Synonym(s)
malignant fibrosis histiocytoma; Myxofibrosarcoma; : Myxofibrosarcoma; Myxoides MFH; myxoid variant
HistoryThis section has been translated automatically.
Weiss and Enzinger 1978
DefinitionThis section has been translated automatically.
Rare spindle-cell and myxoid sarcoma in elderly people; formerly known as myxoid variant of malignant fibrous histiocytoma (MFH), now considered an entity.
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ManifestationThis section has been translated automatically.
Older people; m>w;
LocalizationThis section has been translated automatically.
extremities, trunk, head/neck; dermis, subcutis, deep soft tissue
Clinical featuresThis section has been translated automatically.
Subcutaneous or deeper, fast-growing, less spectacular, less painful, solid swellings or multilobular nodular formations with intact or ulcerated surface.
HistologyThis section has been translated automatically.
- Pronounced polymorphic cell tumor parenchyma with atypical fibroblasts (pseudolipoblasts), a myxoid stroma and prominent elongated blood vessels,
- Immunohistology: CD68 positive in a high percentage; smooth muscle actin focal +; negativity for CD34, S-100 protein, desmin 33, CK (CK7, CK20, CK5/6, CK18)
TherapyThis section has been translated automatically.
Surgical removal with a safety distance > 3cm.
Progression/forecastThis section has been translated automatically.
High recurrence rate independent of grading. Increased risk of metastasis. Depending on the degree of differentiation, 30-50% of metastases occur in lymph nodes and lungs.
LiteratureThis section has been translated automatically.
- Olson MT et al (2012) Myxofibrosarcoma: cytomorphologic findings and differential diagnosis on fine needle aspiration. Acta Cytol 56:15-24
- Palumbo BT et al (2011) Biology, classification, and management of recurrent myxofibrosarcoma 21 years after resection. At J Orthop (Belle Mead NJ). 40:579-582
- Tsai JW et al (2012) Recurrent Amplification at 7q21.2 Targets CDK6 genes in Primary Myxofibrosarcomas and Identifies CDK6 Overexpression as an Independent Adverse Prognosticator. Ann Surg Oncol Apr 3 Epub ahead of print
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Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer