1/4. Malignant peritoneal mesothelioma presenting with respiratory symptoms.Malignant peritoneal mesothelioma is a rare disease associated with mild, nonspecific abdominal symptoms and a wide spectrum of imaging findings, with thickened mesentery and peritoneum being the most common ones. A case of a malignant peritoneal mesothelioma presenting with manifestations of pulmonary disease is reported. Imaging evaluation revealed pleural, lung and pericardial involvement together with retroperitoneal lymphadenopathy, little ascites and extensive omental, but only subtle, mesenteric thickening.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. Doxil-induced regression of pleuro-pulmonary metastases in a patient with malignant meningioma.Metastatic meningioma is a rare disease, which has no effective chemotherapy. We report on a treatment of this condition with Doxil, a liposomal doxorubicin formulation. A 60-year-old woman with massive pleuro-pulmonary metastases from recurrent cranial meningioma was treated with Doxil (50-37.5 mg/m2) for 18 months with near-complete resolution of metastases and disappearance of pleural fluid. The only significant toxicities observed were stomatitis and hand-foot syndrome, which resolved with dose reduction and increase of dosing intervals. Doxil was cleared very slowly in this patient with a monoexponential half-life of 108 h. The patient remains in near-complete response for 6 months after treatment discontinuation. This is the first report on an effective chemotherapy in a patient with typical metastatic meningioma. The exact mechanism accounting for such an effective drug action is not clear, but may be related to a particularly high microvascular permeability to the liposome carriers in these metastatic lesions.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. Human herpes virus 8-negative primary effusion lymphoma (PEL) in a patient after repeated chylous ascites and chylothorax.We describe a case of malignant lymphoma which presented in the body cavities without identifiable tumor masses. Malignant lymphoma cells showed strong atypia with prominent nuclei and basophilic cytoplasm containing vacuoles. The chromosomes showed diploidy and complex abnormalities including translocations and deletions. We diagnosed this patient with primary effusion lymphoma (PEL), even though she tested negative for human herpes virus-8 (HHV-8) which has been suggested to be causally related to PEL. Interestingly, the patient also showed complicated protein-losing enteropathy, and PEL occurred after repeated chylous ascites and chylothorax. The possible pathogenesis of this rare disease is discussed here.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. Systemic mastocytosis associated with chronic myelomonocytic leukemia: clinical features and response to interferon alfa therapy.Systemic mastocytosis is a rare disease that shows marked heterogeneity in clinical manifestations and prognosis. It may be associated with hematologic disorders. We describe a patient with systemic mastocytosis associated with chronic myelomonocytic leukemia accompanied by ascites, pleural effusion, and development of skin lesions along a surgical scar. The disease responded well to interferon alfa therapy. This is the second report of successful treatment of mastocytosis with interferon alfa and the first associated with a hematologic malignancy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |