Signet-ring cell ependymoma: case report with implications for pathogenesis and differential diagnosis.

We describe light microscopic, immunohistochemical and ultrastructural features of a signet-ring cell ependymoma (WHO grade II) identified in a surgically resected left cerebellar cystic tumor from a 64-year-old man. Part of the tumor showed clear-cell differentiation. Immunohistochemical coexpression of glial fibrillary acidic protein and epithelial membrane antigen, characteristic of ependymoma, was detected in both components. Sinuous intermediate junctions, cytoplasmic lumina, and scant astroglial filaments were demonstrated by electron microscopy. Signet-ring cell change was shown to be induced by disproportionate cavitation of either microvillus-bearing cytoplasmic lumina or microrosettes. The staining qualities of clear cells were mainly due to paucity and degeneration of subcellular organelles. Therefore, signet-ring cell ependymomas represent a unique anomaly of intra- and extracellular compartmentalization to be distinguished from various unrelated forms of cytoplasmic volume increase, resulting in an optically similar "empty" appearance of tumor cells. As a clinically relevant consequence, signet-ring cell ependymoma must be included in the differential diagnosis of primary or metastatic neoplasms of the central nervous system, having in common a phenotype characterized by overdeveloped optically lucent cell bodies.

[Rhabdoid meningioma: a potentially aggressive new variant]. / Rhabdoid meningeoma: potenciálisan agresszív új variáns.

Rhabdoid meningioma is a recently recognized clinicopathologic entity characterized histologically by cytoplasmic aggregates of intermediate filaments, and clinically by the propensity of such tumors to pursue an aggressive course. The authors report on clinical, radiologic and pathologic findings in three cases of rhabdoid meningioma identified in a retrospective surgical series of 204 meningothelial tumors. Patients included two females, aged 39 and 55 years, and a 54-year-old male. In the first two cases the tumors were located on the right and left lesser sphenoid wing, respectively; in the third case, the right cerebellopontine angle was affected. All three neoplasms evolved on a background on transitional meningioma and were conspicuous for dis-cohesive tumor cells and suppression of syncytical architecture. Immunohistochemistry and ultrastructural examination confirmed the meningothelial origin of inclusion-bearing rhabdoid cells. Although none of the tumors showed evidence of histologic anaplasia and Ki-67 labeling indices remained inferior to 2%, infiltrative growth into adjacent brain was noted in all three cases. On follow-up ranging from 8 months to 6 years, the patients remained either disease-free or alive with nonprogressive residual tumor. On account of their clinical behavior, well-differentiated rhabdoid meningiomas will be accommodated in the category of atypical meningiomas (WHO grade II). Their pathogenesis is likely to involve disrupted cytoskeletal integration of cell motility and proliferation, of which the rhabdoid phenotype may possibly represent a morphologic correlate.

Comparison of clinical symptoms and magnetic resonance angiographic (MRA) results in patients with trigeminal neuralgia and persistent idiopathic facial pain. Medium-term outcome after microvascular decompression of cases with positive MRA findings.

Neurovascular compression (NC) seems to have been confirmed as the major cause of classical trigeminal neuralgia (TN). In spite of the large number of surgically positive cases, however, there are still cases where no vascular compression of the trigeminal nerve can be found. To evaluate whether NC could be demonstrated preoperatively, high-resolution magnetic resonance angiography (MRA) was performed in 287 consecutive patients with TN and persistent idiopathic facial pain (PIFP) on a 0.5-T and a 1-T MR unit. Depending on the clinical symptoms, the TN cases were divided into typical TN and trigeminal neuralgia with non-neuralgic interparoxysmal pain (TNWIP) groups. Microvascular decompression (MVD) was performed in 103 of the MRA-positive cases. The patients were followed up postoperatively for from 1 to 10 years. The clinical symptoms were compared with the imaging results. The value of MRA was assessed on the basis of the clinical symptoms and surgical findings. The outcome of MVD was graded as excellent, good or poor. The clinical symptoms were compared with the type of vascular compression and the outcome of MVD. The MRA image was positive in 161 (56%) of the 287 cases. There were significant differences between the clinical groups: 66.5% of the typical TN group, 47.5% of the TNWIP group and 3.4% of the PIFP group were positive. The quality of the MR unit significantly determined the ratio of positive/negative MRA results. The surgical findings corresponded with the MRA images. Six patients from the MRA-negative group were operated on for selective rhizotomy and no NC was found. Venous compression of the trigeminal nerve was observed in a significantly higher proportion in the background of TNWIP than in that of typical TN on MRA imaging (24.1% and 0.8%, respectively) and also during MVD (31.2% and 1.2%, respectively). Four years following the MVD, 69% of the patients gave an excellent, 23% a good and 8% a poor result. The rate of some kind of recurrence of pain was 20% in the typical TN and 44% in TNWIP group. The rate of recurrence was 57% when pure venous compression was present. The only patient who was operated on from the PIFP group did not react to the MVD. The clinical symptoms and preoperative MRA performed by at least a 1-T MR unit furnish considerable information, which can play a role in the planning of the treatment of TN.