Faculty Bibliography

Patients with symptomatic Chiari malformation Type I (CM-I) typically exhibit a chronic, slowly progressive disease course with evolution of symptoms. However, some authors have reported acute neurological deterioration in the setting of CM-I and acquired Chiari malformations. Although brainstem dysfunction has been documented in patients with CM-II and hydrocephalus or shunt malfunction, to the authors' knowledge only 1 report describing ventriculoperitoneal (VP) shunt malfunction causing neurological deterioration in a patient with CM-I exists. The authors report on their experience with the treatment of previously asymptomatic CM-I in 2 children who experienced quite different manifestations of acute neurological deterioration secondary to VP shunt malfunction. Presumably, VP shunt malfunction created a positive rostral pressure gradient across a stenotic foramen magnum, resulting in tetraparesis from foramen magnum syndrome in 1 patient and acute ataxia and cranial nerve deficits from syringobulbia in the other. Although urgent shunt revisions yielded partial recovery of neurological function in both patients, marked improvement occurred only after posterior fossa decompression

Local recurrence following radical resection is one of the most common complications of pediatric craniopharyngioma. Only 28 cases of ectopic recurrence of craniopharyngioma have been reported in the literature, and only 13 cases occurred in patients originally treated as children. In this consecutive series of 86 children who underwent radical resection of primary and recurrent craniopharyngiomas, 4 patients (4.7%) experienced ectopic tumor recurrence, accounting for 27% of all recurrences after gross-total resection. The authors report on the successful surgical treatment of these 4 patients and the impact of ectopic craniopharyngioma recurrence on survival

We present a case of synchronous involvement of the pineal and suprasellar regions by a mixed germ cell tumor comprising germinoma and yolk sac tumor components, with a predominant angiomatous component. To our knowledge, it is the first case of this nature to be reported in the literature. Usually, synchronous lesions of this kind are pure germinomas, and some clinicians will forgo a biopsy and assume a germinoma histology if the serum beta-human chorionic gonadotrophin (HCG) is <50 IU/l and the alpha-fetoprotein (AFP) is within normal limits. Secondly, if a biopsy is performed on a lesion that has a prominent angiomatous component, the diagnostic germ cell tumor may be missed at the time of the biopsy. In order to alert clinicians and pathologists to this rare entity, the case is discussed with particular reference to difficulties that were encountered in rendering an accurate diagnosis, and the associated management implications

OBJECT: Given the potential morbidity of cranial irradiation in young children, the risk-benefit analysis of limited surgery plus irradiation versus radical resection may favor the latter strategy. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes of patients 5 years of age and younger who underwent radical resection of craniopharyngiomas. METHODS: Between 1991 and 2008, 19 children age < or = 5 years were diagnosed with a craniopharyngioma and underwent radical resection by the senior author (J.H.W.). Data were retrospectively collected on these 19 patients (11 males, 8 females; mean age 3.2 years) to assess the efficacy and impact of surgical treatment. RESULTS: Eighteen (95%) of 19 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. There was no operative death and 18 of (95%) 19 patients were alive at a mean follow-up of 9.4 years (median 8.3 years). Six patients (31%) had a total of 7 tumor recurrences treated by repeat GTR in 5 patients and Gamma knife surgery in 1 patient. No patient required conventional, fractionated radiation therapy. Disease control was achieved surgically in 17 (89.5%) patients and with surgery and Gamma knife surgery in 1 patient, yielding an overall rate of disease control of 95% without the use of conventional radiotherapy. New-onset diabetes insipidus occurred in 50% of patients. Vision worsened in 1 patient, and there was no long-term neurological morbidity. CONCLUSIONS: In this retrospective series, children aged < or = 5 years with craniopharyngiomas can have excellent outcomes with minimal morbidity after radical resection by an experienced surgeon. Disease control in this population can be successfully achieved with GTR alone in the majority of cases, avoiding the detrimental effects of radiotherapy in this vulnerable population

OBJECT: The purpose of this study was to assess the impact of minimal residual calcification without enhancing tumor on the rate of recurrence after gross-total resection (GTR) of craniopharyngioma in children. METHODS: Data were retrospectively collected in 86 patients younger than 21 years of age in whom 103 craniopharyngioma resections were performed by the senior author between 1986 and 2008. Forty-nine patients (27 boys and 22 girls, with a mean age of 8.6 years) fulfilled the criteria for inclusion in this study by having tumor calcification on the preoperative CT scan, undergoing GTR, and having complete postoperative CT and MR imaging and clinical follow-up. RESULTS: Thirteen patients (27%) had residual calcification (< or = 2 mm in 12 patients; 3.5 mm in 1 patient) on their postoperative CT scan. At a mean follow-up of 9.4 years (median 10 years), 2 (15%) of 13 patients with and 10 (28%) of 36 patients without residual calcification experienced tumor recurrence. There were no significant differences between these groups in terms of the duration of follow-up, time to recurrence, rate of recurrence, or recurrence-free survival. CONCLUSIONS: The absence or presence of minimal residual calcification does not have an impact on the risk of recurrence after GTR in pediatric craniopharyngiomas. The authors recommend withholding irradiation or other adjuvant therapy in the setting of minimal residual calcification without enhancing tumor. Close follow-up with frequent serial imaging in all patients after GTR is imperative to identify and treat early recurrence

We present our technique for extraoperative functional mapping in awake children with supratentorial tumors near eloquent cortex. This technique may help optimize tumor resection and/or the functional outcome, especially when an awake craniotomy is not possible or in the setting of coincident seizures. After subdural electrode implantation, extraoperative functional mapping and seizure focus mapping were performed, followed by staged tumor resection. Gross total resection was achieved in 6 patients and subtotal resection in 2. No complications due to electrode placement or invasive monitoring were observed. Staged resection of pediatric brain tumors near the eloquent cortex appears safe, allows functional mapping in awake patients, and may assist in decisions between maximal tumor resection and optimal functional outcome

PURPOSE: To determine whether pediatric patients treated with surgery only for low-grade tumors in the cerebral hemispheres, supratentorial midline, and exophytic brainstem evidence neurocognitive, academic, adaptive, or emotional/behavioral sequelae. PATIENTS AND METHODS: Ninety-three patients from a natural history study of low-grade astrocytomas were tested an average of 111 days after surgery. Rates of below average (< or = 25th percentile) scores in this sample were compared with test norms, and performances were compared across anatomic sites. Finally, the relationships of pre-, peri-, and postsurgical complications to outcome were investigated. RESULTS: For the entire sample, there was a significantly elevated rate of below average scores across intelligence quotient, achievement, and adaptive behavior, but not behavioral/emotional adjustment measures. Patients with hemispheric, midline, and brainstem tumors did not differ significantly. Patients with left hemisphere tumors generally performed worse than those with right hemisphere tumors. Finally, neurobehavioral outcome was unrelated to pre-, peri-, or postsurgery complications. CONCLUSION: After surgery for low-grade brain tumors, a significant number of patients was found to function below average, by as much as 55% compared with 25% in the normative population. Moreover, these results suggest greater risk for patients with lesions situated in the left cerebral hemisphere. Routine neuropsychological follow-up of children after treatment for low-grade tumors is recommended

OBJECTIVES: We evaluated and compared tumor antigen precursor protein (TAPP) profiles in adult and pediatric brain tumors of 31 genes related to tumor associated antigens (TAA) for possible use in immunotherapy. Antigens were selected based on their potential to stimulate T cell responses against tumors of neuroectodermal origin. METHODS: Thirty-seven brain tumor specimens from 11 adult and 26 pediatric patients were analyzed by quantitative real-time PCR for the relative expression of 31 TAPP mRNAs. The age range of adults (4F:7M) was 27-77 years (median 51.5 +/- 14.5 years) and for pediatrics (12F:14M) was 0.9-19 years (median 8.3 +/- 5.5 years). Histological diagnoses consisted of 16 glioblastomas, 4 low grade astrocytomas, 10 juvenile pilocytic astrocytomas, and 7 ependymomas. RESULTS: The adult gliomas expressed 94% (29 of 31) of the TAPP mRNAs evaluated compared with pediatric brain tumors that expressed 55-74% of the TAPP mRNAs, dependent on tumor histological subtype. Four types of TAPP expression patterns were observed: (1) equal expression among adult and pediatric cases, (2) greater expression in adult than pediatric cases, (3) expression restricted to adult GBM and (4) a random distribution. The pediatric brain tumors lacked expression of some genes associated with engendering tumor survival, such as hTert and Survivin. CONCLUSIONS: The potential TAA targets identified from the TAPP profiles of 31 genes associated with adult and pediatric brain tumors may help investigators select specific target antigens for developing dendritic cell- or peptide-based vaccines or T cell-based immunotherapeutic approaches against brain tumors