Epithelioid sarcoma: clinical, MR imaging and pathologic findings

Area of Science:

• Radiology
• Oncology
• Pathology

Background:

• Epithelioid sarcoma is a rare soft tissue sarcoma.
• Its imaging features and clinical presentation can be diverse.

Purpose of the Study:

• To describe MR imaging characteristics of epithelioid sarcoma.
• To correlate imaging findings with clinical and histologic data.
• To review existing literature on this rare tumor.

Main Methods:

• Retrospective analysis of MR imaging and histologic findings in eight patients.
• Correlation of findings with clinical presentation and outcome.
• Literature review.

Main Results:

• Eight cases of epithelioid sarcoma studied, with tumors ranging from 2.5-19 cm.
• Tumors occurred in extremities, scalp, and paraspinal muscles.
• MR imaging revealed infiltration in seven patients; varied signal characteristics were noted.
• Four patients had lymphadenopathy; one had distant metastases.
• Clinical outcomes were generally poor.

Conclusions:

• Epithelioid sarcoma is an aggressive soft tissue sarcoma with varied presentations.
• It commonly affects distal extremities, is infiltrative, and often involves lymph nodes.
• Suspect epithelioid sarcoma in intramuscular masses or ulcerating cutaneous nodules.