Sarcoma, myxofibrosarcoma

Synonym(s)

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

History
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Weiss and Enzinger 1978

Definition
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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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Dermatology

Solitary, slow-growing, sharply defined, symptom-free, red plaque with loose scale deposits.

Warty dyskeratoma

Rare, mostly solitary (only very occasionally multiple), benign, usually verrucous tumor with histologic si...

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Manifestation
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Older people; m>w;

Localization
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extremities, trunk, head/neck; dermis, subcutis, deep soft tissue

Clinical features
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Subcutaneous or deeper, fast-growing, less spectacular, less painful, solid swellings or multilobular nodular formations with intact or ulcerated surface.

Histology
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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)

Therapy
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Surgical removal with a safety distance > 3cm.

Progression/forecast
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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.

Literature
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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