SynopsisCodesDifferential Diagnosis & PitfallsBest TestsReferences
Clear cell sarcoma
Other Resources UpToDate PubMed

Clear cell sarcoma

Contributors: Benjamin L. Mazer MD, MBA
Other Resources UpToDate PubMed


A rare malignant neoplasm thought to be a variant of melanoma. It occurs in fascia and tendon sheaths, principally in the lower extremities. It does not typically occur concurrently with a primary skin melanoma. It most commonly manifests as a painless mass. Clear cell sarcoma most commonly occurs in women aged 20-40. It is treated with varying combinations of surgical excision, chemotherapy, and radiation. Clear cell sarcoma frequently recurs and metastasizes.


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

402561003 – Malignant melanoma of soft tissues

Differential Diagnosis & Pitfalls

  • Malignancies of deep and soft tissues (Lipoma, Epithelioid sarcoma, Cutaneous neuroma, Malignant peripheral nerve sheath tumor, Osteosarcoma, vascular tumors, metastasis, etc)
  • Skin bacterial abscess
  • Osteoma cutis and osteoid formation / Calcinosis cutis
  • Cutaneous myxoma
  • Cellulitis
  • Necrotizing fasciitis
  • Neurofibromatosis
  • Nodular fasciitis
  • Deep vein thrombosis
  • Trauma
  • Lymphedema
  • Lymphadenopathy
  • Cat-scratch disease

Best Tests

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Last Updated:05/17/2016
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Clear cell sarcoma
A medical illustration showing key findings of Clear cell sarcoma : Ankle pain, Foot pain, Knee pain, Soft tissue swelling
Copyright © 2024 VisualDx®. All rights reserved.