Epithelioid sarcoma: one institution's experience with a rare sarcoma.

Area of Science:

• Oncology
• Soft Tissue Sarcoma Research
• Cancer Epidemiology

Background:

• Epithelioid sarcoma (ES) is an exceptionally rare soft tissue sarcoma.
• Limited data exists on the clinical behavior and treatment response of ES.

Purpose of the Study:

• To describe the clinical characteristics, treatment patterns, and outcomes of epithelioid sarcoma.
• To evaluate the impact of recurrence and resection on survival in ES patients.

Main Methods:

• Retrospective review of 22 epithelioid sarcoma cases from Johns Hopkins Medical Institution.
• Pathologist review of patient charts and histological slides.
• Analysis of recurrence rates, time to recurrence, and overall survival.

Main Results:

• Median age at diagnosis was 27.8 years, with 75% of patients being male.
• Regional lymph node metastasis occurred in 10% of patients.
• Tumor recurrence was common (57.9%), with a median time to recurrence of 1.23 years, but did not impact survival.
• Median overall survival was 56.2 months, with a 5-year survival rate of 92%.

Conclusions:

• Epithelioid sarcoma presents as a rare tumor with a protracted, recurrent course.
• Aggressive and repeated surgical resection appears beneficial for patient outcomes.
• ES characteristically metastasizes to regional lymph nodes, necessitating extended surveillance due to potential late recurrences.