1/3. Mediastinal large-cell lymphoma of B-type, with sclerosis: histopathological and immunohistochemical study of eight cases.Eight cases of mediastinal non-lymphoblastic large-cell lymphoma have been studied by histopathological and immunohistochemical methods. A common clinical, morphological and immunophenotypic pattern was identified. Six of eight cases proved to be of B-cell origin by the expression of B-associated antigens detected by specific monoclonal antibodies. cells of large size with nuclei of varying morphology and a peculiar type of fine compartmentalizing fibrosis were observed in all specimens. Clinically the disease was characterized by the young age of the patients, primary mediastinal involvement, aggressive behaviour and spread to unusual sites (kidneys in four cases). This seems to be a hitherto unrecognized entity in the field of non-Hodgkin's lymphomas, often misdiagnosed because of location and a morphology uncommon for B-cell malignancies. Immunohistochemical analysis on frozen tissue sections appears to be mandatory for a correct diagnosis. Nevertheless, this type of lymphoma could be suspected also on the basis of its peculiar clinicopathological characteristics.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/3. fibrosarcoma in children--a rare tumour with long-term survival even with advanced disease--a report of 3 cases.fibrosarcoma is a rare tumour in children. The potential of malignancy has been questioned. We present three cases of fibrosarcoma in children . The follow-up periods range from 10 to 37 years. The first patient had pulmonary metastases at the time of diagnosis in 1958. The primary tumour in fossa ischio-rectalis was resected in 1960. lung metastases were resected in 1960 and 1989. radiotherapy was given in 1992. He is still alive with metastases 37 years after the first manifestation of disease. The second patient had a primary tumour and several local recurrences in the mandible. He is alive without evidence of disease 4 years after resection of pulmonary metastases and 21 years after resection of the primary tumour. The third patient has no signs of recurrence or metastatic spread 10 years after a wide excision of subcutaneous tumours of the left upper arm. The cases demonstrate a special tumour-entity of low-grade malignancy, which show a good prognosis and a wide spectrum of biological behaviour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/3. Primary alveolar soft-part sarcoma of the mediastinum: a clinicopathological and immunohistochemical study of two cases.AIMS: Primary alveolar soft part sarcomas originating within the thoracic cavity are rare. The cases herein described highlight the ubiquitous distribution of this neoplasm and the importance of considering this tumour in the differential diagnosis of mediastinal tumours. methods AND RESULTS: Two cases of alveolar soft-part sarcoma of the mediastinum are presented. The patients are two men of 22 and 23 years of age. Clinically, the patients presented with symptoms of chest pain. One tumour was in the anterior mediastinum while the second tumour was in the posterior mediastinum. Even though the bulk of the tumours were in mediastinal locations, both patients had pulmonary metastases at the time of diagnosis. Histologically, both tumours showed the classic morphology of alveolar soft part sarcoma, i.e. a proliferation of large polygonal cells with round to oval nuclei, prominent nucleoli and moderate amounts of eosinophilic cytoplasm arranged in a prominent alveolar pattern. periodic acid-Schiff stains showed the characteristic diastase-resistant intracytoplasmic crystals. Immunohistochemical studies showed focal myoglobin reactivity in one case, while cytokeratin, vimentin, S100 protein, chromogranin, synaptophysin, neurofilaments, leu-enkephalin, desmin, smooth muscle actin and muscle-specific actin were negative in both cases. The patient with the anterior mediastinal tumour died of disease 14 months after diagnosis, and the patient with the posterior mediastinal mass remained well for at least 15 years and was later lost to follow-up. CONCLUSIONS: As has been observed in other anatomic areas, namely soft tissues, alveolar soft part sarcomas may follow an uncertain natural history. Interestingly, in our cases, the tumour in the anterior mediastinum followed a fatal course raising the possibility that the anatomic location of the tumour may have play a role in the behaviour of the tumour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |