Case Report: Rapidly fatal Askin’s tumor: a case report and literature review

Abstract

An 18-year-old male presented with a mass in the right anterior chest wall. Chest Computed tomography  revealed a heterogenous mass of 19X13 cm in the right hemithorax with areas of necrosis. There was  associated pleural effusion and infiltration of the soft parts of the chest wall. Bronchoscopy showed a tumor in middle lobe bronchus. CT guided biopsy of the mass was performed. Histological examination showed small round tumor cells with scanty cytoplasm, the nuclei are large and hyperchromatic. The tumor cells were  positive for CD99 and neuron specific enolase, negative for cytokeratin, leukocyte common antigen and  myogenin. Based on these histologic and immunohistochemical findings, the diagnosis of askin's tumor was made. The extension assessment was negative and the patient was given chemotherapy. Two months later, our patient died. Askin's tumor is a rare, highly malignant tumor affecting children and young adults. It is classified as primitive neuroectodermal tumor of the thoracopulmonary region. Prognosis remains poor. In our case, several prognostic factors may explain the shirt 'term survival, despite no distant metastasis were found: important tumor size, impossibility of surgical treatment and pleural effusion.

Key words: Askin tumor, chest wall, primitive neuroectodermal tumor, Ewing sarcoma, malignancy