✓ The authors present the case of a 34-week-old fetus with a meningeal hemangiopericytoma that was diagnosed in utero by using abdominal magnetic resonance imaging. After birth, the neonate underwent transfontanelle ultrasonography and computerized tomography scanning of the head, which confirmed the presence of an extradural hyperdense lesion. Six hours after birth the neonate underwent a craniotomy, which resulted in complete resection of the mass. The postoperative period was uneventful and the newborn was discharged 7 days later. At 2-year follow-up examination there was no evidence of recurrence of the lesion. The authors have found no mention in the literature of this entity diagnosed in the prenatal period.
Sergio Cavalheiro, Fábio Veiga de Castro Sparapani, Antonio Fernandes Moron, Marcia Cristina da Silva and João Norberto Stávale
Eduardo Augusto Iunes, João Norberto Stávale, Rita de Cássia Caldas Pessoa, Ricardo Ansai, Franz Jooji Onishi, Manoel Antonio de Paiva Neto, Antônio de Pádua Bonatelli, Sérgio Cavalheiro and Suzana M. Fleury Malheiros
In this paper, the authors present the case of a patient with multifocal intradural extramedullary ependymoma, and they review 18 previously reported cases.
A 32-year-old man presented to the authors' institution with a 1-month history of partial medullary syndrome. Magnetic resonance imaging of the neuraxis revealed multifocal intradural extramedullary lesions at the bulbomedullary junction and C2–3, T5–11, L-2, L-4, L-5, and sacrum. Histological examination revealed a WHO Grade II ependymoma.
The literature survey yielded 18 cases of ependymoma at the same location; none of them were multifocal at presentation. The authors analyzed the epidemiological, clinical, and surgical features of all 19 cases reported to date, including the present case.
Patients' ages ranged from 24 to 69 years; 15 patients were women and 4 were men. The time elapsed from symptom onset to diagnosis ranged from 1 month to 8 years. Pain (in 13 patients) and medullary syndrome (in 12) were reported as the initial symptoms (information was not provided for 1 patient). Tumors were predominantly located in the thoracic spine (11), but they also occurred in the cervicothoracic (3), cervical (2), and lumbar (2) spine. The remaining tumor was multifocal. Solitary extramedullary tumors were found intraoperatively in 13 patients; 3 were described as exophytic and 3 as extramedullary with some degree of medullary invasion. Histological examination revealed 9 WHO Grade II tumors, 4 Grade III tumors, and 1 myxopapillary tumor. Data obtained for the remaining cases proved inconclusive. The clinical condition improved in 11 patients, remained stable in 2, and worsened (recurrence or progression) in 6. Of the 4 patients with Grade II tumors who presented with recurrence or neuraxis spreading, 3 had meningeal infiltration or adhesion to the pia mater, which does not rule out the possibility of neoplastic remnants in that area.
Intradural extramedullary ependymomas are rare, they predominate in women in the 5th decade of life, and pain is the most frequent initial symptom. The extent of resection and the presence of meningeal infiltration seem to be key determinants of prognosis. The present case is the first intradural extramedullary ependymoma (with the exception of those occurring at the conus medullaris and terminal filum) with multiple lesions at presentation.
Marcos Devanir Silva da Costa, Daniel de Araujo Paz, Thiago Pereira Rodrigues, Ana Camila de Castro Gandolfi, Fabricio Correa Lamis, João Norberto Stavale, Italo Capraro Suriano, Luiz Daniel Marques Neves Cetl and Sergio Cavalheiro
Spinal angiolipomas are rare benign tumors that generally induce slow progressive cord compression. Here, the authors describe a case of sudden-onset palsy of the lower extremities caused by hemorrhagic spinal angiolipoma. An emergent laminectomy was performed to achieve total lesion removal. Follow-up examinations indicated neurological improvement and the absence of recurrence.
Sergio Cavalheiro, María Laura Canullo, Marcos Devanir Silva da Costa, Patricia Alessandra Dastoli, Jardel Mendonça Nicácio and João Norberto Stavale
Glioependymal cysts are a rare, congenital, and benign condition. The authors report a case of a 2-year-old female patient who presented with a large exophytic glioependymal cyst located in the left cerebral peduncle, which began with a history of deteriorating function of the left third cranial nerve (CN III). A left 3-piece fronto-orbitozygomatic approach and a microsurgical complete excision of the cyst were performed without complications by accessing the midbrain through the perioculomotor entry zone. The patient’s CN III function completely recovered after 3 months. Because there are currently no widely approved and used classification systems for these cysts, their reporting, naming, and classification are quite confusing in the literature. Thus, their origin, classification, and most common locations warrant further investigation. In addition to reporting this case, the authors present their review of the literature.