Introduction

Clear cell sarcoma (CCS) is a soft tissue sarcoma (STS) that arises from the tendon and aponeurosis [1-3]. Described by Enzinger in 1965 [4], it accounts for less than 1% of all STSs, with an estimated incidence of 0.014 per 100,000 people [1,3,5]. It stands out for its high metastatic spread and a poor prognosis [6].

In the approach to a tumor, radiography helps to discern bone or soft components. Ultrasound (US) can report cystic or solid lesions, computed tomography (CT) details bone characteristics, and magnetic resonance imaging (MRI), the method of choice to detail soft tissue lesions, informs the relationship with neighboring structures [2,5,7].

Diagnostic confirmation requires biopsy, preferably by percutaneous technique with an image-guided core needle, or, failing that, an open biopsy can be used. This approach is essential to define the therapeutic strategy and establish the prognosis [2,8]. Treatment includes en bloc resection; hand amputation is the most common outcome for this sarcoma due to its aggressiveness. Adjuvant radiation therapy is described in lesions >5 cm [2].

In the present report, the lesion resulting from the resection of the CCS showed significant structural alterations in the patient's hand. Therefore, the plastic surgery team performed a reconstruction technique specifically planned to maximize the functional restoration of the affected hand.