Ewing sarcoma of the hand

Case contributed by Aravinda Perera
Diagnosis certain

Presentation

Enlarging non-painful mass on the dorsum of left hand.

Patient Data

Age: 3 years
Gender: Male
mri

A soft tissue lesion is seen between the left fourth and fifth metacarpals centered within the left fourth dorsal interosseous muscle. This lesion demonstrates increased T1 signal compared to surrounding muscles. Heterogeneous peripheral enhancement with central sparing is demonstrated.

The lesion is displacing and partially effacing the fourth and fifth metacarpals but no definite bone marrow involvement is seen. No periosteal reaction is evident.

The lesion extends dorsally deep to the extensor tendons. The volar aspect of the lesion displaces the flexor tendons anteriorly. 

Subtle signal change and enhancement in the distal fifth metacarpal may represent a stress response.

IMPRESSION:

Nonspecific soft tissue tumor is seen between the fourth and fifth metacarpals without definite bony involvement. The primary consideration would be a sarcoma or fibrous tumor. The mass effect and remodeling of adjacent bones would be against acute pathology such as infection.

Case Discussion

Initial investigation of this dorsal hand mass involved an ultrasound which revealed a heterogeneous vascular lesion. This lesion was further characterized with MRI of the affected hand which demonstrated a nonspecific soft tissue lesion, prompting further workup.

A biopsy of the lesion was performed using a dorsal approach. Formal histological analysis confirmed a diagnosis of Ewing sarcoma. 

Following diagnosis, staging imaging was performed. Fortunately, no sites of metastatic disease were identified. The patient went on to have neoadjuvant chemoradiotherapy prior to undergoing a fourth and fifth ray amputation of the left hand as definitive surgical treatment for the tumor. A local fasciocutaneous advancement flap and reconstruction was required to attain closure and adequate cosmetic outcome following the aforementioned ray amputations.

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