Esophageal extraskeletal neoplasm Ewing's sarcoma: Case report

Area of Science:

• Oncology
• Pathology
• Surgical Oncology

Background:

• Ewing sarcoma is a rare, aggressive small round cell tumor predominantly affecting long bones and soft tissues.
• Extraosseous Ewing sarcoma (EES) can occur in various soft tissue locations, with rare instances reported in the esophagus.
• Ewing sarcoma typically affects individuals under 20, making adult cases, especially in the esophagus, highly uncommon.

Purpose of the Study:

• To report an unusual case of primary paraesophageal Ewing sarcoma in a young adult female.
• To discuss the rare presentation, diagnostic challenges, and treatment of extraskeletal Ewing sarcoma in the esophageal region.
• To contribute to the limited literature on esophageal Ewing sarcoma, particularly its extraskeletal variant.

Main Methods:

• A 26-year-old female presented with symptoms suggestive of esophageal cancer.
• Diagnostic investigations, including biopsy and immunohistochemical staining, confirmed primary paraesophageal Ewing sarcoma.
• The patient underwent neoadjuvant chemotherapy followed by McKeown esophagectomy and adjuvant chemotherapy.

Main Results:

• The patient was diagnosed with primary paraesophageal Ewing sarcoma near the gastroesophageal junction.
• Neoadjuvant chemotherapy showed a good response, enabling successful surgical resection via McKeown esophagectomy.
• The patient experienced a smooth postoperative recovery and completed adjuvant chemotherapy.

Conclusions:

• Ewing sarcoma is an exceptionally rare esophageal malignancy, often presenting as an advanced or disseminated disease.
• Delayed diagnosis is a common challenge due to the rarity and varied presentation of esophageal Ewing sarcoma.
• This case represents a rare instance of paraesophageal Ewing sarcoma, underscoring the importance of considering rare diagnoses in esophageal tumors.