PO-0363Askin Tumour:case Report

IntroductionAskin tumour is a primitive neuroectodermal tumour of the thoracopulmonary region. Pulmonary location is not common. We report a case of an Askin tumour in a child who presented with worsening breathlessness.ObservationA 9 years-old female child with no pathological history, was admitted with severe dyspnea up to one week. A Weight loss was reported. Physical examination showed silence in pulmonary auscultation of the left lung field. A chest X-ray found a left opacity occupying almost the entire of the left lung, mediastinal compression to the right side and nibbled aspect of the fourth rib. Chest Ultrasound showed pleural effusion with finely heterogeneous liquid. Chest CT showed a pulmonary solid tumour with aspect of massive necrosis of the left upper lobe with hemothorax, rib erosion in contact, without lymphadenopathy or vascular invasion or secondary lesion distance. A scano-guided biopsy was performed and immuno-histological examination found a small round cell tumour with morphological aspects favour the diagnosis of primitive peripheral neuroectodermal tumour corresponding to an Askin tumour. The child underwent an intensive chemotherapy and surgery.ConclusionThe management of Askin tumour is not well codified. The prognosis of these tumours remains very unfavourable because of their metastatic risk and potential local recurrence.