Sarcoma, myxofibrosarcoma

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

malignant fibrosis histiocytoma; Myxofibrosarcoma; : Myxofibrosarcoma; Myxoides MFH; myxoid variant

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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.

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.

  1. Olson MT et al (2012) Myxofibrosarcoma: cytomorphologic findings and differential diagnosis on fine needle aspiration. Acta Cytol 56:15-24
  2. 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
  3. 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

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer