Fibrosarcoma sclerosed epitheloid cellC49.9
Synonym(s)
epithelioid sarcoma; epitheloid; Sarcoma; sclerosed epithelioid cell sarcoma; Sclerosed epitheloid cell fibrosarcoma; sclerosing epithelioid fibrosarcoma; Soft tissue tumor Enzinger
HistoryThis section has been translated automatically.
Enzinger, 1970
DefinitionThis section has been translated automatically.
Rare, malignant, slow-growing, nodular soft tissue tumor located in deep soft tissue of the extremities and trunk.
ManifestationThis section has been translated automatically.
Especially in young adulthood and preferentially occurring in the male sex.
LocalizationThis section has been translated automatically.
Especially the sides of the fingers, back of the hands, soles of the feet and the extensor sides of the forearms and thighs are affected.
Clinical featuresThis section has been translated automatically.
Slowly growing, coarse, usually painful, cutaneous-subcutaneously located node, which slowly increases along the fasciae and nerve cords. At the time of diagnosis, the tumor node is usually no larger than 5.0 cm. Later tendency to ulceration. Often the ulceration is in the foreground, so that the tumour can impress under the image of a non-healing ulcer. A specific feature is the spread along nerves and vessels and fasciae.
HistologyThis section has been translated automatically.
- Infiltrating growing, granuloma-like tumor of epithelioid and spindle-shaped cells, central necroses, vascular invasion, often involving tendons and fascia.
- Frequent expression of MUC4 protein; furthermore focal expression of S-100 protein and EMA (= Epithelial Membrane Antigen, also called MUC1)
Differential diagnosisThis section has been translated automatically.
- Clinical:
- Lipoma
- Fibroma
- annular granuloma
- Rheumatic nodules
- Muscle trauma
- Ulcer of different etiology
- Atypical mycobacteriosis
- Dupuytren's contracture in the palm
- Histological:
TherapyThis section has been translated automatically.
Excision with generous safety distance and subsequent radiotherapy for small tumours; en-bloc resection and adjuvant chemotherapy for larger tumours.
Progression/forecastThis section has been translated automatically.
Pronounced local recurrence tendency, late metastasis in lymph nodes and lungs.
LiteratureThis section has been translated automatically.
- Arbajian E et al (2014) Recurrent EWSR1-CREB3L1 gene fusions in sclerosing epithelioid fibrosarcoma. At J Surg Pathol 38: 801-808.
- Aung PP et al (2012) KBA62 and PNL2: 2 new melanoma markers-immunohistochemical analysis of 1563 tumors including metastatic, desmoplastic, and mucosal melanomas and their mimics. On J Surg Pathol 36:265-272.
- Bohner H et al (2003) Primary malignant tumors of the aorta: clinical presentation, treatment, and course of different entities. J Vasc Surgery 38: 1430-1433
- Enzinger FM (1970) Epitheloid sarcoma: a sarcoma simulating a granuloma or a carcinoma. Cancer 26: 1029-1041
- Fisher C (2014). The diversity of soft tissue tumours with EWSR1 gene rearrangements: a review. Histopathology 64:134-150.
- Hantschke M et al (2010) Cutaneous clear cell sarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma. Am J Surg Pathol 34:216-222.
- Rödder-Wehrmann O et al (1990) Epitheloid sarcoma. dermatologist 41: 562-565
- Sarica K et al (1999) Epitheloid sarcoma of the penis. Urol Int 62: 124-126
- Sundaram M et al (2011) Primary multicentric cutaneous epitheloid angiosarcoma. Indian J Dermatol Venereol Leprol 111 PubMed PMID: 21220905.