Staged Gamma Knife radiosurgery for a rosette-forming glioneuronal tumor of the fourth ventricle: a case report.

BACKGROUND: Rosette-forming glioneuronal tumor (RGNT) is a rare slow-growing neoplasm with mixed glial and neurocytic components. Surgical resection is the mainstay of treatment, whereas the role of adjuvant radiation therapies for residual or recurrent tumors has been poorly investigated. CASE PRESENTATION: We describe the case of a patient with a recurrent fourth ventricular RGNT who was treated with two-staged Gamma Knife radiosurgery (GKRS). GKRS was effective in controlling tumor growth and safe up to seven years from treatment. CONCLUSIONS: This case suggests that GKRS may be a safe and effective treatment for patients with recurrent or residual RGNT.

Outcomes after gamma knife radiosurgery for intraventricular meningiomas.

BACKGROUND: Intraventricular meningiomas (IVMs) are rare tumors with considerable treatment-associated morbidity due to their challenging location. Treatment with stereotactic radiosurgery (SRS) is sparsely reported in the literature. We describe our experience over the last 35 years using Gamma knife radiosurgery (GKRS) for IVMs. METHODS: We retrospectively reviewed the GKRS database identifying 2501 meningiomas treated at the University of Pittsburgh Medical Center over the last 35 years. Nineteen patients with (12 males, mean age = 53.2 years, range 14-84) 20 IVMs were identified. Headache was the most frequent presenting symptom (N = 12), and the trigone of the lateral ventricle was the most common location (N = 18). The median tumor volume was 4.8 cc (range, 0.8-17). The median margin dose was 14 Gy (range, 12-25) delivered at 50% isodose line. RESULTS: At a median follow-up of 63.1 months (range, 6-322.4) symptom control was achieved in 18 (94.7%) patients. The overall progression-free survival (PFS) was 95% at 5 years, and 85% at 10-years. After Log-rank test, patients who underwent GKRS within 12 months after diagnosis (vs. ≥ 12 months, X2: 4.455, p = 0.035), patients treated with primary GKRS without prior biopsy (vs. prior biopsy, X2: 4.000, p = 0.046), and patients with WHO grade I meningioma (vs. WHO II, X2: 9.000, p = 0.003) had a longer PFS. Imaging showed peritumoral edema in seven cases at a median of 10.5 (range, 6.13-24.3) months after GKRS. Only three of these patients were symptomatic and were successfully managed with oral medications. Cox´s regression revealed that a V12Gy ≥ 10 cc [HR: 10.09 (95% CI: 2.11-48.21), p = 0.004], and tumor volume ≥ 8 cc [HR: 5.87 (95% CI: 1.28-26.97), p = 0.023] were associated with a higher risk of peritumoral edema. CONCLUSION: GKRS is an effective and safe management option for intraventricular meningiomas. Early GKRS should be considered as a primary management modality for small and medium sized IVM and adjuvant management for residual IVMs.

Rosette Forming Glioneural Tumor Treated with Conformal Radiation.

Rosette forming glioneural tumors (RGNT) are a rare type of low-grade brain tumor included in 2007 in WHO classification. Given the benign nature of the disease, a complete surgical excision has been considered optimum. However, a handful of cases have reported the locally aggressive nature of RGNT. In addition, radiation may also be considered for a tumor located in areas where surgical excision is difficult. We present a similar case, where surgical risk was weighed against resection and we treated the patient with conformal radiation.

Innumerable Meningiomas Arising in a Patient With Tuberous Sclerosis Complex Decades After Radiation Therapy.

Meningioma is the most common radiation-induced brain neoplasm, usually occurring after a latency of 20 - 35 years, with multiplicity in 10% of cases. Radiation-induced meningiomas (RIMs) have not previously been reported in patients with tuberous sclerosis complex (TSC), unlike their well-known occurrence in other familial tumor predisposition syndrome patients. We report a TSC patient who developed numerous intracranial meningiomas twenty five year after radiation therapy for subependymal giant cell astrocytoma (SEGA). Autopsy examination showed innumerable, coalescent, benign, meningothelial meningiomas, WHO grade 1, ranging in size from 0.2 cm to 3.3 cm. Autopsy also showed small residual SEGA, radiation-induced cerebral vasculopathy, and classic TSC features including several small subependymal nodules ("candle gutterings"), white matter radial heterotopia, facial angiofibromas, dental enamel pitting, one ash leaf spot, and multiple hepatic and renal angiomyolipomas. Next-generation sequencing analysis utilizing a 500+ gene cancer panel demonstrated chromosomal loss involving the majority of chromosome 22, including the NF2 gene locus, as well as a truncating nonsense mutation in TSC1 p. R509*. While TSC patients rarely require radiation therapy, this striking case suggests that patients with TSC should be monitored closely if cranial therapeutic radiation is administered.

Central neurocytoma originating in third ventricle with expansion into the cerebral aqueduct and fourth ventricle: Case report and review of literature.

BACKGROUND: Central Neurocytomas (CNs) are rare brain tumors, making up less than 1% of all primary tumors within the CNS. They are commonly located in the lateral ventricles, and often present with visual changes and symptoms of obstructive hydrocephalus. Histopathology shows characteristics similar to ependymomas and oligodendrogliomas, however tumor cells display neuronal differentiation, and immunohistochemical stains typically for synaptophysin. Gross total resection is the most important prognostic indicator of survival. CASE DESCRIPTION: We describe the case of a 48-year-old male with a CN originating in the third ventricle with expansion through the cerebral aqueduct into the fourth ventricle. He presented with bi-frontal headaches, imaging revealed an avidly enhancing tumor occupying the inferior third ventricle, cerebral aqueduct, with expansion into the fourth ventricle. An interhemispheric craniotomy with a transcallosal transchoroidal approach to the third ventricle was performed, this provided a trajectory that paralleled the long axis of the tumor. Postoperative imaging confirmed a near total resection with linear residual enhancement on the anterior wall of the fourth ventricle. Intensity modulated radiotherapy was performed, 7-month follow-up imaging was clean. CONCLUSION: CNs are rare brain tumors, most commonly located within the lateral ventricles. We describe a rare case of a CN spanning from the third ventricle into the cerebral aqueduct and fourth ventricle. To our knowledge, this is only the fourth reported case of such a tumor. Surgical approach must be carefully selected, as gross total resection is the most important prognostic indicator.

Clinical features, radiological findings, and treatment outcomes of high-grade lateral ventricular meningiomas: a report of 26 cases.

High-grade meningiomas in ventricles are rare, where most published series only include a few patients. A retrospective analysis was performed on the clinical features, radiological findings, and treatment outcomes of 26 patients with high-grade meningiomas in lateral ventricles who were surgically treated in our hospital between July 2008 and July 2016. A female predilection (female/male = 1.4:1) was observed with a mean age of 42.4 years. Headache and/or vomiting (65.3%) were the most common initial symptom, and with symptom duration time ranging between 7 days and 5 years (mean 8.5 months). The lateral ventricle trigone area was the most common site (80.7%). Twenty-two patients (84.6%) obtained gross total resection. The 2007 WHO classification was used to classify 22 (84.6%) meningiomas as grade II and the remaining four tumors were graded III. These tumors accounted for a recurrence rate of 38.5% (10 of 26 patients) and a mortality rate of 11.5% (3 deaths) during the follow-up periods. The recurrence rate after the gross total resection was 27.3% (6 of 22 patients). Radiotherapy was administered as an adjuvant treatment in 12 patients (46.2%) after surgery. There were 4 recurrences out of the 12 patients who received radiotherapy and 6 of the 14 patients relapsed without radiotherapy (p = 0.58). The subtotal resection was considered a risk factor for recurrence. The postoperative radiotherapy seemed to have little significance for the high-grade meningiomas in the lateral ventricles. Long-term follow-up is required, regardless of the resection grade, and reoperation is feasible for patients with recurrence.

Pitfalls of Neuroendoscopic Biopsy of Intraventricular Germ Cell Tumors.

OBJECTIVE: A neuroendoscopic biopsy has become common for the diagnosis of ventricular tumors. However, its utility in patients with germ cell tumors (GCTs) has not been well discussed. We examined the usefulness and pitfalls of neuroendoscopic biopsies of intraventricular GCTs at a single institution. METHODS: We retrospectively studied 21 consecutive patients diagnosed with GCTs by a neuroendoscopic biopsy of the ventricular region via the lateral ventricle. We examined the localization of tumors, histologic diagnoses using biopsies, surgical complications, and consistency of the diagnosis at the latest follow-up. RESULTS: Tumor specimens were obtained from a pineal lesion (n = 20), neurohypophysial lesion (n = 5), and lateral ventricular wall lesion (n = 2). In 5 patients, the specimens were obtained from multiple areas. The initial diagnoses were pure germinoma (n = 16), immature teratoma (n = 1), yolk sac tumor (n = 1), and mixed GCT (n = 3). Six of 21 patients needed a second transcranial removal of enhanced residual lesions in the course of the treatment. A discrepancy in the histologic diagnosis between 2 surgeries occurred in 3 patients: All 3 patients had a new diagnosis of teratoma component following transcranial surgery. No postoperative mortality or permanent morbidity related to the neuroendoscopic procedures was noted. CONCLUSION: Neuroendoscopic biopsies are safe and useful for obtaining reliable histologic diagnoses in the management of GCTs. However, for GCTs with mixed histology, biopsies are susceptible to diagnostic errors, especially missing detecting a component of teratoma.

[Multiple primary liponeurocytoma of the central nervous system].

We report a unique case of primary multiple liponeurocytoma. Liponeurocytoma is a rare benign tumor (Grade II) of the posterior cranial fossa with neural- or probably astrocytic-lineage cells; the tumor stroma contains mature adipocytes. This case is the thirty-eighth case of this pathology reported in the world literature and the first case of multiple form of this disease. We have provided a sufficiently thorough neuroradiological and histological picture that allows one to differentiate between a liponeurocytoma and other histological variants. Recommendations for the tactics for managing patients with this rare disease are proposed based on the analysis of treatment of this pathology reported in the world literature.

Análisis coste-efectividad de la cirugía del glioma maligno guiada por fluorescencia con ácido 5-aminolevulínico / Cost-effectiveness of 5-aminolevulinic acid-induced fluorescence in malignant glioma surgery

Objetivo: Evaluar el coste-efectividad del ácido 5-aminolevulínico (5-ALA, Gliolan(R)) en pacientes intervenidos quirúrgicamente de glioma maligno, en condiciones de práctica médica habitual en España. Material y métodos: Se determinaron las ratios de coste incremental por resección completa (RC) y de coste incremental por año de vida ajustado por calidad (AVAC) ganado, sobre la base de los datos recogidos en el estudio observacional VISIONA. Resultados. El coste incremental con 5-ALA frente a la cirugía convencional con luz blanca asciende a 4.550 Euros por RC adicional conseguida y a 9.021 Euros por AVAC ganado. Estos resultados se muestran consistentes en un análisis de sensibilidad. Conclusión: La cirugía del glioma maligno guiada por fluorescencia con 5-ALA conlleva un incremento de costes moderado respecto a la práctica quirúrgica actual y muestra una relación coste-efectividad favorable

Objective: This study evaluates the cost-effectiveness of 5-aminolevulinic acid (5-ALA, Gliolan®) in patients undergoing surgery for malignant glioma, in standard clinical practice conditions in Spain. Material and methods: Cost-effectiveness ratios were determined in terms of incremental cost per complete resection (CR) and incremental cost per additional quality-adjusted life year (QALY), based on data collected in the VISIONA observational study. Results: Incremental cost with 5-ALA versus conventional surgery using white light only amounts to Euros 4550 per additional CR achieved and Euros 9021 per QALY gained. A sensitivity analysis shows these results to be robust. Conclusion: Malignant glioma surgery guided by 5-ALA fluorescence entails a moderate increase in hospital costs compared to current surgical practice and can be considered a cost-effective innovation

Intraventricular neurocytomas: a systematic review of stereotactic radiosurgery and fractionated conventional radiotherapy for residual or recurrent tumors.

To determine optimal treatment for recurrent or residual intraventricular neurocytomas (IVNs), a systematic review of PubMed and EMBASE was conducted comparing fractionated conventional radiotherapy (FCRT) versus stereotactic radiosurgery (SRS). Inclusion criteria included histological IVN documentation, at least 6 months of follow-up observation and described dose of FCRT or SRS administered. Descriptive statistical and Kaplan-Meier analyses were performed. The literature search yielded 451 articles. Sixteen studies met inclusion criteria. The local tumor control proportion was 93% and 88% in the SRS and FCRT subgroups, respectively. The relative risk (RR) of local recurrence was 0.57 less (95% CI: 0.21-1.57; log-rank p=0.85) and the RR of all-cause mortality was 0.23 less (95% CI: 0.05-1.05; log-rank p=0.22) in SRS subgroup compared to the FCRT subgroup but did not reach statistical significance. Recurrence was significantly associated with presence of histological atypia (log-rank p<0.001). Severe complications were lower in SRS subgroup (5.5% versus 7.5%, p=0.74); however, distant tumor recurrence was slightly lower in the FCRT subgroup (1.5% versus 5.5%, p=0.24). The role of SRS or FCRT in the management of residual or recurrent IVNs will continue to depend on the balance between the risks and benefits of SRS and FCRT until better quality data are available.

Long-term disease-free survival in a patient with cerebral recurrence from adenocarcinoma of the fallopian tube.

Cerebral recurrence from Müllerian cancer is a rare event and prognosis of patients with such a condition is poor. We report a case of cerebral recurrence from International Federation of Gynecology and Obstetrics classification stage IV tubal cancer presenting with inguinal lymphadenopathy. The patient achieved more than 7 years' disease-free survival after irradiation to the brain despite the inauspicious event. The present case had a rare clinical course in terms of primary site, primary symptom, failure site, and clinical outcome. Patients with brain metastasis from Müllerian cancer have a chance for long-term survival under specified circumstances, such as solitary metastasis, no extracranial metastasis, no recurrence preceding brain metastasis and small tumor size.

Atypical teratoid/rhabdoid tumor with 26-year overall survival: case report.

Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive embryonic brain tumor predominantly seen in young children. The authors report an unusual case of a fourth ventricle AT/RT in an infant who survived for 26 years. The tumor was resected when the patient was 6 months of age, and radiation therapy (40-Gy total dose) was performed thereafter. The patient was free from the disease for 26 years until a recurrent tumor was found in the spinal cord. The spinal cord neoplasm was a "collision tumor" with 2 components: benign schwannoma and recurrent AT/RT. The patient died of dissemination of the recurrent tumor 5 months after it was excised. This is the longest survival of a patient with AT/RT ever reported and indicates that long-term survival, more than 20 years, can be achieved in infantile-onset AT/RT. Despite intensive treatment, the prognosis for AT/RT is very poor, especially in children younger than 3 years of age. The benefits of upfront radiation therapy for AT/RT should be carefully assessed with respect to its inevitable toxicity in very young children. However, early upfront radiation therapy may be of therapeutic interest to prevent aggressive progression of the disease.

Subarachnoid dissemination of intraventricular tumors following simultaneous endoscopic biopsy and third ventriculostomy.

OBJECT: Endoscopic biopsy with concomitant third ventriculostomy (ETV) is a well-established diagnostic and therapeutic maneuver in patients presenting with noncommunicating hydrocephalus resulting from a tumor of the pineal region or posterior third ventricle. Fenestration of the floor of the third ventricle theoretically provides a conduit for the subarachnoid dissemination of an intraventricular tumor. The aim of this study was to ascertain the rate of leptomeningeal dissemination following this surgical procedure. METHODS: The authors conducted a review of all patients for whom an ETV and simultaneous endoscopic biopsy procedure or tumor resection had been performed at their institutions between 1995 and 2008. Patients were divided into high or low risk groups by leptomeningeal metastatic potential based on pathology. All available postoperative clinical and radiographic data, including MR imaging of the brain and spinal cord, as well as CSF sampling were evaluated when available. A review of the literature was then conducted to establish rates of distant leptomeningeal dissemination for comparative purposes. RESULTS: Thirty-two patients satisfied the criteria for study inclusion. Pathology revealed that 22 had a high risk for leptomeningeal dissemination. New leptomeningeal disease (1 yolk sac tumor and 1 pineoblastoma) occurred in 2 patients. The median clinical and brain MR imaging follow-ups overall were 34 (range 2-103 months) and 38 months (range 1-94 months), respectively. Follow-up MR imaging of the spine was performed in 12 patients (median 7 months postoperation), and CSF was analyzed in 15 patients (median 1 month postoperation). A Kaplan-Meier survival analysis predicted a 2-year metastasis-free survival of 94.7% for high-risk patients. Baseline rates of dissemination when ETV was not performed were in general between 8 and 24% for various high-risk pathologies according to a literature review. CONCLUSIONS: The rate of leptomeningeal metastasis of tumors in this biopsy and ETV study was not increased when compared with rates from large series in the literature.

Short communication: conformal therapy for peri-ventricular brain tumors: is target volume deformation an issue?

Physiologic variations in ventricular volumes could have important implications for treating patients with peri-ventricular brain tumors, yet no data exist in the literature addressing this issue. Daily megavoltage computed tomography (CT) scans in a patient with neurocytoma receiving fractionated radiation revealed minimal changes, suggesting that margins accounting for ventricular deformation are not necessary.

An evaluation of conformal and intensity-modulated radiotherapy in whole ventricular radiotherapy for localised primary intracranial germinomas.

AIMS: To evaluate different treatment techniques in whole ventricular radiotherapy (WVRT) for localised intracranial germinomas with respect to target volume and organ at risk (OAR) dose. As a second end point, a comparison of OAR doses between WVRT and whole brain radiotherapy was made. MATERIALS AND METHODS: Co-registered computed tomography-magnetic resonance images (MRI) of five patients were studied. Planning target volumes for whole ventricles (phase 1, PTV(1)) and boost to the primary tumour (phase 2, PTV(2)) were prescribed to 24 and 16 Gy. For phase 1, lateral parallel-opposed pairs (PP), non-coplanar three- and four-field (3F and 4F) and optimised four- and seven-field intensity-modulated radiotherapy (4FIMRT and 7FIMRT) plans were compared. A conformal non-coplanar six-field technique was used for phase 2. RESULTS: For phase 1, statistically significant high-dose sparing of normal brain was achieved with increasing treatment complexity for supratentorial brain (STB) minus PTV(1) (STB-PTV(1)) and infratentorial brain (ITB) minus PTV(1) (ITB-PTV(1)). Median pituitary gland dose sparing was 1 Gy with IMRT techniques (P=not significant). CONCLUSION: WVRT using 7FIMRT is the most conformal technique, which offers significant sparing of normal brain from high-dose irradiation, a mean reduction of 1 Gy in the pituitary gland (P<0.05) with no significant reduction in other OARs. Yet even with the most complex technique the absolute reduction in mean dose to normal brain tissue achieved was modest in clinical terms. Whether this could translate into a reduction in late sequelae in a predominantly adolescent patient population remains hypothetical.

Attention mediates radiation's impact on daily living skills in children treated for brain tumors.

BACKGROUND: Radiotherapy is associated with an increased survival rate in children with brain tumors, but also with cognitive decline. This study examined the time-dependent effects of radiation treatment on adaptive functioning in children with brain tumors. The potentially mediating effects of attention span (Trial 1 of the Rey Auditory Verbal Learning Test [RAVLT]) assessed within 7 years of diagnosis were explored. PROCEDURE: Twenty-two children treated with cranial radiation for third ventricle or cerebellar tumors were included in this archival study. The mean age at diagnosis was 7.62 years (SD = 4.78) and 10.16 years (SD = 3.83) at evaluation. We examined the extent to which auditory attention span was able to mediate the relationship between time elapsed since the initiation of radiation treatment (M = 2.43 years; SD = 2.37) and adaptive functioning (Vineland Adaptive Behavior Scales [VABS]). RESULTS: Attention span was found to mediate the relationship between time since the initiation of radiation and daily living skills. These findings were shown to be specific to attention and not a reflection of generalized neuropsychological decline, nor were they a result of increasing time since diagnosis in and of itself. CONCLUSIONS: The results of this study suggest that time since radiation may directly decrease attention and poor attention in turn may be associated with lower adaptive functioning on tasks of daily living.

Acute transient encephalopathy following paclitaxel treatment in an adolescent with a recurrent suprasellar germinoma.

Paclitaxel is an antineoplastic agent that is used in the treatment of a variety of solid tumors. Dose-limiting side effects of myelosuppression and peripheral neuropathy are well known. Paclitaxel has minimal penetration of the blood-brain barrier and central nervous system side effects are rare. However, transient encephalopathy following paclitaxel infusion has been described in adults but not in children. We present the case of a 14-year-old female with a recurrent suprasellar germinoma who developed an acute encephalopathy 4-6 hr following paclitaxel infusion.

[Diagnosis and micro-neurosurgery for the fourth cerebral ventricle tumors].

OBJECTIVE: To investigate the diagnostic method and analyze the result of microneurosurgical treatment for tumors of the fourth cerebral ventricle. METHODS: Tumor of the fourth ventricle was clinically diagnosed in 86 patients basing on the preliminary assessment of symptom and CT or MRI findings. Of these 86 patients treated with micro-neurosurgery, the tumors in 62 were totally removed, subtotally in 19, and partially in 5. Forty-two patients received postoperative radiotherapy. RESULTS: Three patients died postoperatively within ten days, and symptoms in 83 were improved after treatment. The average survival period was over 3 years. The pathology included 32 medulloblastomas, 23 ependymoma, 15 astrocytoma, 10 hemangiblastomas, 2 choroid plexus papillomas, and 4 epidermoid cysts. CONCLUSION: Medulloblastoma, astrocytoma and hemangiblastoma are suggested to be removed totally whenever technically possible according to the site, character and volume of the tumor. For ependymoma, if close to the brain stem, is recommended to be subtotally removed. Postoperative radiotherapy may be beneficial for malignant types.

Recurrent intracranial germinoma along the endoscopic ventriculostomy tract. Case report.

The authors report a case of a recurrent intracranial germinoma along the site of an endoscopic third ventriculostomy (ETV) after complete local tumor control using 3D conformal radiation therapy. A 13-year-old girl presented with sudden left upward gaze limitation for 4 days. A pineal region tumor and obstructive hydrocephalus were noted on magnetic resonance (MR) images. An ETV and tumor biopsy procedure were performed, which revealed the lesion to be a germinoma. The patient's visual symptoms and hydrocephalus disappeared postoperatively. Chemotherapy using cisplatin, etoposide, vincristine, and cyclophosphamide was initiated on postoperative Day 10. An MR image obtained 10 weeks after surgery and 2 weeks after chemotherapy revealed a significant (> 50%) reduction of the lesion. Radiation therapy was administered at 50.4 Gy to the target and 36 Gy to the periphery. Ten months after surgery, an MR image revealed further shrinkage of the tumor mass. One year after surgery, follow-up MR imaging demonstrated a small mass lesion at the entry site of the ETV, measuring 1.0 x 1.4 x 1.5 cm. An operation was performed to remove the small lesion, and pathological findings revealed it to be of the same histology as the primary tumor.