[Askin's tumor--a rare entity. Case report with references to the literature]

Askin's Tumour (synonym: primitive neuroectodermal tumour) is a rare neoplasm of the chest wall. In 1979 Askin and Rosai described an unique clinicopathological entity, characterised as a malignant small-cell tumour of the soft tissues of the chest wall in childhood and adolescence. We report on a case of a 28-year old male who had a massive tumour in the left thorax with association to the chest wall. The clinical symptoms were dyspnoea and increasing chest pain. The tumour was revealed as a sarcoma of the lung by CT-guided fine needle aspiration. A pneumectomy of the left lung with partial resection of ribs IV. and V. was performed. Postoperative histology revealed an Askin's tumour of the chest wall with infiltration of the lung. After surgical treatment in our hospital we transferred the patient to an oncological centre for adjuvant chemotherapy. As a consequence of aggressive growth of tumour therapy should be performed in oncological centres in clinical studies. The treatment includes radical surgical resection, neoadjuvant and adjuvant chemotherapy plus radiation. In our case the primary resection was performed because of increasing symptoms. It is difficult to establish an accurate preoperative diagnosis of Askin's tumour. Microscopy and immunohistological stain of the specific marker--neuron-specific enolase--are essential. Multimodal treatment allows a long-term survival, but often the prognosis is infaust.