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Medicine Dec 2017Fibromyxosarcoma is common in head and neck, vessel, omentum, and reproductive system, with low-grade malignant behavior. However, primary atrial fibromyxosarcoma with...
RATIONALE
Fibromyxosarcoma is common in head and neck, vessel, omentum, and reproductive system, with low-grade malignant behavior. However, primary atrial fibromyxosarcoma with highly malignant behavior is extremely rare.
PATIENT CONCERNS
A 34-year-old female presented with oppression in the chest, short breath, and onset of headache as initial symptoms. The preoperative echocardiogram showed a medium-size echogenic mass close to the posterior leaflet of the mitral valve in the left atrium.
DIAGNOSIS
Primary atrial fibromyxosarcoma with multiple-system metastases.
INTERVENTIONS
The patient underwent surgery, and the tumor was removed completely. The diagnosis of left atrium fibromyxosarcoma was confirmed through postoperative histopathological examination. Positron emission tomography/computed tomography scan was performed, which revealed multiple metastases to left adnexa, bilateral adrenal glands, left iliacus, right lateral ventricle, and skeletal system.
OUTCOMES
The patient died of cerebral hernia caused by hemorrhage from the metastatic brain tumor, 30 days after the surgery, without receiving chemotherapy or radiotherapy.
LESSONS
Cardiac fibromyxosarcoma is a rare primary malignant cardiac neoplasm, probably with systemic metastases. The possibility of malignancy should be considered as differential diagnosis for cardiac mass.
Topics: Adult; Echocardiography; Fatal Outcome; Female; Heart Atria; Heart Neoplasms; Humans; Myxosarcoma; Neoplasm Metastasis; Positron Emission Tomography Computed Tomography
PubMed: 29245258
DOI: 10.1097/MD.0000000000008930 -
American Journal of Veterinary Research Jul 2001To evaluate results of centrosome hyperamplification in naturally developing tumors of dogs.
OBJECTIVE
To evaluate results of centrosome hyperamplification in naturally developing tumors of dogs.
SAMPLE POPULATION
Tumor specimens from 9 dogs with tumors (rhabdomyosarcoma, osteosarcoma, chondrosarcoma, myxosarcoma, and mammary gland tumor) and 2 canine osteosarcoma cell lines.
PROCEDURE
3 antibodies for centrosome proteins (ie, anti-gamma-tubulin, anti-BRCA1, and anti-pericentrin) were used for immunohistochemical analysis. Double immunostaining for centrosomes was used to confirm the specificity of these antibodies for centrosomes. Mutational analysis of the canine p53 gene was carried out by polymerase chain reaction-single-strand conformation polymorphism analysis, and expression of canine MDM2 protein was evaluated by use of immunohistochemical analysis, using anti-MDM2 antibody.
RESULTS
Immunohistochemical analysis of dog osteosarcoma cell lines with apparent aneuploidy revealed frequent hyperamplification of centrosomes in the osteosarcoma cell lines. Similar hyperamplified centrosomes were detected in the tumor tissues from all of the 9 tumors. The frequency of cells with hyperamplified centrosomes (3 to 20/cell) in each tumor tissue ranged from 9.50 to 48.1%, whereas centrosome hyperamplification was not observed in normal lymph nodes from these dogs. In 8 of the 9 tumors, mutation of p53 gene or overexpression of MDM2, or both, was detected.
CONCLUSIONS AND CLINICAL RELEVANCE
Various types of naturally developing tumors in dogs often have hyperamplification of centrosomes associated with chromosome instability. Hyperamplification of centrosomes is a novel tumor marker for use in cytologic and histologic examinations of clinical specimens obtained from dogs.
Topics: Animals; Centrosome; Chromosome Aberrations; DNA, Neoplasm; Dog Diseases; Dogs; Female; Gene Expression Regulation, Neoplastic; Genes, p53; Immunohistochemistry; Male; Mammary Neoplasms, Animal; Nuclear Proteins; Polymorphism, Single-Stranded Conformational; Proto-Oncogene Proteins; Proto-Oncogene Proteins c-mdm2; RNA, Neoplasm; Reverse Transcriptase Polymerase Chain Reaction; Sarcoma; Sequence Analysis, DNA; Tumor Cells, Cultured
PubMed: 11453492
DOI: 10.2460/ajvr.2001.62.1134 -
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 -
BMC Cancer Jun 2010Myxofibrosarcoma is common in the extremities of elderly people and is characterized by a high frequency of local recurrence.
BACKGROUND
Myxofibrosarcoma is common in the extremities of elderly people and is characterized by a high frequency of local recurrence.
CASE PRESENTATION
We report a 37 year old female who presented with a 4-month history of facial pain and a 3-month history of painful progressive swelling in the preauricular area. She underwent a total parotidectomy. The tumor was histopathologically and immunohistochemically diagnosed as a low-grade myxofibrosarcoma. The patient was free of disease 9 months after surgery with uneventful post-operative clinical course.
CONCLUSIONS
Parotid area swelling should always alert doctors. To our knowledge, this is the first case of parotid myxofibrosarcoma. It should be added to the differential diagnosis of diseases of the parotid. We have to recognize this disease and seek adequate treatment for it.
Topics: Adult; Female; Fibrosarcoma; Humans; Myxosarcoma; Parotid Neoplasms
PubMed: 20513245
DOI: 10.1186/1471-2407-10-246 -
The Bone & Joint Journal Dec 2016Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities. Locally infiltrative...
AIMS
Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities. Locally infiltrative growth means that the risk of local recurrence is high. We reviewed our experience to make recommendations about resection strategies and the role of the multidisciplinary team in the management of these tumours.
PATIENTS AND METHODS
Patients with a primary or recurrent MFS who were treated surgically in our unit between 1997 and 2012 were included in the study. Clinical records and imaging were reviewed. A total of 50 patients with a median age of 68.4 years (interquartile range 61.6 to 81.8) were included. There were 35 men; 49 underwent surgery in our unit.
RESULTS
The lower limb was the most common site (32/50, 64%). The mean size of the tumours was 8.95 cm (1.5 to 27.0); 26 (52%) were French Fédération Nationale des Centres de Lutte Contre le Cancer grade III. A total of 21 (43%) had positive margins after the initial excision; 11 underwent further excision. Histology showed microscopic spread of up to 29 mm beyond macroscopic tumour. Local recurrence occurred in seven patients (14%) at a mean of 21 months (3 to 33) and 15 (30%) developed metastases at a mean of 17 months (3 to 30) post-operatively.
CONCLUSION
High rates of positive margins and the need for further excision makes this tumour particularly suited to management by multidisciplinary surgical teams. Microscopic tumour can be present up to 29 mm from the macroscopic tumour in fascially-based tumours. Cite this article: Bone Joint J 2016;98-B:1682-8.
Topics: Aged; Aged, 80 and over; Disease Management; Female; Fibrosarcoma; Humans; Lower Extremity; Male; Medical Audit; Middle Aged; Myxosarcoma; Neoplasm Grading; Neoplasm Recurrence, Local; Patient Care Team; Prognosis; Retrospective Studies; Soft Tissue Neoplasms; Survival Analysis; Upper Extremity
PubMed: 27909132
DOI: 10.1302/0301-620X.98B12.37568 -
The Indian Medical Gazette Mar 1891
PubMed: 29000624
DOI: No ID Found -
Indian Journal of Ophthalmology Mar 1971
Topics: Child, Preschool; Exophthalmos; Humans; Male; Myxosarcoma; Ophthalmologic Surgical Procedures; Orbital Neoplasms; Treatment Outcome
PubMed: 15744961
DOI: No ID Found -
Journal of the American Veterinary... Apr 2013
Topics: Animals; Dog Diseases; Dogs; Female; Heart Neoplasms; Heart Valve Diseases; Myxosarcoma
PubMed: 23547667
DOI: 10.2460/javma.242.8.1067 -
The Canadian Veterinary Journal = La... Apr 2018A 9-year-old golden retriever dog was diagnosed with a left retrobulbar mass. Fine-needle aspirations and incisional biopsies resulted in discordant diagnoses:...
A 9-year-old golden retriever dog was diagnosed with a left retrobulbar mass. Fine-needle aspirations and incisional biopsies resulted in discordant diagnoses: myxosarcoma/myxoma or rhadomyosarcoma, respectively. Immunohistochemistry following exenteration allowed definitive diagnosis of malignant peripheral nerve sheath tumor with fibromyxomatous differentiation. Fifteen weeks after surgery, an aggressive recurrence resulted in euthanasia.
Topics: Animals; Dog Diseases; Dogs; Female; Immunohistochemistry; Myxoma; Neoplasm Recurrence, Local; Nerve Sheath Neoplasms; Orbit Evisceration
PubMed: 29606723
DOI: No ID Found -
Journal of the American Veterinary... Dec 2016CASE DESCRIPTION A 15-year-old neutered female mixed-breed dog (dog 1) and an 11-year-old neutered female Labrador Retriever (dog 2) were examined because of unilateral...
CASE DESCRIPTION A 15-year-old neutered female mixed-breed dog (dog 1) and an 11-year-old neutered female Labrador Retriever (dog 2) were examined because of unilateral exophthalmus, third eyelid protrusion, and periorbital swelling that failed to respond to antimicrobial treatment. CLINICAL FINDINGS Both dogs underwent ultrasonographic, CT, and MRI examination of the head. In both dogs, advanced imaging revealed a poorly defined, peripherally contrast-enhancing, mucous-filled cystic mass that radiated from the temporomandibular joint and infiltrated the periorbital tissues and retrobulbar space. Both dogs underwent surgical biopsy of the periorbital mass. A viscous, straw-colored fluid was aspirated from the retrobulbar region in both dogs. The initial histologic diagnosis for dog 1 was zygomatic sialadenitis and sialocele. However, the clinical signs recurred, and histologic examination of specimens obtained during a second surgical biopsy resulted in a diagnosis of myxoma. The histologic diagnosis was myxosarcoma for dog 2. TREATMENT AND OUTCOME In both dogs, clinical signs recurred within 2 weeks after surgery and persisted for the duration of their lives. Dog 1 received no further treatment after the second surgery and was euthanized 34 months after initial examination because of multicentric lymphoma. Dog 2 was treated with various chemotherapy agents and was euthanized 11 months after initial examination because of a dramatic increase in periocular swelling and respiratory stertor. CLINICAL RELEVANCE Temporomandibular myxomatous neoplasia can be confused with zygomatic sialocele on the basis of clinical signs but has characteristic MRI features. Representative biopsy specimens should be obtained from areas close to the temporomandibular joint to avoid misdiagnosis.
Topics: Animals; Antineoplastic Agents; Dog Diseases; Dogs; Female; Myxoma; Temporomandibular Joint Disorders
PubMed: 27875090
DOI: 10.2460/javma.249.11.1301