A case of clear cell sarcoma—A rare malignancy

INTRODUCTION: Clear cell sarcoma (CCS) is a rare tumour of the soft tissue often misdiagnosed, as it shares characteristics with malignant melanoma (MM). Previously, CCS has been characterised, as malignant melanoma of the soft tissue, contemporary immunohistochemical techniques, however, have made this designation obsolete. The true incidence remains unknown, but CCS is believed to represent less than one percent of all sarcomas. PRESENTATION OF CASE: A 22-year-old patient presented with a mass sized 2.6 × 2.7 × 2.7 cm of the left gluteal region, pain, and malaise. Initially, the symptoms were interpreted as an infection. Subsequent, pathological diagnosis after surgical removal was tentatively MM albeit definitive pathological diagnosis was CCS. DISCUSSION: The patient of this case underwent definitive surgical treatment with 2 cm margin. In spite of time delay, because of prolonged time for definitive diagnosis, PET-CT and sentinel lymph node biopsy did not show any metastasis. One-year postoperatively, multidisciplinary follow-up is without suspicion of relapse. CONCLUSION: Accurate and timely diagnosis of CCS are imperative, as initial misdiagnosis, may cause delay and further tumour growth, which is correlated to the prognosis.