Cornelia S. von Koch

Central neurocytoma: a review

Central neurocytomas are rare intraventricular neoplasms of the central nervous system, compromising 0.25–0.5% of brain tumors. The diagnosis and management of these tumors remains controversial since most clinical series are small. Typically, patients with central neurocytomas have a favorable prognosis, but in some cases the clinical course is more aggressive. Although histological features of anaplasia do not predict biologic behavior, proliferation markers including MIB-1 might be more useful in predicting relapse. The most important therapeutic modality is surgery, and a safe maximal resection confers the best long-term outcome. In cases of a subtotal resection, standard external beam radiation can be added or radiation can be delayed until tumor progression occurs. Smaller residual tumor volumes or recurrences can be treated with more conformal radiation or focused radiosurgery. Re-operation for recurrence should be considered if the procedure can be safely performed. Chemotherapy may be useful for recurrent central neurocytomas that cannot be resected and have been radiated, although long-term responses have not been reported for chemotherapy. Overall, this paper reviews the findings of the larger studies and highlights some of the important case reports that contribute to the current management of central neurocytomas.

Clinical Outcome in Children Undergoing Tethered Cord Release Utilizing Intraoperative Neurophysiological Monitoring

Release of tethered spinal cord by sectioning of the filum terminale carries a risk of injuring neighboring motor and sensory nerve roots involved in bowel and bladder control. Therefore, intraoperative neurophysiological monitoring techniques have been developed to prevent neurological complications postoperatively. We performed a retrospective chart review of 63 patients who had undergone tethered cord release. We excluded adult patients, those lost to follow-up and patients with either a myelomeningocele and/or lipoma. This limited our study to 25 pediatric patients, aged 4 months to 12 years, who underwent tethered cord release for either a thickened filum terminale and/or a low-lying conus. For intraoperative monitoring, we utilized electrical stimulation of the filum terminale, lumbosacral nerve roots and electromyography recordings. Ventral nerve roots were identified and their electrical thresholds obtained. The mean was 0.32 V, the mode 0.1 V and the range 0.05– 1.0 V. These values were compared to electrical thresholds obtained by stimulation of the filum terminale. The mean was 26.1 V, the mode 20.0 V and the range 8–100 V. In over 70% of patients, muscle activation via the filum required 100 times the voltage needed to activate a motor root. This motor root to filum threshold of 1:100 was useful in identifying the filum. Clinical outcome showed no significant worsening with respect to bowel and bladder control or pain and motor indices. Significant bowel and bladder improvement was seen in 4 out of 25 patients, motor improvement in 9 out of 25 patients and improvement of pain in 4 out of 25 patients. Three patients developed postoperative urinary tract infections, but no cerebrospinal fluid leaks or pseudomeningoceles were encountered. These results suggest that patients with a thickened filum or low-lying conus can safely undergo tethered cord release. Intraoperative neurophysiological monitoring provides a helpful adjunct to distinguish nerve roots from the filum. A ratio, rather than an absolute number, is beneficial in distinguishing motor roots from the filum and eliminates variability due to patients’ individual differences in electrical thresholds.

Fetal Magnetic Resonance Imaging Enhances Detection of Spinal Cord Anomalies in Patients With Sonographically Detected Bony Anomalies of the Spine

Although fetal magnetic resonance imaging (MRI) is being increasingly used to evaluate sonographically suspected abnormalities, its utility in the evaluation of the spinal canal is not well studied. Because it is not susceptible to the limitations of fetal position, oligohydramnios, and shadowing from bony structures, we hypothesize that fetal MRI is better suited to assess the contents of the spinal canal compared with prenatal sonography. The purpose of this investigation was to determine whether fetal MRI could detect spinal abnormalities in cases in which they had not been originally suspected on prenatal sonography.

The role of PCV chemotherapy in the treatment of central neurocytoma: illustration of a case and review of the literature.

BACKGROUND Most central neurocytomas follow a benign clinical course. However, more aggressive variants have been described requiring additional surgical resection, radiation, or chemotherapy. Chemotherapy has rarely been used as an adjuvant therapy for central neurocytomas. METHODS We report a case of a 20-year-old girl who underwent four subtotal resections, over the course of 3 years, for a large central neurocytoma that continued to progress. She was not a candidate for stereotactic radiosurgery, given the large tumor size. To avoid radiation injury in a young patient, she was treated with six cycles of chemotherapy including procarbazine, CCNU, and vincristine. Procarbazine was stopped after 2 cycles because of the development of a rash. Serial magnetic resonance imaging was used to follow treatment response. RESULTS Her tumor started to decrease in size after 2 cycles of chemotherapy and continued to shrink until it stabilized after 5 cycles of chemotherapy. A small area of residual tumor with minimal enhancement persisted along the left lateral ventricle and remained stable for at least 16 months after the completion of chemotherapy. CONCLUSIONS To our knowledge, this is only the fourth report describing the use of chemotherapy for progression of central neurocytomas as a treatment alternative to radiation therapy. The use of procarbazine, CCNU, and vincristine has not been previously described for the treatment of a central neurocytoma and presents an additional treatment option.

In utero surgery for hydrocephalus

IntroductionNeonatal hydrocephalus is one of the most common congenital anomalies affecting the nervous system.Discussion Currently, ultrasonography allows for early detection of fetal ventriculomegaly and presents the family with several treatment options: termination of pregnancy, early delivery and neonatal shunting, and delivery at term followed by shunting. Despite ventricular decompression after birth, the cognitive outcome is variable as prolonged in utero hydrocephalus has a detrimental effect. In the early 1980s, fetal intervention was explored with the intention of improving outcome. However, patient selection was poor. Fetal ventriculomegaly from other conditions was not adequately distinguished from fetal hydrocephalus. In addition, fetal surgical techniques were not advanced. Consequently, the results were poor and a de facto moratorium on fetal shunting was imposed. However, recent improvements in fetal imaging, such as magnetic resonance imaging, and advances in fetal surgical techniques offer the possibility that properly selected fetuses with hydrocephalus can benefit from an in utero intervention.

Selective Posterior Rhizotomy and Intrathecal Baclofen for the Treatment of Spasticity

Spasticity occurs in children and adults due to a wide range of conditions, including cerebral palsy, head and spinal cord trauma, cerebrovascular accidents and multiple sclerosis. Multiple treatment options have been described, including medical and surgical treatments. Medical treatments include intramuscular botulinum A toxin, oral baclofen and supportive bracing. Surgical approaches include selective posterior rhizotomy, intrathecal baclofen and orthopedic procedures to address deformities. Many reports have been published on these different treatment options, but rarely has a comparison been made between them. Therefore, this review is aimed at comparing selective posterior rhizotomy and intrathecal baclofen injection for spasticity due to cerebral palsy, especially in children.

Familial medulloblastoma: Case report of one family and review of the literature

OBJECTIVE AND IMPORTANCE Medulloblastoma is the most common malignant brain tumor and the most common malignant solid tumor in children. Most medulloblastomas are sporadic, but rare familial forms have been described. To the best of our knowledge, only 10 case reports of familial medulloblastoma have been published. A variety of candidate genes have been suggested to be involved in familial medulloblastomas. However, the exact pathogenesis and genetics involved in familial medulloblastoma remain unknown. CLINICAL PRESENTATION We describe the presentation of medulloblastoma in two siblings (one of each sex) and their great-uncle. The three cases differ with regard to age at onset and pathological subtype of medulloblastoma. INTERVENTION OR TECHNIQUE Immunostaining of tissue blocks for gene products involved in medulloblastoma differed in the two siblings for &bgr;-catenin and was similar with staining for gli. CONCLUSION This article is only the second report in the literature to address the genetics of familial medulloblastoma in the absence of characterized conditions such as Li-Fraumeni’s cancer syndrome and basal cell nevus, Rubinstein-Taybi’s, and Turcot’s syndromes. The discrepancy in &bgr;-catenin staining in the two siblings suggests that the two tumors differentiated through divergent pathways. We briefly summarize all published cases of familial medulloblastoma and review the literature on the genes involved in medulloblastoma formation.