DOI: 10.36347/sjmcr.2023.v11i02.022

Pages: 193-197

Introduction: Clear cell sarcoma (CCS), is an extremely rare type of sarcoma, frequently diagnosed in young adults, typically arises in the extremities of the limbs and has a predilection for occurring in the tendons and aponeuroses. In the past CCS was called malignant melanoma of the soft parts because the tumor shares clinical, histological and immunohistochemical similarities with amelanotic melanoma, but it is a unique lesion, distinct from melanoma. Case Report: A 33-year old woman with no significant history was referred with an unpainful bulky ulcerated tumor of the right ankle, appeared 05 years prior to presentation following a minimal trauma. The CT of the ankle revealed a bulky mass of the medial surface locally infiltrative associated to nodules of the surrounding adipose tissues and a unilamellar periosteal reaction without bone lysis or osteocondensation. MRI of the ankle showing a lobulated mass arising in the deep structures in iso-intense signal relative to adjacent muscle on T1 and heterogeneous high-signal intensity on T2 infiltrating surrounding adipose tissues without infiltration of muscles. Histological examination showed dermo-hypodermic malignant tumor proliferation. Round cells were arranged in nests separated by dense collagenous septa, with an abundant eosinophilic to clear cytoplasm. The immunohistochemical supplement were positive for Melan-A and HMB-45. Cytokeratin, Vimentin, Desmin and CD99 were negative. The extension assessment made of a head-chest-abdominal-pelvic CT scan showed multiple homolateral inguinal lymphadenopathies necrotic. No other metastasis was detected. The anatomo-clinical confrontation allowed to retain the diagnosis of CCS. Discussion: The tumor occurs most often in adolescents and young adults and was slightly more common in female than in male patients, CCS typically arises in the extremities of the limbs, its occurrence in the upper limbs is less common, described as a slowly growing mass painless. ..........