Evaluation of the Perioperative and Postoperative Course of Surgery for Pineal Germinoma in the SIOP CNS GCT 96 Trial.

Background: CNS germinoma, being marker-negative, are mainly diagnosed by histological examination. These tumors predominantly appear in the suprasellar and/or pineal region. In contrast to the suprasellar region, where biopsy is the standard procedure in case of a suspected germ-cell tumor to avoid mutilation to the endocrine structures, pineal tumors are more accessible to primary resection. We evaluated the perioperative course of patients with pineal germinoma who were diagnosed by primary biopsy or resection in the SIOP CNS GCT 96 trial. Methods: Overall, 235 patients had germinoma, with pineal localization in 113. The relationship between initial symptoms, tumor size, and postoperative complications was analyzed. Results: Of 111 evaluable patients, initial symptoms were headache (n = 98), hydrocephalus (n = 93), double vision (n = 62), Parinaud syndrome (n = 57), and papilledema (n = 44). There was no significant relationship between tumor size and primary symptoms. A total of 57 patients underwent primary resection and 54 underwent biopsy. Postoperative complications were reported in 43.2% of patients after resection and in 11.4% after biopsy (p < 0.008). Biopsy was significantly more commonly performed on larger tumors (p= 0.002). Conclusions: These results support the practice of biopsy over resection for histological confirmation of pineal germinoma.

Predictors of postoperative long-term seizure outcome in pediatric patients with focal cortical dysplasia type II at a German tertiary epilepsy center.

OBJECTIVE: Focal cortical dysplasia (FCD) is a common cause of early-onset intractable epilepsy, and resection is a highly sufficient treatment option. In this study, the authors aimed to provide a retrospective analysis of pre- and postoperative factors and their impact on postoperative long-term seizure outcome. METHODS: The postoperative seizure outcomes of 50 patients with a mean age of 8 ± 4.49 years and histologically proven FCD type II were retrospectively analyzed. Furthermore, pre- and postoperative predictors of long-term seizure freedom were assessed. The seizure outcome was evaluated based on the International League Against Epilepsy (ILAE) classification. RESULTS: Complete resection of FCD according to MRI criteria was achieved in 74% (n = 37) of patients. ILAE class 1 at the last follow-up was achieved in 76% (n = 38) of patients. A reduction of antiepileptic drugs (AEDs) to monotherapy or complete withdrawal was achieved in 60% (n = 30) of patients. Twelve patients (24%) had a late seizure recurrence, 50% (n = 6) of which occurred after reduction of AEDs. A lower number of AEDs prior to surgery significantly predicted a favorable seizure outcome (p = 0.013, HR 7.63). Furthermore, younger age at the time of surgery, shorter duration of epilepsy prior to surgery, and complete resection were positive predictors for long-term seizure freedom. CONCLUSIONS: The duration of epilepsy, completeness of resection, number of AEDs prior to surgery, and younger age at the time of surgery served as predictors of postoperative long-term seizure outcome, and, as such, may improve clinical practice when selecting and counseling appropriate candidates for resective epilepsy surgery. The study results also underscored that epilepsy surgery should be considered early in the disease course of pediatric patients with FCD type II.

Intraoperative MRI-guided Resection in Pediatric Brain Tumor Surgery: A Meta-analysis of Extent of Resection and Safety Outcomes.

BACKGROUND: The objective of this meta-analysis was to analyze the impact of intraoperative magnetic resonance imaging (iMRI) on pediatric brain tumor surgery with regard to the frequency of histopathologic entities, additional resections secondary to iMRI, rate of gross total resections (GTR) in glioma surgery, extent of resection (EoR) in supra- and infratentorial compartment, surgical site infections (SSIs), and neurologic outcome after surgery. METHODS: MEDLINE/PubMed Service was searched for the terms "intraoperative MRI," "pediatric," "brain," "tumor," "glioma," and "surgery." The review produced 126 potential publications; 11 fulfilled the inclusion criteria, including 584 patients treated with iMRI-guided resections. Studies reporting about patients <18 years, setup of iMRI, surgical workflow, and extent of resection of iMRI-guided glioma resections were included. RESULTS: IMRI-guided surgery is mainly used for pediatric low-grade gliomas. The mean rate of GTR in low- and high-grade gliomas was 78.5% (207/254; 95% confidence interval [CI]: 64.6-89.7, p < 0.001). The mean rate of GTR in iMRI-assisted low-grade glioma surgery was 74.3% (35/47; 95% CI: 61.1-85.5, p = 0.759). The rate of SSI in surgery assisted by iMRI was 1.6% (6/482; 95% CI: 0.7-2.9). New onset of transient postoperative neurologic deficits were observed in 37 (33.0%) of 112 patients. CONCLUSION: IMRI-guided surgery seems to improve the EoR in pediatric glioma surgery. The rate of SSI and the frequency of new neurologic deficits after IMRI-guided surgery are within the normal range of pediatric neuro-oncologic surgery.

Headache and Shunt-Related Impact on Activities of Daily Life in Patients Growing Up with a Ventriculoperitoneal Shunt.

BACKGROUND/AIMS: Ventriculoperitoneal shunts (VPS) are a mainstay of treatment for hydrocephalus. Morbidity related to infection and dysfunction is well known, whereas data on psychosocial outcome and quality of life are scarce. Our aim was to assess headache burden and shunt-related impact on daily life in children growing up with a VPS. METHODS: Patients between 3 and 21 years of age were identified and their families were contacted. A set of standardized questionnaires was used to assess headache and quality of life. RESULTS: Fifteen patients with a mean age of 12 years agreed to participate in this study. Satisfaction with the VPS and improvement of the patient's condition was reported by 87%. A total of 67% denied negative impact on everyday life, reported a headache less than once a month, and never or rarely miss school. However, 53% take precautions before special activities. CONCLUSIONS: The results demonstrate that modern VPS systems are tolerated very well and do not per se reduce quality of life. However, patients adapt to the presence of a VPS by avoiding risks and taking precautions. Chronic headache is not a major problem and not necessarily attributed to the VPS. Nevertheless, most patients asked for technical improvements, which might guide future research and the technical development of VPS.

The usefulness of dynamic O-(2-18F-fluoroethyl)-L-tyrosine PET in the clinical evaluation of brain tumors in children and adolescents.

UNLABELLED: Experience regarding O-(2-(18)F-fluoroethyl)-L-tyrosine ((18)F-FET) PET in children and adolescents with brain tumors is limited. METHODS: Sixty-nine (18)F-FET PET scans of 48 children and adolescents (median age, 13 y; range, 1-18 y) were analyzed retrospectively. Twenty-six scans to assess newly diagnosed cerebral lesions, 24 scans for diagnosing tumor progression or recurrence, 8 scans for monitoring of chemotherapy effects, and 11 scans for the detection of residual tumor after resection were obtained. Maximum and mean tumor-to-brain ratios (TBRs) were determined at 20-40 min after injection, and time-activity curves of (18)F-FET uptake were assigned to 3 different patterns: constant increase; peak at greater than 20-40 min after injection, followed by a plateau; and early peak (≤ 20 min), followed by a constant descent. The diagnostic accuracy of (18)F-FET PET was assessed by receiver-operating-characteristic curve analyses using histology or clinical course as a reference. RESULTS: In patients with newly diagnosed cerebral lesions, the highest accuracy (77%) to detect neoplastic tissue (19/26 patients) was obtained when the maximum TBR was 1.7 or greater (area under the curve, 0.80 ± 0.09; sensitivity, 79%; specificity, 71%; positive predictive value, 88%; P = 0.02). For diagnosing tumor progression or recurrence, the highest accuracy (82%) was obtained when curve patterns 2 or 3 were present (area under the curve, 0.80 ± 0.11; sensitivity, 75%; specificity, 90%; positive predictive value, 90%; P = 0.02). During chemotherapy, a decrease of TBRs was associated with a stable clinical course, and in 2 patients PET detected residual tumor after presumably complete tumor resection. CONCLUSION: Our findings suggest that (18)F-FET PET can add valuable information for clinical decision making in pediatric brain tumor patients.

Fluorescence-guided surgery with 5-aminolevulinic acid for resection of brain tumors in children--a technical report.

BACKGROUND: 5-aminolevulinic acid (5-ALA) can be used as an adjunct for the surgery of adult malignant glioma and improves the rate of gross total resection and patient survival. So far, only three casuistic reports of fluorescence-guided surgery used in children have been published. We report our pilot series of 16 pediatric brain tumors treated with 5-ALA. METHODS: Sixteen patients (mean age 9 years, range 1-16 years) received a standardized 5-ALA dose according to the published protocol after informed parental consent. The fluorescence status (positive versus negative) in correlation with histology as well as blood samples and adverse clinical symptoms were recorded. RESULTS: Histology revealed pilocytic astrocytoma (n = 7), classical medulloblastoma (n = 4), anaplastic astrocytoma (n = 1), glioblastoma (n = 3) and anaplastic ependymoma (n = 1). Positive fluorescence was observed in cases of anaplastic astrocytoma, glioblastoma, and medulloblastoma, respectively. Significant increases were registered for alanine aminotransferase (14.92 ± 1.106 U/l vs. 37.70 ± 3.795 U/l, P = 0.0020) and gamma glutamyl transpeptidase (12.69 ± 1.638 U/l vs. 39.29 ± 6.342 U/l, P = 0.0156), correlated with young age. No further adverse reactions were evident. CONCLUSION: Positive fluorescence was observed in two high-grade gliomas and one medulloblastoma after oral administration of 5-ALA. Thus, 5-ALA appears capable of inducing fluorescence in pediatric high-grade tumors. Adverse reactions observed in children were similar to those reported for adults, although very young children might be at increased risk. Further studies are required to elucidate pharmacokinetic and pharmacodynamic properties of 5-ALA in children and to assess its prognostic role in the resection of pediatric brain tumors.

Clinical and radiological presentation of spinal epidural haemangiomas: clinical series in a tertiary care centre during a 10-year period.

PURPOSE: Haemangiomas are very frequent benign spinal tumours. However, pure epidural location is extremely rare. At present, only 52 cases have been reported in the literature during the last 10 years. We proposed to analyse clinical and radiological features of this rare entity treated in a tertiary care centre over the last 10 years. METHODS: A study of a retrospective surgical series (2002-2012) was conducted. The clinic's electronic database was searched for "spinal" and/or "vertebral haemangiomas", which were treated by surgery and/or vertebroplasty. Clinical, radiological and histopathological data were analysed. RESULTS: In total, the series comprised 30 spinal haemangiomas. There were 6 epidural (20 %), 17 vertebral (57 %) and 7 intradural lesions (23 %). There were four men and two women, mean age 28.3 years, with epidural lesions. One patient presented with localised back pain only, two with radiculopathy and focal neurological deficit, two with radiculopathy only and one with isolated focal neurological deficit, respectively. The onset of symptoms was progressive in four cases over weeks to months and sudden in two cases. Preoperative MRI imaging revealed features of meningioma, neurinoma or metastasis. CONCLUSION: Epidural haemangiomas are extremely rare spinal lesions. They may mimic more common spinal tumours clinically and radiologically. The usual treatment is gross total resection confirming the diagnosis histologically.

Giant dumbbell-shaped intra- and extracranial nerve schwannoma in a child presenting with glossopharyngeal neuralgia syncope syndrome: a case report and review of the literature.

Lower cranial nerve schwannomas are benign tumors of the neurolemmocytes of the cranial nerves. Among children, cranial nerve schwannomas are extremely rare and are predominantly associated with neurofibromatosis (NF) type 2. The purpose of the current case report is to describe a unique giant extra- and intracranial foramen jugular schwannoma in a young boy with lower cranial nerve deficits and glossopharyngeal neuralgia syncope syndrome and to review the pertinent literature. In the current case report, we illustrate the course of disease in a 14-year-old boy with a 4-month history of recurrent syncope and a big bulge on the left side of his neck. Audiometry showed deafness of the left ear. Magnetic resonance imaging (MRI) demonstrated a giant unilateral dumbbell-shaped intra- and extracranial foramen jugular schwannoma with a volume of 156 cm3 causing severe brain stem compression and obstructive hydrocephalus. The tumor was removed completely in a two-step surgery. The tumor was confirmed during surgery to originate from the glossopharyngeal nerve. The histological examination revealed the characteristic features of a schwannoma. The MRI 3 months after the second surgery confirmed complete tumor removal. The genetic examination for NF was negative. Review of literature showed that dumbbell-shaped lower cranial nerve schwannomas in the childhood population are rare.

Solitary spinal epidural cavernous angiomas in children presenting with acute neurological symptoms caused by hemorrhage.

Spinal solitary epidural cavernous angiomas are rare benign vascular malformations, which occur even less frequently in children than in adults. It is uncommon to find such lesions without adjacent vertebral involvement. Occasionally, these lesions can lead to neurological symptoms through growth or due to intralesional hemorrhage. In this report the authors describe 2 children presenting with acute symptoms and neurological deficits caused by hemorrhage within solitary spinal epidural cavernous angiomas. A 13-year-old girl and a 9-year-old girl, previously healthy, were admitted to the authors' department due to acute radicular pain and neurological deficits. In both cases MR imaging revealed a solitary epidural mass with signs of bleeding and compression of the spinal cord. Complete resection of the lesion via a dorsal approach was performed in both patients. The histological examination of the lesions revealed the characteristic structures of a cavernous angioma with hemosiderin deposits and acute hemorrhage. Both patients recovered fully after surgical removal of the lesions. Review of the literature confirmed that spinal epidural cavernous angiomas are extremely rare in the pediatric patient population, described currently in only 2 instances, but without acute hemorrhage. These cases suggest that epidural cavernous angiomas also have to be considered in the pediatric patient population in the differential diagnosis of intraspinal lesions with acute or progressive neurological symptoms. Microsurgical resection of these cavernous malformations is an effective and curative treatment option.

Symptomatic intracystic hemorrhage in pineal cysts. Report of 3 cases.

Pineal cysts are benign and often asymptomatic intracranial entities. Occasionally they can lead to neurological symptoms through growth or due to intracystic hemorrhage. The purpose of the current report is to describe their clinical characteristics and treatment options. In the current study, the authors illustrate the course of disease in 3 patients who developed neurological symptoms due to hemorrhage into a pineal cyst. Two of their patients had additional cerebral disease, and regular MR imaging examinations were conducted. This circumstance allowed documentation of growth and intracystic hemorrhage. After the occurrence of new neurological symptoms with severe headache, MR images showed a fluid-fluid interface due to intracystic hemorrhage. The third patient presented with acute triventricular hydrocephalus and papilledema due to aqueductal stenosis caused by intracystic hemorrhage. In all 3 cases, excision of the pineal cysts via an infratentorial/supracerebellar approach was performed. Histological examination revealed the characteristic structure of pineal cyst in all cases, with hemorrhagic residues in the form of hemosiderin deposits. All patients recovered fully after surgical removal of the cysts. Furthermore, resolution of occlusive hydrocephalus could be demonstrated in those cases with ventricular enlargement. Pineal cysts without neurological symptoms are often discovered as incidental findings on cranial MR images. In contrast, neurological symptoms such as severe headache, diplopia, or Parinaud syndrome, may occur as a result of pineal apoplexy due to intracystic hemorrhage. The authors' cases confirm that MR imaging can identify intracystic hemorrhage by a characteristic fluid-fluid interface. Their experience suggests that microsurgical resection of cysts may be an effective and curative treatment option.