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Malignant fibrous histiocytoma - Skin
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Malignant fibrous histiocytoma - Skin

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Contributors: Chris G. Adigun MD, Jeffrey D. Bernhard MD, Belinda Tan MD, PhD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma. More recent analysis of this tumor has revealed that it is a morphologic pattern, rather than a distinct pathologic entity. MFH is essentially a synonym for undifferentiated pleomorphic sarcoma, not otherwise specified, and requires extensive immunohistochemical examination to classify individual lesions into defined histogenic subtypes of sarcoma. The etiology of these aggressive soft tissue sarcomas is unknown. They occur most commonly in the skeletal muscle or retroperitoneum, typically beneath the fascia, but occasionally arise in the subcutaneous tissue. May occur in tattoos.

The most common forms of MFH to present in the skin include the myxofibrosarcoma, formerly known as myxoid MFH, and the undifferentiated pleomorphic sarcoma, formerly known as pleomorphic and storiform MFH. Both of these tumors characteristically occur later in adult life and are rare in young people. There is a predominance of cases in men over women. The most common site of presentation is on the limbs, but lesions may occur on any area of the body. These tumors may enlarge rapidly and may be as large as 5-10 cm at initial clinical presentation. MFH usually presents as a flesh-colored subcutaneous nodule, with minimal if any overlying epidermal change.

Approximately 50% of patients will have distant metastasis at initial presentation, with the overwhelming majority of these metastases occurring in the lung (90%). Other sites of metastasis include the bone (8%) and liver (1%). However, of those tumors that arise in the subcutis, only approximately 10% are expected to metastasize. Recurrence after treatment is common.

Pediatric Patient Considerations:
Less than 5% of cases of MFH occur in childhood. When they do, sites of predilection are the head and neck regions. Although prognosis is slightly better than for adults, overall it is poor, with high rates of metastasis and recurrence.

For more information, see OMIM.

Codes

ICD10CM:
C49.9 – Malignant neoplasm of connective and soft tissue, unspecified

SNOMEDCT:
404014008 – Malignant fibrous histiocytoma of skin

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Last Updated: 03/29/2017
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Malignant fibrous histiocytoma - Skin
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Malignant fibrous histiocytoma : Arm, Cheek, Leg, Nose, Plaque with ulcer, Red color, Scalp, Smooth nodule, Smooth papule
Clinical image of Malignant fibrous histiocytoma
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