Abstract
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Introduction:
Clear cell sarcoma (CCS) is a rare malignant tumor few described in the literature, with a predilection for lower extremities and a tendency to involve tendons and fascia. Patients usually present late while the disease is progressing without them noticing it. late symptom because of the slow progression of the disease. Clinical and radiological findings are not sufficient. Histological and immunohistochemical analysis are necessary to make the diagnosis. The effective treatment is surgical by carcinological tumor resection.
Case presentation:
We report the case of a 47 years old male patient undergoing initial biopsy-excision surgery of a lesion labeled tenosynovial giant cell tumor on MRI. After histological and immunohistochemical analysis, the diagnosis showed a clear cell sarcoma of the anterior tibial tendon. The patient underwent an extensive
revision surgery .
Results:
The immunohistochemical results correspond to a high-grade clear cell sarcoma with translocation of the EWSR1 gene. The extension workup after initial excision showed no tumor residue or lesions suspicious of distant metastasis. The pathology results of the post-relapse are favorable and consistent with resection in a healthy area.
Conclusion and outlook:
The patient did not present any local or systemic recurrence and showed a good function of his anterior tibial tendon plasty at the 1-year follow-up.
Key words: clear-cell sarcoma, soft tissue tumor, anterior tibial tendon
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