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BMJ Case Reports May 2021Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon soft-tissue malignancy. LGFMS preferentially affects trunks and extremities of young adults; however, occasional...
Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon soft-tissue malignancy. LGFMS preferentially affects trunks and extremities of young adults; however, occasional cases have been reported in different sites of head and neck region including oral cavity, larynx and oropharynx. LGFMS usually exhibit areas of collagenised and myxoid stroma with appearance of spindle cells in whorling pattern. It is a challenge to diagnose it accurately as most of the time it is misdiagnosed as benign neoplastic entity of spindle cells. There have been only few isolated cases of LGFMS reported in head and neck region and LGFMS originating from the parapharyngeal space has never been reported before. We recently experienced a case of low grade fibomyxoid sarcoma in parapharyngeal space of neck. LGFMS have the propensity to locally recur and to metastasise. Due to its rarity in head and neck region, there are no well-established treatment and follow-up guidelines.
Topics: Fibrosarcoma; Humans; Myxosarcoma; Neoplasm Recurrence, Local; Parapharyngeal Space; Sarcoma; Soft Tissue Neoplasms; Young Adult
PubMed: 34031060
DOI: 10.1136/bcr-2020-237083 -
Caspian Journal of Internal Medicine Mar 2021It is a rare cardiac malignant primary tumor that seems to derive from the same cellular line as myxomas, but the prognosis is very different. It is a rare cardiac...
BACKGROUND
It is a rare cardiac malignant primary tumor that seems to derive from the same cellular line as myxomas, but the prognosis is very different. It is a rare cardiac malignant primary tumor that seems to derive from the same cellular line as myxomas, but the prognosis is very different. It is a rare cardiac malignant primary tumor that seems to derive from the same cellular line as myxomas, but the prognosis is very different. Cardiac myxosarcoma is a rare neoplasm that appears to rise from the same cellular source like myxoma. It is difficult to differentiate a myxoma tumor from a myxosarcoma tumor because of its appearance and pathology examination. Myxosercoma tumor requires surgery and chemoradiotherapy, but myxoma is treated only by surgery.
CASE PRESENTATION
We describe a case of a 58-year-old patient with a left atrium myxosarcoma, presenting with congestive heart failure. Transthoracic echocardiogram (TTE) showed a large polypoid and mobile mass in the left atrium, the patient underwent cardiac surgery and the tumor was successfully extracted, and histopathological result revealed typical features of myxoma. 15 days after surgery, he underwent explorative laparatomy because of progressive GI bleeding. Laparatomy revealed extensive metastatic masses in abdomen and the pathology diagnoses was myxosaroma. Unfortunately, in spite of supportive care, the patient expired on postoperative day one.
CONCLUSION
It is difficult to differentiate a myxoma tumor from a myxosarcoma tumor because of its appearance and pathology examination. Maybe magnetic resonance imaging can help us to achieve more data suggesting malignancy.
PubMed: 34012543
DOI: 10.22088/cjim.12.2.228 -
Oxidative Medicine and Cellular... 2021Myxosarcomas are rare malignant tumors of soft connective tissues, classified into various subtypes, including myxoid liposarcoma, myxoid chondrosarcoma, and myxoid...
Myxosarcomas are rare malignant tumors of soft connective tissues, classified into various subtypes, including myxoid liposarcoma, myxoid chondrosarcoma, and myxoid leiomyosarcoma. In this study, we proposed to study the demographic, tumor characteristics, and overall survival rate and compared the treatment modalities between these cancers. Patient data collected based on locoregional metastasis presentation of the abovementioned tumors with a cutoff study of survival duration up to 10 years were obtained from the SEER database during 1975-2016. Our results indicated that elderly patients and females were more in locoregional myxoid leiomyosarcoma than myxoid liposarcoma and myxoid chondrosarcoma with locoregional metastasis. The white race represented the most patients who suffered from these cancers than other races. The heart is the primary site for the abovementioned cancers, in addition to the female genitals to the myxoid leiomyosarcoma. Myxoid liposarcoma and myxoid chondrosarcoma patients with locoregional metastasis were suffering from grade II, while locoregional myxoid leiomyosarcoma patients with blank grading were due to missed data. Surgery was the most common treatment modality in this study compared with radiotherapy and chemotherapy. Kaplan-Meier analysis showed a significant difference in survival time between the three subtypes by using histology, and myxoid leiomyosarcoma showed prolonged survival than others. Elderly, female, white, unknown grade, surgery, no radiation, and no chemotherapy variables were independent factors associated with overall survival among these cancers. Multivariate analysis also showed significant differences in overall survival between the three tumors by histology, and myxoid leiomyosarcoma was with a better prognosis than others. Multivariate analysis of locoregional myxoid leiomyosarcoma showed the statistical significance of black race, grade, and radiotherapy, indicating them as independent prognostic factors of locoregional myxoid leiomyosarcoma. We conclude that surgery was the primary treatment modality against these cancers than radiotherapy and chemotherapy. And the locoregional myxoid leiomyosarcomas showed a better prognosis and higher survival rate than locoregional myxoid liposarcoma and locoregional myxoid chondrosarcoma.
Topics: Female; Humans; Leiomyosarcoma; Middle Aged; Myxosarcoma; Survival Rate; United States
PubMed: 34007412
DOI: 10.1155/2021/9999529 -
Medicine Mar 2021Myxofibrosarcoma (MFS) is a locally aggressive tumor and has the potential to be fatal because of distant metastasis. Immunotherapy targeting either programmed cell...
INTRODUCTION
Myxofibrosarcoma (MFS) is a locally aggressive tumor and has the potential to be fatal because of distant metastasis. Immunotherapy targeting either programmed cell death protein 1 (PD-1) or programmed death ligand 1 (PD-L1) has recently shown a curative effect on multiple cancers including melanoma, non-small cell lung cancer, and renal cell carcinoma. Although the immunotherapy has been applied in sarcoma, there is little information about the efficiency to treat metastatic MFS.
PATIENT CONCERNS
A 42-year-old male presented to the clinic with a mass in the left thigh. Mass resection and ligament replacement surgery were performed.
DIAGNOSES
The patient was diagnosed as high-grade MFS (federation nationale des centres de lutte contre le cancer, Grade 3) with pulmonary metastasis.
INTERVENTIONS
In the past few years, he was treated with surgery, chemoradiotherapy, and Anlotinib (an angiogenesis inhibitor), but the metastatic lesion continued to progress. About 40% to 50% of tumor cells in his pulmonary tissues were showed positive PD-L1 expression and his tumor mutational burden was 215Muts. Thus, he received Camrelizumab (PD-1 inhibitor).
OUTCOMES
Six months after the initiating immunotherapy of Camrelizumab, the size of pulmonary lesions showed marked shrinkage, indicating a partial response. After a follow-up of 18 months, the patient remained in good condition without progressive disease.
CONCLUSION
This case described here demonstrated that immunotherapy of PD-1 inhibitor is a promising treatment option for refractory MFS with PD-L1 positive or tumor mutational burden -high, which could contribute to effective tumor response.
Topics: Adult; Antibodies, Monoclonal, Humanized; Antineoplastic Agents; Dissection; Humans; Immunotherapy; Lung Neoplasms; Male; Myxosarcoma; Neoplasm Grading; Neoplasm Staging; Programmed Cell Death 1 Receptor; Progression-Free Survival; Soft Tissue Neoplasms; Thigh; Treatment Outcome
PubMed: 33761725
DOI: 10.1097/MD.0000000000025262 -
Journal of Medical Case Reports Feb 2021Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of... (Review)
Review
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.
Topics: Adult; Fibrosarcoma; Humans; Male; Mediastinum; Myxosarcoma; Neoplasm Recurrence, Local; Soft Tissue Neoplasms
PubMed: 33526082
DOI: 10.1186/s13256-020-02605-4 -
The Journal of Veterinary Medical... Apr 2021A 13-year-old intact Pomeranian bitch presented with a 2-month history of abdominal distension and anorexia. Ultrasonography and computed tomography revealed a large...
A 13-year-old intact Pomeranian bitch presented with a 2-month history of abdominal distension and anorexia. Ultrasonography and computed tomography revealed a large tumor in the abdominal cavity without metastases. The tumor was surgically resected and histopathologically characterized by spindle-shaped to atypical-shaped neoplastic cells with basophilic stroma in the omental adipose tissue. Immunohistochemistry revealed that the neoplastic cells were positive for vimentin but negative for cytokeratin, S-100 protein, and α-SMA. The bitch was diagnosed as a myxosarcoma arising from the greater omentum. Postoperatively, metronomic chemotherapy with cyclophosphamide and piroxicam was initiated. The tumor recurred on postoperative day 49. Although the bitch died 102 days after the initial examination, her general condition was maintained until death.
Topics: Adipose Tissue; Animals; Dog Diseases; Dogs; Female; Immunohistochemistry; Myxosarcoma; Neoplasm Recurrence, Local; Omentum
PubMed: 33504735
DOI: 10.1292/jvms.20-0509 -
Oncology Research and Treatment 2020The aim of this retrospective study is to verify whether preoperative systemic inflammatory markers (serum C-reactive protein [CRP] and neutrophil-lymphocyte ratio...
BACKGROUND
The aim of this retrospective study is to verify whether preoperative systemic inflammatory markers (serum C-reactive protein [CRP] and neutrophil-lymphocyte ratio [NLR]) can help in predicting the disease-specific survival (DSS) and local recurrence (LR) rate in adult patients affected by localized myxofibrosarcoma (MFS) of the extremities.
METHODS
We reviewed 126 adult patients with primary, localized MFS of the limbs. We analyzed DSS and LR.
RESULTS
Median age at the time of surgery was 68 years (range 19-92). Median CRP was 0.4 mg/dL and median NLR was 2.8. A worse DSS was found in patients who had preoperative CRP >0.5 mg/dL (p = 0.002) and in those with NLR >3.5 (p < 0.001). In multivariate analysis, tumor size and grade as well as preoperative CRP values and NLR were confirmed to be prognostic factors in terms of DSS. An increased risk of LR was found in multivariate analysis in patients with a tail sign and with high gadolinium enhancement at preoperative MRI.
CONCLUSIONS
Patients with high preoperative CRP and NLR levels, as well as large and high-grade tumors, might be considered as candidates for additional, more aggressive treatment approaches or more stringent follow-up schedules.
Topics: Adult; Aged; Aged, 80 and over; Biomarkers; C-Reactive Protein; Extremities; Female; Fibrosarcoma; Humans; Inflammation; Lymphocytes; Male; Middle Aged; Multivariate Analysis; Myxosarcoma; Neoplasm Recurrence, Local; Neutrophils; Preoperative Period; Prognosis; Retrospective Studies; Soft Tissue Neoplasms; Survival Rate; Young Adult
PubMed: 32810863
DOI: 10.1159/000509429 -
Oncology Research and Treatment 2020The aim of this retrospective analysis is to understand the natural history of myxofibrosarcoma (MFS), in particular whether the prognosis can be influenced by...
BACKGROUND
The aim of this retrospective analysis is to understand the natural history of myxofibrosarcoma (MFS), in particular whether the prognosis can be influenced by histologic grade.
METHODS
We reviewed 229 adult patients with primary MFS of the limbs. We analyzed disease-specific survival (overall survival [OS]) and local recurrence (LR).
RESULTS
Median age was 70 years (range, 19-92). Sixteen (7.0%) were grade 1, 38 (16.6%) grade 2, and 175 (76.4%) grade 3. A worse OS was found in grade 3 MFS (73.1%) than in grade 2 and 1 MFS (91.9 and 100%, respectively) at 5 years (p = 0.031). Locally recurred MFS had a worse OS (p = 0.018). A better LR-free rate (100% at 5 years) was observed in grade 1 MFS; however, a similar rate was observed between grade 2 and 3 tumors (77.1 and 80.0% at 5 years, respectively, p = 0.412).
CONCLUSIONS
Grade 3 MFS has the worst prognosis. Grade 1 MFS have the lowest risk of LR. These data could help identify a high-risk patient group, thus selecting a more careful follow-up for higher-risk patients. Since MFS mostly affects the elderly population, it might be useful to reserve adjuvant treatments (radiotherapy and chemotherapy) to higher-risk patients.
Topics: Adult; Aged; Extremities; Female; Fibrosarcoma; Follow-Up Studies; Humans; Male; Middle Aged; Myxosarcoma; Neoplasm Grading; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Soft Tissue Neoplasms; Survival Rate; Young Adult
PubMed: 32268331
DOI: 10.1159/000506844 -
BMC Medical Imaging Aug 2019Myxoid tumors pose diagnostic challenges for radiologists and pathologists. All myxoid tumors can be differentiated from each other using fluorescent in-situ...
BACKGROUND
Myxoid tumors pose diagnostic challenges for radiologists and pathologists. All myxoid tumors can be differentiated from each other using fluorescent in-situ hybridization (FISH) or immunohistochemical markers, except for myxomas and myxofibrosarcomas. Myxomas and myxofibrosarcomas are rare tumors. Myxomas are benign and histologically bland, whereas myxofibrosarcomas are malignant and histologically heterogenous. Because of the histological heterogeneity, low grade myxofibrosarcomas may be mistaken for myxomas on core needle biopsies. We evaluated the performance of T1-weighted signal intensity (T1SI), tumor volume, and radiomic features extracted from magnetic resonance imaging (MRI) to differentiate myxomas from myxofibrosarcomas.
METHODS
The MRIs of 56 patients (29 with myxomas, 27 with myxofibrosarcomas) were analyzed. We extracted 89 radiomic features. Random forests based classifiers using the T1SI, volume features, and radiomic features were used to differentiate myxomas from myxofibrosarcomas. The classifiers were validated using a leave-one-out cross-validation. The performances of the classifiers were then compared.
RESULTS
Myxomas had lower normalized T1SI than myxofibrosaromas (p = 0.006) and the AUC using the T1SI was 0.713. However, the classification model using radiomic features had an AUC of 0.885 (accuracy = 0.839, sensitivity = 0.852, specificity = 0.828), and outperformed the classification models using T1SI (AUC = 0.713) and tumor volume (AUC = 0.838). The classification model using radiomic features was significantly better than the classifier using T1SI values (p = 0.039).
CONCLUSIONS
Myxofibrosarcomas are on average higher in T1-weighted signal intensity than myxomas. Myxofibrosarcomas are larger and have shape differences compared to myxomas. Radiomic features performed best for differentiating myxomas from myxofibrosarcomas compared to T1-weighted signal intensity and tumor volume features.
Topics: Aged; Case-Control Studies; Diagnosis, Differential; Female; Fibrosarcoma; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Myxoma; Myxosarcoma; Radiographic Image Interpretation, Computer-Assisted; Retrospective Studies
PubMed: 31416421
DOI: 10.1186/s12880-019-0366-9 -
Journal of Toxicologic Pathology Jul 2019An extraskeletal osteosarcoma was detected in the auricle of a 110-week-old female Wistar Hannover rat. Grossly, the tumor, measuring 15 mm in size, was observed in the...
An extraskeletal osteosarcoma was detected in the auricle of a 110-week-old female Wistar Hannover rat. Grossly, the tumor, measuring 15 mm in size, was observed in the subcutis as a solid and hard mass. Histologically, the majority of the mass comprised mature, compact bone. It was surrounded by neoplastic cells showing a variety of histologies, such as sarcoma, not otherwise specified, and myxosarcoma away from the bone-forming region. However, these different histological regions were considered to be components of a single bone tumor, based on the common expression of osterix and a similar mixture of constituent cells in each region. The tumor was diagnosed as an extraskeletal osteosarcoma because of the presence of infiltrative growth and abnormal mitosis and its development in the auricle without attachment to the skeleton. The present case is a rare histological type of an extraskeletal osteosarcoma with independent and different histological elements in rats.
PubMed: 31404367
DOI: 10.1293/tox.2018-0046