✓ The authors present a case of a gigantic cystic craniopharyngioma that was treated with intratumoral injections of bleomycin. The mass had eroded the skull base and extended to the sphenoid bone. A total of eight intratumoral injections through an Ommaya reservoir were given. Six months after treatment, there was complete regression of the lesion and improvement in both visual and endocrinological symptomatology.
Sergio Cavalheiro, Fábio Veiga de Castro Sparapani, José Orlando Bidó Franco, Marcia Cristina da Silva and Fernando Menezes Braga
Sergio Cavalheiro, Fábio Veiga de Castro Sparapani, Antonio Fernandes Moron, Marcia Cristina da Silva and João Norberto Stávale
✓ 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.
Rodrigo Panico Gorayeb, Sergio Cavalheiro and Samuel Tau Zymberg
Object. The authors analyzed data obtained in 36 patients younger than 1 year of age who were treated with endoscopic third ventriculostomy (ETV) for obstructive hydrocephalus at their institution.
Methods. There were 17 boys and 19 girls who ranged in age from 3 days to 11 months (mean 4.7 months). The causes of the hydrocephalus were Chiari Type II malformation (11 cases), aqueductal stenosis (11 cases), and other (14 cases). The success rate was 64% (p < 0.05, confidence interval 0.48–0.8) and there were four complications, mainly meningitis. The follow-up period ranged from 22 to 69 months (mean 47.4 months).
Conclusions. Based on the 64% success rate in children younger than 1 year of age, ETV should be the treatment of choice for obstructive hydrocephalus in this age group, although larger studies involving specific causes of hydrocephalus are needed.
Maisa Yoshimoto, Sílvia Regina Caminada de Toledo, Nasjla Saba da Silva, Jane Bayani, Ana Paula Antunes Pascalicchio Bertozzi, Joã Norberto Stavale, Sergio Cavalheiro, Joyce Anderson Duffles Andrade, Maria Zielenska and Jeremy A. Squire
Object. The purpose of this study was to examine chromosomal gains and losses in 11 pediatric adamantinomatous craniopharyngiomas by using comparative genomic hybridization (CGH), as well as to review the cytogenetic literature that has contributed to the characterization of these tumors. One source of confusion in the cytogenetic and CGH literature concerning craniopharyngioma is that the authors of most studies fail to distinguish between pediatric and later-onset forms of the disease. Thus, this study was focused on pediatric craniopharyngioma.
Methods. To determine an overview of the genetic events leading to the development of these tumors, 10 adamantinomatous craniopharyngiomas were analyzed using CGH; none of the tumor specimens demonstrated gains or losses of DNA sequence.
Conclusions. In view of these findings as well as those published in the majority of previous cytogenetic studies of craniopharyngiomas, the authors conclude that the recurrent acquisition of chromosomal imbalances does not play a major role in tumorigenesis and that chromosomal gains and losses are a relatively rare event in primary tumors of pediatric origin.
Sergio Cavalheiro, Concezzio Di Rocco, Sergio Valenzuela, Patricia A. Dastoli, Gianpiero Tamburrini, Lucca Massimi, Jardel M. Nicacio, Igor V. Faquini, Daniela F. Ierardi, Nasjla S. Silva, Benedetta Ludovica Pettorini and Silvia R. C. Toledo
The authors assessed the efficacy of intratumoral interferon-α (IFNα)–based chemotherapy in pediatric patients with cystic craniopharyngiomas.
In a prospective multicenter study of 60 pediatric patients, the authors assessed the efficacy of intratumoral INFα2A-based chemotherapy. The study was conducted between 2000 and 2009 at 3 locations: the Medical School of the Federal University of São Paulo, Catholic University of Rome, and the Neurosurgery Institute of Santiago, Chile. The assessment included clinical and radiological control examinations, side effects observed, and total dose used.
Sixty cases of cystic craniopharyngioma were analyzed. The cohort consisted of 35 male and 25 female children (mean age 11 years). Clinical and radiological improvement was achieved in 76% of the cases. New endocrinological deficits were observed in 13% of the cases. In approximately 30% of the patients, the evolution included some light side effects, the most common being headache (33%) and eyelid edema (28%). The number of cycles varied from 1 to 9 (mean 5 cycles), and the total dose applied per cycle was 36,000,000 IU.
This has been the largest documented series of intratumoral chemotherapy using INFα for the control of cystic craniopharyngiomas. The treatment has proved efficacious; there was no mortality, and morbidity rates were low.
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.
Francisco Vaz Guimarães Filho, Giselle Coelho, Sergio Cavalheiro, Marcos Lyra and Samuel T. Zymberg
Ideal surgical training models should be entirely reliable, atoxic, easy to handle, and, if possible, low cost. All available models have their advantages and disadvantages. The choice of one or another will depend on the type of surgery to be performed. The authors created an anatomical model called the S.I.M.O.N.T. (Sinus Model Oto-Rhino Neuro Trainer) Neurosurgical Endotrainer, which can provide reliable neuroendoscopic training. The aim in the present study was to assess both the quality of the model and the development of surgical skills by trainees.
The S.I.M.O.N.T. is built of a synthetic thermoretractable, thermosensible rubber called Neoderma, which, combined with different polymers, produces more than 30 different formulas. Quality assessment of the model was based on qualitative and quantitative data obtained from training sessions with 9 experienced and 13 inexperienced neurosurgeons. The techniques used for evaluation were face validation, retest and interrater reliability, and construct validation.
The experts considered the S.I.M.O.N.T. capable of reproducing surgical situations as if they were real and presenting great similarity with the human brain. Surgical results of serial training showed that the model could be considered precise. Finally, development and improvement in surgical skills by the trainees were observed and considered relevant to further training. It was also observed that the probability of any single error was dramatically decreased after each training session, with a mean reduction of 41.65% (range 38.7%–45.6%).
Neuroendoscopic training has some specific requirements. A unique set of instruments is required, as is a model that can resemble real-life situations. The S.I.M.O.N.T. is a new alternative model specially designed for this purpose. Validation techniques followed by precision assessments attested to the model's feasibility.
Shlomi Constantini, Aaron Mohanty, Samuel Zymberg, Sergio Cavalheiro, Conor Mallucci, Dieter Hellwig, Yusuf Ersahin, Hiroshi Mori, Carmelo Mascari, José Aloysio Costa Val, Wolfgang Wagner, Abhaya V. Kulkarni, Spyros Sgouros and Shizuo Oi
Analysis of the safety and morbidity of neuroendoscopic biopsies (NEBs), as well as the reliability in obtaining an accurate diagnosis, has until now been based on studies with relatively small sample sizes. Through the cooperative efforts of several international medical centers, authors of the present study collected data on a large number of patients to obtain better insight into this issue. When possible, they compared pathology obtained through an NEB with the “gold-standard” pathology obtained in open surgery.
Thirteen randomly chosen medical centers in 9 countries collected data for patients who had undergone NEB, which were then analyzed for universal complications, bleeding, navigation technique, pathology, mismatch between biopsy results and final diagnosis, and a number of other potentially influential factors.
Data for 293 patients were analyzed. Sixty percent of the patients were male, and patient ages ranged from 0.1 to 78.7 years (median age 20.4 years). The most common tumor locations were pineal (33.1%), thalamic (16.7%), tectal (13%), and hypothalamic (4.4%). Fifty percent of the tumors were larger than 20 mm, 36% were between 10 and 20 mm, and 14% were smaller than 10 mm. Intraoperative bleeding was seen in 275 patients (94%). The amount of blood was noted as mild in 75%, moderate in 13%, and severe in 6%. Infection occurred in 8 patients (3%). Death occurred in 1 patient (0.3%), which was caused by severe intraoperative bleeding. Biopsies were informative in 265 patients (90.4%). Seventy-eight patients (26.6%) had open surgery following the NEB. For these patients, the pathology results from the NEB were compared with those from the open surgery that followed. In 14 cases (17.9%) there was disagreement on the pathology. Of these cases, a meaningful mismatch, in which the erroneous NEB pathology could have led to an inappropriate management decision, occurred in 9 cases (11.5%). Most of these meaningful mismatches were lesions diagnosed as low-grade or pilocytic astrocytoma on the NEB and later proved to be high-grade astrocytoma (4 cases) and 1 case each of meningioma, cavernoma, primitive neuroectodermal tumor, neurocysticercosis, and pineocytoma.
In experienced hands, NEBs can be performed with low morbidity and mortality, providing meaningful pathological data for the majority of patients with a wide range of tumor types, locations, and presentations. These biopsies also offer other advantages, such as the ability to perform concomitant endoscopic third ventriculostomy and septum pellucidotomy. However, due caution must be maintained, since pathology obtained from an NEB, as with stereotactic biopsies, may be subject to sampling errors, especially when the results seem to indicate a low-grade glial tumor.
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.