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Epithelioid sarcoma
Other Resources UpToDate PubMed

Epithelioid sarcoma

Contributors: Vivian Wong MD, PhD, Whitney A. High MD, JD, MEng, Noah Craft MD, PhD
Other Resources UpToDate PubMed


Epithelioid sarcoma (ES) is a rare, malignant soft tissue tumor. It accounts for less than 1% of all soft tissue sarcomas. ES can mimic other dermatological entities, confounding the diagnosis.

ES presents usually as an indolent, hard, tender nodule on the distal extremities of a young person (typically less than 40 years). Men are preferentially affected. Lesions on the head and neck have been reported. Aberrant mesenchymal tissue differentiation may contribute to the pathogenesis of ES. A history of trauma may precede the eruption of this lesion, although a causal relationship has not been established. The lesions often ulcerate.

ES often has an unpredictable clinical course. ES often has a tendency for recurrence and aggressive growth. Regional lymph node involvement and distant metastases to the lung, bones, brain, and other organs, and spread along connective tissue planes and neurovascular bundles, have been reported. A delay in diagnosis is not uncommon.

In a proximal / axial variant, with an adverse prognosis due to higher recurrence, earlier metastasis has been described. Ulcerating, angiomatoid / angiosarcoma-like, and fibrous histiocytoma-like / fibroma-like variants have also been reported.


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

59238007 – Epithelioid sarcoma

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Differential Diagnosis & Pitfalls

  • Necrotizing granuloma
  • Epithelioid vascular neoplasms
  • Amelanocytic Melanoma
  • Angiosarcoma of skin
  • Metastatic cutaneous carcinoma
  • Synovial sarcoma
  • Rhabdomyosarcoma
  • Malignant mesothelioma
  • Giant cell tumor of tendon sheath
  • Clear cell sarcoma of the tendon and aponeurosis
  • Fibrosarcoma
  • Atypical fibrous xanthoma
  • Fibrous histiocytoma
  • Cutaneous squamous cell carcinoma
  • Chronic granulomatous inflammation, such as Rheumatoid nodule
  • Nodular fasciitis
  • Fibromatosis
  • Granuloma annulare
  • Schwannoma
  • Synovial sarcoma
  • Epithelioid Hemangioendothelioma
  • Epithelioid Cutaneous leiomyosarcoma
  • Malignant extrarenal Rhabdoid tumor of soft tissue
  • Necrobiosis lipoidica

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Last Updated:11/10/2021
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Epithelioid sarcoma
A medical illustration showing key findings of Epithelioid sarcoma : Erythema, Hemorrhagic skin lesion, Perineum, Skin ulcer, Tumor, Inguinal region, Firm nodule
Clinical image of Epithelioid sarcoma - imageId=5615696. Click to open in gallery.
Copyright © 2024 VisualDx®. All rights reserved.