Ewing sarcoma of the hand: A rare case report with diagnostic and management considerations

Ewing sarcoma (ES) is a rare malignant tumour primarily affecting adolescents, typically involving the axial and appendicular skeleton. Its occurrence in the hand is exceedingly rare, comprising less than 1 % of all cases. Early diagnosis is often challenging due to its nonspecific symptoms and similarities to other conditions. This case report highlights a rare presentation of Ewing sarcoma in the hand.

A 20-year-old male presented with a six-month history of swelling and discomfort in the left thumb, without prior injury or inflammatory symptoms. Physical examination revealed a firm, non-tender, fusiform swelling involving the proximal and distal phalanges. Radiographic imaging showed a mass affecting the bone, with signs of periosteal reaction and cortical disruption. MRI revealed a well-demarcated soft tissue mass contacting the flexor tendon, skin, and neurovascular bundle, with bone involvement. Potential differential diagnoses included giant cell tumour, enchondroma, and aneurysmal bone cyst. Following consent, thumb amputation was performed, revealing a grey tumour surrounding the bone and soft tissues. Histopathological analysis revealed monomorphic small round cells with finely split chromatin and CD99 positivity which confirmed Ewing's sarcoma. Postoperative chemotherapy included Vincristine, Ifosfamide, Doxorubicin, Etoposide, and Actinomycin-D. After one year of follow-up, there was no evidence of recurrence or metastasis.

Ewing sarcoma of the hand is rare, often misdiagnosed as benign conditions or infection due to its soft tissue presentation and bone involvement. Timely diagnosis and surgical intervention, followed by chemotherapy, are crucial for effective treatment. The prognosis for hand Ewing sarcoma is favourable with early treatment, and survival rates for localized disease are around 70-80 %.

Importance of early suspicion in atypical soft tissue swellings of the hand in young adults is crucial. Ewing sarcoma in the hand, although rare, should be considered in young patients presenting with unexplained soft tissue masses. Early detection and a multidisciplinary approach involving surgery and chemotherapy can lead to favourable outcomes.