1/7. Huge malignant localized fibrous tumor of the pleura.Localized fibrous tumor is an unfrequent mesenchymal neoplasm. The malignant variant of the pleura is exceptional and differential diagnosis with the more frequent benign type or with other neoplasms such as soft tissue sarcoma 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 intrathoracic mass whose preoperative diagnosis, from an open biopsy, was consistent with sarcoma and, in a second review, with fibrous tumor of the pleura without any indication about malignancy. A right pleuropneumonectomy and pericardial resection was performed through a right hemiclam-shell approach. 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 treatment. Six months after the operation a wide local recurrence was evident and a systemic treatment with ifosfamide and Adriamicina is still in progress. So far a good response has been documented. Preoperative diagnosis of malignancy has an important role as a therapeutic strategy in management of fibrous tumours of the pleura. When there is suspicion of a malignant form neoadjuvant chemotherapy can represent a further tool to control poorly differentiated and large tumors, and a wide surgical resection of the lesion must be performed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/7. Poorly differentiated synovial sarcoma: a case report.Poorly differentiated synovial sarcoma is a rare soft tissue tumor. We studied a case arising in the pleural cavity of a young subject, characterised by the presence of spindle cell, small cell, and large epithelioid cell areas. We performed stains for mucosubstances and analysed the expression of cytokeratins 5/6, 7, 8, 18, 19, CEA, CD34, Ber-Ep4 and calretinin to characterize the phenotype of this neoplasm. We furthermore assessed immunohistochemically the presence of p53, Bcl-2, Bax and caspase 3, four apoptotic markers, to evaluate a relationship between apoptotic activity and the behaviour of this tumor. Our findings showed a strong presence of calretinin, p53 and Bcl-2 in all three areas. The possibility that poorly differentiated synovial sarcoma could be calretinin-positive was a new data, to our knowledge, and it could be of some importance in diagnostic pathology. Moreover, the negligible positivity for Bax and caspase 3 suggested that the minor role of programmed cell death could be one of the causes of the aggressive behaviour of this tumor. These data also suggest that the reduction of apoptotic phenomena in poorly differentiated synovial sarcoma could be considered one of the major mechanisms of tumoral growth.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
3/7. Severe hypoglycaemia associated with a giant solitary fibrous tumor of the pleura.solitary fibrous tumors (SFT) of the pleura are a rare neoplasm, with benign biological behaviour. Recurrences are rare, and no distant metastases are described in the literature. SFT can secrete hormone-like substances, responsible for paraneoplastic syndromes. The authors describe a case of severe hypoglycaemia due to insulin-like growth factor ii (IGF-2)'s secretion by a giant SFT of the pleura. Hypoglycaemia was controlled by the resection of the tumor. diagnosis and surgical management of these neoplasms are also discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/7. Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review.Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour of intermediate behaviour. It can arise from various sites including the liver, spleen, pleura, or lung. Cutaneous EHE can be primary or secondary. This report describes the case of a 51 year old man who presented with a history of dry cough, shortness of breath, and pleural effusion, and who developed two cutaneous nodules in the anterior abdominal wall a few weeks later. He had a previous history of asbestos exposure. Computed tomography scan showed a left sided pleural effusion and nodular pleural mass. histology of both the pleural and cutaneous lesions was compatible with EHE. Electron microscopic examination demonstrated the presence of weibel-palade bodies. The patient underwent elliptical excision of the metastatic cutaneous nodules after decortication of the primary pleural tumour and adjuvant treatment. A few reports have described metastasis of intrathoracic EHE to the skin. Despite treatment with interferon, the patient developed more cutaneous lesions two years after the initial diagnosis. Even though the tumour has the classic light histological and ultrastructural features of EHE, it behaved in an aggressive manner.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/7. recurrence and malignant transformation in solitary fibrous tumour of the pleura.Solitary fibrous tumours of the pleura are mesenchymal neoplasms and recurrence with malignant progression after complete resection rarely occurs. In the case of a 76-year-old patient we found multifocal recurrence with malignant transformation within six months following primary excision. Even though the vast majority of these tumours are benign, complete resection as well as clinical and radiological follow-up are highly recommended because of the potentially adverse biological behaviour and the lack of radical treatment options other than surgery.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/7. Metastasizing ectopic thymoma arising in the right thoracic cavity and mimicking diffuse pleural mesothelioma--an autopsy study of a case with review of literature.A case of metastasizing ectopic thymoma arising in the right thoracic cavity and infiltrating the right lung and pleura in a fashion reminiscent of diffuse pleural mesothelioma is presented. An involuted, non-neoplastic thymus was demonstrated microscopically in the anterior mediastinum. No case of thymoma showing the combination of ectopic location and biological malignancy has yet been reported in the literature. The pathogenesis of ectopic thymuses and thymomas is discussed from an embryological standpoint. The discrepancy between morphology and biological behaviour of thymomas is also mentioned with a review of the literature.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/7. Pleural lymphoma in a patient presenting with malabsorption: an illustration of the clinicopathological behaviour in a case of enteropathy associated T cell lymphoma.A case of enteropathy associated T cell lymphoma (EATCL) is described, which was diagnosed by biopsy of a lymphomatous pleural mass. Retrospective radiological review showed that this lesion had been present when an initial diagnosis of coeliac disease had been made 12 months previously and a detailed description of the natural history of the lymphoma during this period was thus available. The findings show that EATCL may behave in an indolent fashion and masquerade as coeliac disease, delaying the correct diagnosis. The relation of this disorder with coeliac disease and lymphocytic gastritis is discussed with reference to recent published works.- - - - - - - - - - ranking = 4keywords = behaviour (Clic here for more details about this article) |