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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 -
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 -
Brachytherapy 2016Prior studies illustrated a reduction in wound complications with the use of staged reconstruction (SR) and negative pressure wound therapy when treating soft tissue...
PURPOSE
Prior studies illustrated a reduction in wound complications with the use of staged reconstruction (SR) and negative pressure wound therapy when treating soft tissue sarcoma (STS) with surgical resection followed by high-dose-rate adjuvant brachytherapy. The purpose of this study is to compare the outcomes of SR and immediate reconstruction (IR) brachytherapy in recurrent STS.
METHODS AND MATERIALS
A retrospective review of 40 patients with recurrent STS of the local extremity and trunk treated with resection followed by adjuvant brachytherapy alone. Margin status was defined as positive (SM(+)) if there was microscopic involvement (R1) or ≤1 mm margin and negative (SM(-)) if >1 mm margin was obtained. SR and IR were compared regarding toxicity, local control, and limb preservation.
RESULTS
Median followup was 27 months. When comparing the SR (n = 22) and IR (n = 18) cohorts, there was a significantly lower final SM(+) rate in SR (32% vs. 83%, p < 0.01). A 2-year local control benefit seen with SR (80% vs. 34%; p = 0.012) and a final SM(-) (81% vs. 39%; p = 0.023). SR was associated with less toxicity on multivariate analysis, including a 90% decrease in persistent edema, an 80% decrease in wound dehiscence, and a 94% decrease in nonhealing wounds, when compared to IR. Ten of 31 (32%) extremity cases required eventual amputation from either chronic wound complications (n = 4) or local recurrence (n = 6). SR predicted for a benefit in 2-year limb preservation (88% vs. 50%; p = 0.008).
CONCLUSION
In our series, the treatment with SR brachytherapy resulted in less morbidity and an improved final SM(-) rate. This technique translated to an improvement in both local control and limb preservation of recurrent STS.
Topics: Adult; Aged; Aged, 80 and over; Brachytherapy; Extremities; Female; Fibrosarcoma; Follow-Up Studies; Humans; Liposarcoma; Male; Margins of Excision; Middle Aged; Multivariate Analysis; Myxosarcoma; Negative-Pressure Wound Therapy; Neoplasm Recurrence, Local; Radiotherapy Dosage; Radiotherapy, Adjuvant; Plastic Surgery Procedures; Retrospective Studies; Sarcoma; Soft Tissue Neoplasms; Surgical Flaps; Time Factors; Wound Healing; Young Adult
PubMed: 27180128
DOI: 10.1016/j.brachy.2016.03.013 -
BMJ Case Reports Mar 2016Primary intracranial myxofibrosarcoma is exceedingly rare, with less than 10 cases published. We present a case of a 23-year-old man with previous history of a primary...
Primary intracranial myxofibrosarcoma is exceedingly rare, with less than 10 cases published. We present a case of a 23-year-old man with previous history of a primary low grade myxofibrosarcoma of the left parietal-occipital convexity resected in March 1999. He subsequently underwent several interventions for multiple local recurrent disease until March 2004. At that time, complete remission was documented. About 8 years later, in February 2012, the patient was admitted to the emergency room with refractory acute pulmonary oedema. On work up, sustained monomorphic ventricular tachycardia and hyperechoic myocardial mass with invasion of the right ventricular cavity were detected. Electrical cardioversion was unsuccessful and irreversible cardiac arrest followed. The autopsy confirmed multiple bilateral lung metastases, malignant pulmonary embolism and myocardial invasion by the primary tumour, with intracavitary cardiac thrombosis and absence of intracranial disease. To the best of our knowledge, this is the first report of extracranial metastases of this neoplasm.
Topics: Brain Neoplasms; Fatal Outcome; Fibrosarcoma; Heart Neoplasms; Humans; Lung Neoplasms; Male; Myxosarcoma; Neoplasms, Second Primary; Pulmonary Edema; Young Adult
PubMed: 27013654
DOI: 10.1136/bcr-2015-214052 -
Anais Brasileiros de Dermatologia 2016Myxofibrosarcoma is recognized as a malignant neoplasm of fibroblastic origin with increased prevalence in the elderly, presenting as nodules or tumors that may extend...
Myxofibrosarcoma is recognized as a malignant neoplasm of fibroblastic origin with increased prevalence in the elderly, presenting as nodules or tumors that may extend to the dermis and skeletal muscle, preferably in the lower limbs. Histologically it is characterized by a proliferation of spindle cells in a myxoid stroma. Myxofibrosarcoma has a high potential for local recurrence and metastasis, mainly when it presents a high or intermediate histological grade. We report the case of an eighty-four year old patient with a difficult diagnosis of a highly aggressive tumor.
Topics: Aged, 80 and over; Biopsy; Fibroma; Humans; Immunohistochemistry; Lymphatic Metastasis; Magnetic Resonance Imaging; Male; Myxosarcoma; Neoplasm Recurrence, Local; Skin; Skin Neoplasms
PubMed: 26982788
DOI: 10.1590/abd1806-4841.20163922 -
Journal of the American Veterinary... Sep 2015
Topics: Animals; Bone Nails; Cat Diseases; Cats; Female; Myxosarcoma
PubMed: 26331415
DOI: 10.2460/javma.247.6.597 -
Journal of Clinical and Diagnostic... Mar 2015Myxosarcoma is a rare malignant neoplasm of connective tissue which is characterized by tissue that resembles primitive mesenchyme, and contains relatively...
Myxosarcoma is a rare malignant neoplasm of connective tissue which is characterized by tissue that resembles primitive mesenchyme, and contains relatively undifferentiated cells that show rapid growth and invasion. The cells are stellate or spindle-shaped and are present in a loose matrix which contains mucoid material, reticulum, and collagen fibers. This paper reports a rare case of a myxosarcoma of the left maxilla in a 50-year-old male, which clinically presented as a soft-tissue mass on the buccal gingiva and radiographically showed a mixed radiolucent-radiopaque appearance.
PubMed: 25954712
DOI: 10.7860/JCDR/2015/11482.5662 -
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 -
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 -
Journal of the American Veterinary... Dec 2014
Topics: Animals; Bone Neoplasms; Dog Diseases; Dogs; Male; Myxosarcoma
PubMed: 25406700
DOI: 10.2460/javma.245.11.1221