1/7. Intracranial Castleman's disease of solitary form. Case report.This 62-year-old woman presented with clumsiness in her right hand. magnetic resonance imaging demonstrated a small lesion mimicking a meningioma, which had arisen from the tentorium and contained notable edema. Full recovery was achieved by total removal of the lesion, which was diagnosed as a lymphoid mass resembling giant lymph node hyperplasia on histological examination. The lack of notable findings on whole-body and laboratory studies was compatible with a rare case of intracranial Castleman's disease of solitary form. The authors document clinical, neuroradiological, and pathological features of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. MR imaging features of clear-cell meningioma with diffuse leptomeningeal seeding.Clear-cell meningioma is a rare disease entity showing a more aggressive nature, clinically, than those of other subtypes of meningioma. It occurs in younger persons and commonly in the spinal canal. The recurrence rate has been reported to be as high as 60%. We present a case of clear-cell meningioma in a 17-year-old man in whom initial MR imaging showed localized leptomeningeal enhancement that had progressed into the entire subarachnoid space after surgical resection of the primary tumor.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Primary leptomeningeal lymphoma with long-term survival: a case report.Primary leptomeningeal lymphoma (PLML) is a rare disease. The most common presentation is symptoms of increased intracranial pressure. confusion, dysarthria, hearing loss, paraparesis and lumbosacral spinal root symptoms have also been reported. Chemotherapy and radiotherapy have been tried, but its prognosis is usually poor. We experienced a case of PLML with a relatively benign course in an 18-year-old girl. Initial diagnosis was made as idiopathic intracranial hypertension. Lumbosacral shunt was done with good response for 3 years. When headache recurred, she was reevaluated and was correctly diagnosed as PLML.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. Successful treatment of extracranially metastasized pineal gland germinoma with high-dose methotrexate.germinoma of the pineal gland is a rare disease usually confined to the brain which responds well to radiotherapy. Spinal seeding occurs in approximately 4% of cases and distant metastases are extremely rare. We report on a 27-year-old female with an intracranially metastasized pineal gland germinoma, meningeal carcinomatosis and distant bone metastases. Treatment was initiated with intrathecal methotrexate (MTX) and continued with high-dose intravenous MTX. The therapy was very well tolerated apart from reversible hepatic toxicity requiring a dose reduction. The patient was in complete remission after three courses followed by two consolidation cycles; the patient has now been in continuous complete remission for more than 22 months. This is the first report to show that MTX is a potent drug in treating pineal gland germinoma. Long-term side effects of radiotherapy such as reduced mental function or hypopituitarism can probably be avoided. Single-agent high-dose MTX may provide high efficacy with limited adverse effects, especially at a more advanced tumor stage with spinal seeding and extracranial disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. 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) |
6/7. Isolated meningeal tuberculoma mimicking meningioma: case report.A 47-year-old German female patient is described with intracranial tuberculoma of the dura mater without history or evidence of tuberculosis and a negative tuberculin reaction. Computed tomography and magnetic resonance imaging and angiography resembled a meningioma. diagnosis was verified by histology and identification of mycobacterium tuberculosis. After removal of the granuloma and high-dose chemotherapy, the patient was free of disease. tuberculoma of the dura mater is an extremely rare disease in the Western European population.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Cerebral aspergillosis in immunologically competent patients.aspergillosis of the central nervous system is a rare disease, especially if the patient's immune system is not compromised. The authors report three cases of cerebral aspergillosis in the immunocompetent state: a rhinocerebral form in a diabetic patient, a direct extension from chronic aspergillus otitis media, and a postoperative aspergillus brain abscess after brain tumor surgery. In spite of the poor prognosis of cerebral aspergillosis, two of the patients survived. The pathogenesis, predisposing factors, radiologic findings including magnetic resonance image, and the outcome are presented. The pertinent literature of cerebral aspergillosis is also reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |