Rapidly fatal Askin's tumor: a case report and literature review.

Mustapha Laine,Ismail Abderrahmani Ghorfi,Ahmed Abid,Fouad Kettani,Dalal Lambatten

doi:10.11604/pamj.2014.18.104.4549

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.

Highlights

Askin in 1979 [1], described for the first time a malignant and aggressive tumor of the chest wall, affecting young patients. This tumor is known as Askin tumor, and very rare cases are reported worldwide. This tumor is at present classified as primitive neuroectodermal tumor (PNET) of the thoracopulmonary region and strikes by its histological, immunohistochemical and cytogenetic similarities with Ewings sarcoma [2].To date, no treatment is codified and prognosis remains dark
An 18 years old male presented with a five months history of left chest pain, cough and dyspnea
Physical examination revealed a mass in the right anterior chest wall, dull percussion note and reduced breath sounds over the right lower thorax

Summary

Introduction

Askin in 1979 [1], described for the first time a malignant and aggressive tumor of the chest wall, affecting young patients. Since, this tumor is known as Askin tumor, and very rare cases are reported worldwide. An 18 years old male presented with a five months history of left chest pain, cough and dyspnea. He had previously been in good health. CT guided biopsy of the mass was performed, microscopic examination (Figure 3, Figure 4) showed small round tumor cells with scanty cytoplasm in a rare fibrous stroma. The patient died 2 months after diagnosis, mainly attributed to tumor progression

Findings

Discussion

Conclusion