100%,Rx1 = 7.1%). The more challenging SIB plan to boost the normoxic tumor rim achieved normoxic tumor V 95%,Rx2 = 90.9%, hypoxic tumor V 95%,Rx1 = 62.7%, and normal tissue V 100%,Rx2 = 5.3%. Average per-field gamma passing rates using 3%/1.0 mm, 3%/0.7 mm, and 3%/0.5 mm criteria were 98.8% ± 2.8%, 96.6% ± 4.1%, and 90.6% ± 5.9%, respectively. Composite delivery of the hypoxia boost plan and gamma analysis (3%/1 mm) gave passing results of 95.3% and 98.1% for the 2 measured orthogonal dose planes., Conclusions: This simple and cost-effective approach using 3D-printed compensators for small-animal IMRT provides a methodology enabling preclinical studies that can be readily translated into the clinic. The presented oxygen-guided dose-painting demonstrates that this methodology will facilitate studies driving much needed biologic personalization of radiation therapy for improvements in patient outcomes., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Case: We present a case of a 35-year-old-man with a giant sclerosing epithelioid fibrosarcoma (SEF) of the thigh. The patient presented with a history of a painful thigh mass. Plain radiographs revealed a soft-tissue mass with extensive calcifications, whereas on magnetic resonance imaging, a lobulated mass between the adductors and the posterior muscles of the thigh was noted. A wide en block resection was undertaken, and the histopathology confirmed the diagnosis of SEF. Postoperative radiation therapy was followed. The patient had no signs of recurrence at the 4-year follow-up., Conclusion: The clinicopathological, imaging characteristics, and treatment options of this rare soft-tissue tumor are discussed., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B471)., (Copyright © 2021 by The Journal of Bone and Joint Surgery, Incorporated.)

tetracationic (TMPyP) and tricationic porphyrin (TriMPyCOOHP) derivatives were synthesized, characterized and investigated for binding with DNA by Isothermal Titration Calorimetry as well as by UV-Vis spectroscopy in order to study the effect of structural variation on tumor targeting efficacy of cationically charged porphyrin derivatives. Fluorescence cell imaging studies performed in cancer cell lines corroborated the findings of aforementioned studies. Photocytotoxicity experiments in A549 cell lines revealed relatively higher light dependent cytotoxic effects exerted by TMPyP compared to TriMPyCOOHP. In-vivo experiments in tumor bearing animal model revealed relatively longer retention of 68 Ga-TMPyP in tumorous lesion compared to that of 68 Ga-TriMPyCOOHP. The study reveals that removal of one of the positive charges of the tetracationic porphyrin derivatives significantly reduces their DNA binding ability and cytotoxicity as well as brings changes in the pharmacokinetic pattern and tumor retention in small animal model., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of occurrence are the lower extremities, thorax, inguinal area, and upper limbs. Primary mediastinal sarcomas are even rarer. To the best of our knowledge, only seven cases of primary mediastinal LGFMS have been reported in the literature. Here, we report a case of primary mediastinal LGFMS., Case Presentation: A 26-year-old Pakistani man presented with fever and vomiting for the past 2 months. On a routine chest x-ray, a mediastinal mass was incidentally found. Computed tomography (CT) scan showed a large circumscribed lobulated soft tissue density mass lesion in an anterior mediastinum. Grossly, the resected mass measured 17.0 × 12.0 × 11.0 cm. The cut surface was gray white with a whorled-like appearance and foci of calcification and cystic changes. Histologically, a spindle cell lesion was seen with alternating myxoid and hyalinized areas. The shaped cells were arranged in bundles. Immunohistochemical staining showed positive reactivity patterns with MUC4 and focally for epithelial membrane antigen (EMA). The diagnosis was confirmed as LGFMS. The patient is free of symptoms and recurrence 22 months after the surgery., Conclusion: In conclusion, we report a rare case of primary mediastinal LGFMS in a young male patient that was discovered incidentally. Our patient is on regular follow-up to look for evidence of recurrence as these tumors are prone to recurrences.

Primary intracranial fibrosarcoma (PIF) was a rare tumor with a high relapse rate and dismal survival rate. This study aimed to delineate the clinical characteristics of primary intracranial fibrosarcoma (PIF) and the risk factors for outcomes. We reviewed 15 PIF patients, who underwent surgical treatment at our institution from January 2009 to December 2018. Meanwhile, 36 cases from the prior literature between November 1962 and December 2019 were also retrieved and pooled to identify the risk factors. In our cohort, while cystic component (46.7%), perilesional edema (83.3%), and vascular flow void (66.7%) were commonly observed, no patient was accurately diagnosed. The 2-year relapse-free survival (RFS) and overall survival (OS) were 12.2% and 30.2%, respectively. Based on the pooled data, tumor size (p = 0.006), Ki-67 index (p = 0.004), and radiotherapy dose (p = 0.029) were prognostic factors for RFS in univariate analysis. In the univariate analysis, tumor size (p = 0.002), NGTR (p = 0.049), and high Ki-67 index (p = 0.019) were significant predictors for OS; and further multivariate analysis (n = 18) showed that large tumor size (≥ 5 cm; HR 14.613, p = 0.022) and high Ki-67 index (≥ 30%; HR 5.879, p = 0.020) were the independent risk factors for OS. Due to the rarity and nonspecific clinicoradiological features, the correct diagnosis of PIF before surgery was challenging. The outcomes of PIF were poor, and GTR plus radiotherapy (at least 60 Gy) might benefit to the outcomes and were recommended. Future study with a large cohort was needed to verify our findings.

Myxofibrosarcoma is one of the most common soft tissue sarcomas in the elderly. It is characterized by an extremely high rate of local recurrence, higher than other soft tissue tumors, and a relatively low risk of distant metastases.Magnetic resonance imaging (MRI) is the imaging modality of choice for the assessment of myxofibrosarcoma, which plays a key role in the preoperative setting of these patients. MRI features associated with the high risk of local recurrence are: high myxoid matrix content (water-like appearance of the lesions), high grade of contrast enhancement and presence of an infiltrative pattern ("tail sign"). On the other hand, MRI features associated with worse sarcoma specific survival are: large size of the lesion, deep location, high grade of contrast enhancement. Recognizing the above-mentioned imaging features of myxofibrosarcoma may be helpful in stratifying the risk for local recurrence and disease-specific survival. Moreover, the surgical planning should be adjusted according to the MRI features., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Soft tissue sarcomas are a large group of different tumor types both in humans and in animals. Among them, fibrosarcoma is the most frequent malignant mesenchymal tumoral form in cats, representing up to 28% of all cat skin tumors, while human fibrosarcoma, fortunately, only represents 5% of all sarcomas and 0.025% of the world-wide burden of tumors. This low incidence in humans leads to consideration of this group of tumoral diseases as rare, so therapeutic options are few due to the difficulty of starting clinical trials. In this context, the identification of research models for fibrosarcomas could be of great interest to deepen knowledge in this field and recognize new or possible biological pathways involved in tumor progression and metastasis. Angiogenesis is considered a fundamental scattering cause of tumor aggressiveness and progression in all forms of cancer, but only a few research parameters were developed and reported to express them quantitatively and qualitatively. The role in angiogenesis of microenvironmental stromal cells, such as fibroblasts, lymphocytes, mast cells, and macrophages, was largely demonstrated since this topic was first approached, while quantification of new vessels and their blood capacity in tumoral area is a relatively recent approach that could be well developed thanks to expertise in immunohistochemistry and image analysis. In this paper, a crossing study evaluating microvascular density (MVD), endothelial area (EA), and Ki-67 proliferative index was reported for a series of formalin-fixed and paraffin-embedded tissue samples from 99 cat patients, affected by cat post-injection fibrosarcoma, by using a till ×400 magnification light microscopy. We aim to demonstrate that cat pets may be considered a useful animal model for better studying the correspondent human diseases and we report, for the first time to our knowledge, experimental data in terms of correlation among MVD, EA, and Ki-67 strictly involved in aggressiveness and tumoral progression.

Benign and malign tumors can affect the temporomandibular joint (TMJ) as any other articulation. Nevertheless, TMJ tumors are rare and mostly benign. Their clinical expression is varied including symptomatology similar to TMJ dysfunctional disorders, otologic or neurologic pathologies. In some cases, they remain totally asymptomatic. Hence, diagnosis is difficult since the symptomatology can be misleading with TMJ dysfunctional disorders or otologic disorders wrongly diagnosed. There is thus frequently a long delay between symptoms onset and diagnosis. The great variety of TMJ lesions explains the wide range of possible treatment modalities, mostly based on surgery. We provide here a review of the lesions originating from the TMJ. Tumoral or cystic mandibular lesion affecting the TMJ through local extension will not be discussed. Osteoma, osteoid osteoma, osteoblastoma, chondroma, osteochondroma, chondroblastoma, tenosynovial giant cell tumors, giant cell lesions, non-ossifying fibroma, hemangioma, lipoma or Langerhans cell histiocytosis are all possible diagnosis among the benign tumors found in the TMJ. Pseudotumors include synovial chondromatosis and aneurysmal bone cyst. Finally, malign tumors of the TMJ include mainly sarcomas (osteosarcoma, chondrosarcoma, synovial sarcoma, Ewing sarcoma, and fibrosarcoma), but also multiple myeloma and secondary metastases. We will review the clinical, radiological and histological aspects of each of these lesions. The treatment and the recurrence risk will also be discussed., (Copyright © 2020 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Background/aim: Myxofibrosarcoma (MFS) is characterized by an infiltrative growth pattern. This study aimed to determine the correlation between overall survival (OS) and morphological features of MFS as well as examine the reproducibility of these findings on preoperative magnetic resonance imaging (MRI)., Patients and Methods: Fifty-eight MFS patients underwent preoperative MR imaging with the following features analysed: i) tumour size, ii) localization, iii) margins, iv) morphology, v) signal characteristics, vi) contrast enhancement, vii) presence and extent of perilesional oedema, and viii) presence of the tail sign., Results: Only circumscribed perilesional oedema was associated with a significantly better survival compared to diffuse oedema (p=0.010), which was found in the majority of cases. The tail sign was found in less than 50% of the cases. Cohen's kappa coefficients confirmed a relatively high interrater variability., Conclusion: Perilesional diffuse oedema on MR imaging of MFS is significantly correlated with a poor overall survival. The interrater variability in interpretation of MR examinations varies from slight to substantial agreement. Preoperative MR imaging with detailed planning of the resection seem to be a logical approach to achieve negative resection margins and recurrence-free survival., (Copyright© 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)