Epithelioid sarcoma of the hand and upper limb: Clinical and prognostic features

Epithelioid sarcoma is a little-known malignant tumor that affects young people. Preferentially localized to the hand, it is characterized by a misleading clinical and anatomopathological appearance. The aim of this study was to clarify the clinical and long-term prognostic specificities of this tumor of the hand and upper limb.

We retrospectively analyzed the clinical databases of 3 sarcoma referral centers for patients with epithelioid sarcoma of the upper limb. The affected areas on each finger were specified. Survival analysis was performed using the Kaplan Meyer method.

Eighteen patients were included over a mean period of 5.6 years. The average age of the population was 38.9 years. The hand and wrist were the most affected areas of the upper limb. Involvement of the thumb, index finger and first web space accounted for 86% of the patients. There was no injury of the little finger. All hand lesions were palmar. The tumor size ranged from 5 mm to 150 mm. Recurrence occurred in 59% of patients. Death occurred in 8 patients. All presented with large tumors, proximal localization and distant metastases. The 5-year survival rate was 70.1%, and the 10-year survival rate was 57.4%.

This study proposes a precise topographical analysis of the epithelioid sarcoma of the hand. A proximal location above the wrist and a large tumor size are important poor prognostic factors. We recommend that any undetermined palmar skin lesion localized to the thumb, index finger or first web space in individuals aged 20 and 40 undergo a systematic biopsy rather than simple surveillance or additional imaging tests. Delayed diagnosis due to these alternative approaches could have serious consequences.

IV.