Desmoplastic small round cell tumor of the pleura

In July 2006 a 19-year-old woman presented with a history of few months of back ache. Routine chest radiograph and computed tomography (CT) examination revealed a thickening of the parietal pleura with a collapsed right lung. The abdominal pelvic CT scan was normal. Thoracotomy was performed with decortication lung, parietal pleurectomy and pleural biopsy. The histological examination and immunohistochemical coexpression of cytokeratin AE1-AE3, vimentin and desmin confirmed the diagnosis of desmoplastic small round cell tumor of the pleura. After resection, the patient received radiotherapy and chemotherapy with doxorubicin, ifosfamide, etoposide and cisplatin in six cycles which resulted in complete remission. The patient was seen regularly in consultation; the last examination was in December 2010 and clinically and radiologically showed a stable condition. The whole follow-up period was 53 months. We thought this case to be worth presentation because of the unusually long survival, which is probably due to the aggressive complex anticancer treatment.