Object The authors assessed the efficacy of intratumoral interferon-α (IFNα)–based chemotherapy in pediatric patients with cystic craniopharyngiomas. Methods 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. Results 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. Conclusions 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.

PurposeTo analyze the pathways to brainstem tumors in childhood, as well as safe entry zones.MethodWe conducted a retrospective study of 207 patients less than 18 years old who underwent brainstem tumor resection by the first author (Cavalheiro, S.) at the Neurosurgical Service and Pediatric Oncology Institute of the São Paulo Federal University from 1991 to 2011.ResultsBrainstem tumors corresponded to 9.1 % of all pediatric tumors operated in that same period. Eleven previously described “safe entry zones” were used. We describe a new safe zone located in the superior ventral pons, which we named supratrigeminal approach. The operative mortality seen in the first 2 months after surgery was 1.9 % (four patients), and the morbidity rate was 21.2 %.ConclusionsAnatomic knowledge of intrinsic and extrinsic brainstem structures, in association with a refined neurosurgical technique assisted by intraoperative monitoring, and surgical planning based on magnetic resonance imaging (MRI) and tractography have allowed for wide resection of brainstem lesions with low mortality and acceptable morbidity rates.

OBJECTIVEIn this study, the authors retrospectively reviewed two cohorts of patients with occipital encephalocele (OE) and demonstrate the technical aspects of an innovative and unprecedented method for fetal OE correction.METHODSThis was a cross-sectional retrospective study of 22 patients who underwent surgery from July 2012 to July 2018. The inclusion criteria for participants were a gestational age between 19 weeks and 27 weeks, a maternal age ≥ 18 years, a normal fetal karyotype, the presence of microcephaly, and a cystic hernial sac with the cystic component accounting for more than 80% of the total volume, which was larger than 10 ml. The fetuses with OE and microcephaly underwent intrauterine repair. The exclusion criteria were the presence of a fetal anomaly not associated with OE, chromosomopathy, presence of the brainstem inside the hernial sac or venous sinuses inside the herniated content, the risk of premature birth, placenta previa, and maternal conditions that would constitute an additional risk for fetal and maternal health, as well as refusal for fetal surgery. The authors reviewed the potential for microcephaly reversal and the long-term neurocognitive development.RESULTSThe authors report the cases of 9 fetuses with OE and microcephaly who underwent intrauterine OE repair at gestational ages of less than 27 weeks (fetal group). One case selected for fetal surgery also presented with placental abruption. All patients who underwent the fetal operation had microcephaly reversal, and 3 patients required ventriculoperitoneal shunting in the 1st year of life. The authors reviewed the cases of 12 patients who underwent postnatal repair. In 10 cases, fetal surgery was refused (postnatal group), and in 2 cases, the inclusion criteria for fetal surgery were not met. The authors evaluated the neurocognitive development of the patients with the Bayley Scales of Infant Development II. The median score for the fetal group was 98.7, and that for the postnatal group was 27.8.CONCLUSIONSThe intrauterine repair of OE may stop the progression of encephalocele sac herniation and result in microcephaly reversal. The fetal group had a better cognitive outcome than the postnatal group. The technique required to correct this defect is feasible for those with previous experience in the correction of fetal myelomeningocele. However, more studies are needed to ensure the efficacy of this procedure.

Intramedullary schwannomas unrelated with neurofibromatosis are uncommon tumors, but if correctly diagnosed and properly treated they may have a good prognosis.They have a wide range of clinical presentations, commonly presenting as a slowly progressive motor or sensory syndrome. We present a case report of a patient without neurofibromatosis with a surgically treated cervical intramedullary schwannoma.

Objective: To assess whether the cystic craniopharyngiomas can be controlled with the use of intratumoral applications of interferon alpha. Method: Nineteen patients with the diagnosis of cystic craniopharyngioma were treated with intratumoral chemotherapy with interferon alpha from January 2002 to April 2006. All patients underwent placement of an intracystic catheter connected to an Ommaya reservoir. Through this reservoir were made applications during chemotherapy cycles. Each cycle corresponded to application of 3,000,000 units of interferon alpha three times per week on alternate days totalizing 36,000,000 units. Response to treatment was evaluated by calculating the tumor volume on MRI control after one, three and six months after the end of each cycle. Patients who developed worsening of symptoms or who had insignificant reduction in tumor volume during follow-up underwent repeat cycle chemotherapy. Results: Four patients received four cycles of chemotherapy, three patients received three cycles, six patients received two cycles and six patients received one. The lower percentage of reduction in tumor volume was 60% and the bigger reduction was 98.37%. Eleven patients had a reduction greater than 90%. Five patients had a tumor reduction between 75 and 90% and in three patients the tumors were reduced by less than 75%. No deaths occurred during treatment and side effects of interferon alpha were well tolerated. No treatment was discontinued. Follow-up after the last application ranged from one year and five months to three years and nine months. Conclusion: The intratumoral chemotherapy with interferon alpha decreases the volume of cystic craniopharyngiomas and so far can be considered a new therapeutic alternative.