1/9. Neuro-ophthalmologic manifestations of Maffucci's syndrome and Ollier's disease.patients with Ollier's disease (multiple skeletal enchondromas) and Maffucci's syndrome (multiple enchondromas associated with subcutaneous hemangiomas) may develop skull base chondrosarcomas or low-grade astrocytomas as a delayed consequence of these disorders. We report three patients with Ollier's disease and Maffucci's syndrome who had diplopia as the initial manifestation of intracranial tumors. Since patients with Maffucci's syndrome and Ollier's disease are at risk for the delayed development of brain and systemic neoplasms, neuroophthalmologists must be aware of the need for long-term surveillance in patients affected by these conditions.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/9. Intravascular papillary endothelial hyperplasia of the central nervous system--four case reports.Four rare cases of intracranial intravascular papillary endothelial hyperplasia (IPEH) manifesting as cranial nerve disturbances occurred in 16-, 18-, 24-, and 28-year-old females. magnetic resonance imaging showed all lesions as isointense with strong enhancement on T1-weighted images, and as hyperintense on T2-weighted images. All lesions were removed via craniotomies. Histological examination found vascular structures and papillary spaces lined with endothelial cells showing immunoreactivity for CD31. Complete removal was curative in two cases, whereas incomplete removal resulted in cure in one case and residual deficits in one case. Iatrogenic deficits should be avoided in IPEH treatment by surgery. Differentiation from neoplasm such as angiosarcoma depends on histological characteristics.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/9. Primary liposarcoma of the orbit. Problems in the diagnosis and management of five cases.The clinicopathologic features and the results of diagnostic studies were reviewed in five patients with primary orbital liposarcoma. The major clinical complaints were diplopia and proptosis; visual function was typically well maintained. In three cases, computed tomographic (CT) scans displayed lesions with a central fat density rimmed by a variably radiodense pseudocapsule, leading to the erroneous clinical impression that the lesions were cystic in character. ultrasonography ruled out a truly cystic tumor by demonstrating internal acoustic interfaces. magnetic resonance imaging (MRI) in one case showed hyperintense signals in T-1-weighted images, suggesting the presence of fat within the lesional tissue. In the other two cases, CT scans showed reticulated densities accentuating the septa of the orbital fat in the absence of clear-cut circumscription. A potentially useful feature pointing to a neoplasm was bowing of an involved extraocular muscle, which is usually straightened and splinted in either Graves' disease or inflammatory pseudotumor. Histopathologically, two lesions were well-differentiated liposarcomas and three were myxoid liposarcomas, all featuring univacuolar signet ring lipoblasts. Scattered bizarre hyperchromatic mesenchymal cells without prominent lipidization were present in the lesions. None of the patients has experienced regional or distant metastases with follow-ups from onset of 1 to 7 years (mean, 5.2 years); three required orbital exenteration after local recurrence; and two who refused exenteration received only orbital radiotherapy.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/9. Metastasis within a pituitary adenoma presenting with bilateral abducens palsies: case report and review of the literature.A 56-year-old woman with metastatic breast carcinoma presented on her terminal hospital admission with diplopia secondary to bilateral abducens nerve palsies. Postmortem examination revealed a sellar mass composed of an unsuspected pituitary adenoma infiltrated by metastatic carcinoma. review of the literature revealed only seven patients with this rare neoplasm-to-neoplasm metastasis, none of whom were reported to have had abducens palsies.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
5/9. Acute acquired comitant esotropia simulating late onset accommodative esotropia.Acute acquired comitant esotropia in the older child, adolescent, and young adult may represent uncorrected accommodative esotropia. Three young patients developed spontaneous diplopia associated with acute comitant esotropia. In all cases, cycloplegic refraction revealed high uncorrected hyperopia suggesting late onset accommodative esotropia. However, for one patient an intracranial neoplasm precipitated the strabismus and for the other patients the hyperopic correction did not alter the deviation. Clinicians confronted with older children or adolescent patients with acute comitant esotropia associated with large uncorrected hyperopia should not hastily classify the deviation as being accommodative in etiology. Although the latter is possible, the hyperopia may be coincidental and masking an underlying mechanism.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/9. Immunofluorescent and immunoperoxidase characteristics of IgDlambda myeloma involving the orbit.A biopsy specimen from a 59-year-old man with unilateral proptosis revealed an orbital plasmacytic tumor. in vitro immunoperoxidase and immunofluorescence studies and direct immunoperoxidase staining of Bouin's fixed, paraffin-embedded tissue sections demonstrated that the tumor consisted of plasma cells with surface and intracellular IgDlambda immunoglobulin. Systemic evaluation demonstrated multiple myeloma. A review of previously published reports combined with the case presented here suggests that IgD myeloma has a predilection for orbital involvement. Thus, while IgD myeloma accounts for only 2% of all myelomas, it may account for a significant percentage of myelomas with orbital involvement. The distinctive clinical course of IgD myeloma, particularly its poor prognosis, warrants consideration of the immunologic and serologic findings in patients with orbital involvement by myeloma. The application of immunofluorescence and immunoperoxidase techniques to the study of orbital lymphoreticular neoplasms should result in a clearer understanding of these neoplasms.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
7/9. Remitting sixth nerve palsy in skull base tumors.OBJECTIVE: Spontaneous recovery of a sixth nerve palsy is thought to rule out a neoplastic origin. We reviewed cases of sixth nerve palsy that improved without treatment but that ultimately proved to be caused by a tumor at the base of the skull. DESIGN: Case series. SETTING: Hospital-based, neuro-ophthalmology referral practice. patients: Seven patients with an age range from 7 to 61 years had sixth nerve palsy secondary to a slow-growing neoplasm at the skull base. MAIN OUTCOME MEASURES: Return of lateral rectus function and resolution of diplopia without intervention. RESULTS: Seven patients with sixth nerve palsy caused by skull base tumors experienced spontaneous improvement of their deficit. Recovery time ranged from 1 week to 18 months. No patient was diabetic or had evidence of vascular disease. In one patient, the palsy improved once prior to becoming a fixed deficit, and spontaneous improvement occurred on two to five occasions in the other patients. CONCLUSION: Spontaneous recovery of a sixth nerve palsy can occur in the presence of an extramedullary compression by a tumor at the base of the brain. Possible mechanisms for recovery include remyelination, axonal regeneration, relief of transient compression (eg, resorption of hemorrhage), restoration of impaired blood flow, slippage of a nerve previously stretched over the tumor, or immune responses to the tumor.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/9. Antineuronal (anti-Ri) antibodies in a patient with steroid-responsive opsoclonus-myoclonus.A 45-year-old woman developed opsoclonus, myoclonus, and severe truncal and gait ataxia. serum and CSF contained IgG antibodies that appear to be identical to "anti-Ri" antibodies associated with paraneoplastic opsoclonus and ataxia. The patient had a fluctuating course with exacerbations that responded well to corticosteroids and later to cyclophosphamide. Her anti-Ri antibody titer has declined significantly but still remains high. After more than 3 years of follow-up, no neoplasm has been detected.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/9. hemangioendothelioma of the cavernous sinus: case report.OBJECTIVE AND IMPORTANCE: hemangioendothelioma is an uncommon vascular neoplasm, and its intracranial occurrence is extremely rare. The occurrence of such a tumor within the cavernous sinus has not been reported previously. CLINICAL PRESENTATION: We report a 36-year-old woman who presented with right-sided retro-orbital pain and diplopia caused by a right sixth nerve paresis. magnetic resonance imaging revealed a lesion within the right inferior cavernous sinus. INTERVENTION: The tumor was excised completely via an orbitozygomatic approach. CONCLUSION: hemangioendothelioma is a rare, indolent vascular tumor, and its characteristics are reviewed. The need for complete excision is emphasized, and the advantages of the orbitozygomatic approach for the removal of tumors of the cavernous sinus is discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |