Extraskeletal Ewing Sarcoma Disguised as a Vascular Malformation.

Area of Science:

• Orthopedic Oncology
• Pediatric Oncology
• Surgical Pathology

Background:

• Extraskeletal Ewing sarcoma (EES) is a rare soft tissue tumor, representing only 3% of upper extremity lesions.
• EES can be misdiagnosed due to its varied presentation, often mimicking benign conditions like vascular malformations.
• Early and accurate diagnosis is crucial for improving patient outcomes.

Purpose of the Study:

• To highlight the diagnostic challenges of extraskeletal Ewing sarcoma in the upper extremity.
• To emphasize the importance of considering EES in the differential diagnosis of pediatric upper extremity lesions.
• To advocate for a multidisciplinary approach in managing EES.

Main Methods:

• Case report presentation of two pediatric patients with extraskeletal Ewing sarcoma of the upper extremity.
• Review of clinical presentation, initial diagnostic imaging, and histopathological findings.
• Discussion of immunohistochemical confirmation and treatment considerations.

Main Results:

• Two pediatric patients initially diagnosed with vascular malformations were confirmed to have extraskeletal Ewing sarcoma via histopathology and immunohistochemistry.
• The study underscores the potential for misdiagnosis of EES in the upper extremity.
• Prompt diagnosis through a high index of suspicion and appropriate workup is essential.

Conclusions:

• Hand surgeons should maintain a high index of suspicion for extraskeletal Ewing sarcoma in pediatric patients presenting with upper extremity lesions, even when initial imaging suggests benign pathology.
• A multidisciplinary approach involving oncologists, pathologists, and surgeons is vital for optimal management and improved prognosis.
• Timely diagnosis and treatment algorithm implementation are critical for managing this rare pediatric malignancy.