Huge malignant localized fibrous tumor of the pleura

Localized fibrous tumor is an unfrequent mesenchymal neoplasm. The malignan t variant of the pleura is exceptional and differential diagnosis with the more frequent benign type or with other neoplasms such as Soft tissue sarco ma and mesothelioma is rarely possible in a preoperative setting. The best treatment of this disease is radical surgical resection. No definitive data exist about the role of chemotherapy, We report a case of a giant right in trathoracic mass whose preoperative diagnosis, from an open biopsy, was con sistent with sarcoma and, in a second review, with fibrous tumor of the ple ura without any indication about malignancy. A right pleuropneumonectomy an d pericardial resection was performed through a right hemiclamshell approac h. Histology demonstrated an aggressive behaviour: high mitosis rate, Ki 67 of 34% and diffuse necrosis were present. In consideration of the apparent local radicality we did not perform any adjuvant ti treatment, Six months after the operation a wide local recurrence was evident and a systemic trea tment with Ifosfamide and Adriamicina is still in progress. So far a good r esponse has been documented. Preoperative diagnosis of malignancy has an im portant role as a therapeutic strategy in management of fibrous tumours of the pleura, When there is suspicion of a malignant form neoadjuvant chemoth erapy can represent a further tool to control poorly differentiated and lar ge tumors, and a wide surgical resection of the lesion must be performed.