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Journal of Medical Case Reports Dec 2014Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, peaking in the seventh decade, and it is mainly encountered in the lower... (Review)
Review
INTRODUCTION
Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, peaking in the seventh decade, and it is mainly encountered in the lower extremities. Myxofibrosarcoma of the head and neck are extremely rare. To the best of our knowledge, only 19 cases have been described in the head and neck so far. This is a literature review and retrospective chart review of our experience in head and neck myxofibrosarcoma treatment in our department.
CASE PRESENTATION
In this case report we describe a 35-year-old Caucasian man who presented the first case of myxofibrosarcoma arising from the pterygopalatine fossa. The peculiar anatomical location and the extent in the midcheek region make this case a hard "challenge" for the surgeon, in order to guarantee wide surgical margins of resection. A total right maxillectomy was accomplished by means of the Weber-Ferguson approach, preserving the orbital floor. The excised portion was reconstructed using the free rectus abdominis myocutaneous flap. Postoperative radiotherapy was given to the area adjacent to the lesion, with a total dose of 60 Gy. No relapse occurred in the 27-month postoperative follow-up.
CONCLUSIONS
The case described suggests the importance of combined surgical and adjuvant radiotherapy to avoid local and distant recurrences of the tumor. In our opinion, combined surgical and adjuvant radiotherapy followed by close clinical observation to search for a metastatic disease is advisable in all cases. Further studies are needed to confirm the efficacy of combined radio-chemotherapy for head and neck myxofibrosarcoma in terms of long-term disease-free survival.
Topics: Adult; Combined Modality Therapy; Fibrosarcoma; Head and Neck Neoplasms; Humans; Male; Myxosarcoma; Radiotherapy, Adjuvant; Surgical Flaps; Tomography, X-Ray Computed
PubMed: 25547541
DOI: 10.1186/1752-1947-8-468 -
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 -
Frontiers in Bioscience (Elite Edition) Jun 2018We investigated the impact of haplotype of major histocompatibility complex (MHC)-B on the outcome of infection of Synthetic Dam Line (SDL) broiler strain with Rous...
We investigated the impact of haplotype of major histocompatibility complex (MHC)-B on the outcome of infection of Synthetic Dam Line (SDL) broiler strain with Rous Sarcoma Virus (RSV). Genomic analysis of MHC-B haplotypes, revealed a total of 12 known standard haplotypes that constituted to twenty-five different genotypes and one new haplotype of 217 bp size, designated B. The inoculation of RSV-A in SDL chicks resulted in the development of tumors of progressive or regressive phenotypes with varying tumor profile index (TPI). Haplotypes B, B and Bhad low TPI scores (1 or 2) with less mortality and were resistant to RSV-A tumor. The haplotypes B, B, B, Band Bhad significantly higher TPI scores (5 or 6), indicating a susceptibility to RSV-A. The genotype, B /B, had a mean TPI score of 3.67 ± 1.33, which was closer to the resistant haplotype. Sequence analysis of the new haplotype (B) revealed 99.5% similarity with B2 haplotype. Metastases was observed in 44% of chicks and comprised of mixed fibrosarcoma and myxosarcoma.
Topics: Animals; Chickens; Haplotypes; Major Histocompatibility Complex; Rous sarcoma virus; Sarcoma, Avian
PubMed: 29772523
DOI: 10.2741/E837 -
Journal of Veterinary Diagnostic... Jun 2024Synovial myxoma, a rare joint tumor in dogs, has traditionally been considered benign, acknowledging that local invasion into regional tissues including bone may be...
Synovial myxoma, a rare joint tumor in dogs, has traditionally been considered benign, acknowledging that local invasion into regional tissues including bone may be present. Given the diagnostic challenges in distinguishing synovial myxoma from other joint lesions through clinical features and diagnostic imaging, definitive diagnosis relies on characteristic gross and histologic features. Within the inner surface of the joint capsule, synovial myxomas form nodules of stellate-to-spindle cells within abundant myxomatous matrix. We present here 2 cases of synovial myxoma with metastasis to regional lymph nodes and compare these 2 cases to 3 cases without evidence of lymph node metastasis. Aside from lymphovascular invasion in one case with metastasis, there were no overt histologic features of the primary tumor to suggest aggressive biologic behavior. The finding of lymph node metastasis warrants reconsideration of the term "synovial myxoma" for this neoplasm. We suggest the term "synovial myxosarcoma," considering that histologic features of the primary tumor do not predict biologic behavior. Our case series highlights the importance of lymph node sampling in suspected synovial myxosarcoma cases as well as thorough histologic examination, emphasizing careful evaluation for lymphovascular invasion.
PubMed: 38828841
DOI: 10.1177/10406387241257254 -
British Journal of Cancer Aug 2021Soft tissue sarcomas (STS) are generally considered non-immunogenic, although specific subtypes respond to immunotherapy. Antitumour response within the tumour...
BACKGROUND
Soft tissue sarcomas (STS) are generally considered non-immunogenic, although specific subtypes respond to immunotherapy. Antitumour response within the tumour microenvironment relies on a balance between inhibitory and activating signals for tumour-infiltrating lymphocytes (TILs). This study analysed TILs and immune checkpoint molecules in STS, and assessed their prognostic impact regarding local recurrence (LR), distant metastasis (DM), and overall survival (OS).
METHODS
One-hundred and ninety-two surgically treated STS patients (median age: 63.5 years; 103 males [53.6%]) were retrospectively included. Tissue microarrays were constructed, immunohistochemistry for PD-1, PD-L1, FOXP3, CD3, CD4, and CD8 performed, and staining assessed with multispectral imaging. TIL phenotype abundance and immune checkpoint markers were correlated with clinical and outcome parameters (LR, DM, and OS).
RESULTS
Significant differences between histology and all immune checkpoint markers except for FOXP3+ and CD3-PD-L1+ cell subpopulations were found. Higher levels of PD-L1, PD-1, and any TIL phenotype were found in myxofibrosarcoma as compared to leiomyosarcoma (all p < 0.05). The presence of regulatory T cells (Tregs) was associated with increased LR risk (p = 0.006), irrespective of margins. Other TILs or immune checkpoint markers had no significant impact on outcome parameters.
CONCLUSIONS
TIL and immune checkpoint marker levels are most abundant in myxofibrosarcoma. High Treg levels are independently associated with increased LR risk, irrespective of margins.
Topics: Aged; B7-H1 Antigen; Biomarkers, Tumor; CD3 Complex; CD4 Antigens; CD8 Antigens; Female; Fibrosarcoma; Forkhead Transcription Factors; Humans; Leiomyosarcoma; Male; Middle Aged; Myxosarcoma; Programmed Cell Death 1 Receptor; Retrospective Studies; T-Lymphocytes, Regulatory; Tissue Array Analysis; Tumor Microenvironment; Up-Regulation
PubMed: 34127811
DOI: 10.1038/s41416-021-01456-0 -
Journal of Veterinary Diagnostic... Jan 2022A 5-y-old, male degu () was presented with a subcutaneous mass in the ventral aspect of the cervical area. The mass was removed surgically. Histologically, the mass was...
A 5-y-old, male degu () was presented with a subcutaneous mass in the ventral aspect of the cervical area. The mass was removed surgically. Histologically, the mass was a densely cellular, expansile neoplasm, with compression of thymic tissue to the periphery. The neoplasm consisted of solid sheets of polygonal cells, mixed with fewer small lymphocytes. Rare Hassall bodies were scattered throughout the mass. Polygonal cells were positive for anti-keratin/cytokeratin AE1/AE3 antibody, and small lymphocytes were positive for anti-CD3 antibody. The histopathologic and immunohistochemical findings were consistent with a thymoma. In addition, an autopsy revealed myxosarcoma of the right thoracic wall with metastasis to the lung. To our knowledge, thymoma originating from the cervical component of the thymus has not been documented previously in a rodent species.
Topics: Animals; Male; Octodon; Rodent Diseases; Rodentia; Thymoma; Thymus Neoplasms
PubMed: 34515595
DOI: 10.1177/10406387211045643 -
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 -
Journal of Comparative Pathology Nov 2023This report documents the pathological features of primary cardiac myxoid tumour (MT) in 11 dogs. Macroscopically, all the tumours were located in the tricuspid valve... (Review)
Review
This report documents the pathological features of primary cardiac myxoid tumour (MT) in 11 dogs. Macroscopically, all the tumours were located in the tricuspid valve (TV), its septal leaflet being predominantly affected. Therefore, it appears that the TV is the most common site of occurrence for cardiac MT in dogs. Two gross anatomical types of canine valvular MT were evident. Seven of the 11 tumours were round or oval with a smooth or gently lobulated and glistening surface, while the other four were gelatinous, multilobulated and polypoid, with an irregular surface. Microscopically, in nine cases the tumours had an abundant myxoid matrix within which elongated spindle-shaped cells with no remarkable cytological atypia were sparsely embedded, suggesting a benign character (ie, myxoma). In the other two cases the tumours consisted of variably dense, haphazardly arranged, interlacing streams of anaplastic spindle-shaped or polygonal cells containing many mitotic figures, indicative of a malignant form of myxoma (ie, myxosarcoma). Isolated or clustered collections of myxoma cells (eg, cords, rings, syncytia) characteristic of human atrial myxoma were only rarely evident or lacking in all 11 cases, indicating that rarity or absence of such structural features may be specific to valvular MTs. Immunohistochemical findings were indicative of smooth muscle differentiation of the neoplastic cells. Tumour embolization to the intrapulmonary arteries and/or tumour implantation on the endocardium of the right heart chambers was evident only in the four cases of irregular-surfaced MT.
Topics: Humans; Dogs; Animals; Heart Neoplasms; Myxoma; Endocardium; Myxosarcoma; Dog Diseases
PubMed: 37944473
DOI: 10.1016/j.jcpa.2023.10.004 -
World Journal of Surgical Oncology Nov 2012Myxofibrosarcoma is a common sarcoma in the extremities of older people, but is rare in the head and neck region. Here, we report the case of a 42-year-old male patient... (Review)
Review
Myxofibrosarcoma is a common sarcoma in the extremities of older people, but is rare in the head and neck region. Here, we report the case of a 42-year-old male patient in whom myxofibrosarcoma generated from the sinus piriformis. Histopathologically, the tumor was characterized by spindle cellular proliferation with moderate cellular density in fibromyxoid stroma. Immunohistochemically, the tumor cells showed positive reactivity for vimentin, Ki-67, smooth muscle actin, and CD34, but negative staining for S-100. Based on these results, the tumor was diagnosed as a low-grade myxofibrosarcoma. Resection of the tumor was performed via a transcervical approach. The patient's postoperative clinical course was uneventful and no local recurrence or distant metastasis has been found so far. The pathology, clinical characteristics, and treatment of myxofibrosarcoma are also reviewed.
Topics: Adult; Fibrosarcoma; Humans; Male; Myxosarcoma; Pharyngeal Neoplasms; Prognosis; Review Literature as Topic
PubMed: 23152982
DOI: 10.1186/1477-7819-10-245 -
Journal of Surgical Case Reports Jan 2015The vast majority of adult primary cardiac tumours (75%) are benign. The differentiation between malignant and benign lesions based on imaging is often difficult....
The vast majority of adult primary cardiac tumours (75%) are benign. The differentiation between malignant and benign lesions based on imaging is often difficult. Furthermore, it is challenging to distinguish between a cardiac myxoma and a myxosarcoma histologically. We report the case of a 48-year-old female who underwent resection of myxoma. Fourteen months postoperatively, she developed dyspnoea and evidence of local recurrence was observed. An open biopsy was performed and compared with the initially resected specimen. A primary cardiac myxosarcoma was diagnosed. Extended resection of the tumour including a part of the left atrium and the left lung was performed. Follow-up at 4 years shows no radiological evidence of any further recurrence and the patient is satisfied with a good quality of life. Despite the infrequent nature and particularly in view of the poor prognosis of cardiac myxosarcoma with a median overall survival of ∼12-17 months, we were able to demonstrate in our case that, with an extensive medical and surgical therapy and an interdisciplinary approach, a long-term disease-free survival can be achieved.
PubMed: 25583907
DOI: 10.1093/jscr/rju146