Dissemination Patterns and Short-Term Management of Multifocal Rosette-Forming Glioneuronal Tumors.

BACKGROUND: Multifocal rosette-forming glioneuronal tumors (RGNTs) are challenging to manage. Gross total resection is often impossible, and data on adjunctive therapies are limited. We reviewed cases of multifocal RGNTs in the literature with special focus on dissemination patterns and management. METHODS: A literature review was conducted using PubMed and the key words "(multifocal OR multicentric OR satellite OR dissemination) AND glioneuronal." RESULTS: There were 21 cases of multifocal RGNTs identified. Follow-up was available in 18 cases at a median of 17 months. Progression-free survival and overall survival at 1 year were 84% and 94%, respectively. Of all cases, 43% had cerebrospinal fluid (CSF) dissemination, 48% had intraparenchymal spread, and 10% had both. The presence of CSF dissemination led to palliative care and/or death in 20% of cases (n = 2). None of the cases with intraparenchymal spread progressed. Radiotherapy was used in 50% of cases with CSF dissemination, chemotherapy was used in 20%, and CSF shunting was used in 36%. No tumors with intraparenchymal spread required adjunctive therapy or shunting. CONCLUSIONS: RGNTs with CSF dissemination are more likely to behave aggressively, and early adjunctive therapies should be discussed with patients. Tumors with intraparenchymal spread grow slowly, and maximal safe resection followed by observation is likely sufficient in the short term. Long-term behavior of multifocal RGNTs is still unclear.

Endoscopic diagnosis of an MRI-occult, low-grade glioma with ependymal dissemination.

A 21-year-old man presented with triventricular hydrocephalus due to a tectal mass. He underwent an endoscopic third ventriculostomy, and multiple nodules were identified at the floor of the third ventricle intraoperatively. Surgical pathology of one of these lesions demonstrated that the tissue represented a low-grade astrocytoma. The case highlights the existing potential of neuroendoscopy to reveal neuroimaging-occult lesions, in spite of the significant advances of MRI. Furthermore, the combination of the age of the patient, the nonenhancing MRI appearance, and the multifocality of the lesions constitutes a rare and interesting neoplastic presentation within the brain. The constellation of findings likely represents dissemination of a low-grade tectal glioma via the CSF compartment.

Efficacy of simultaneous single-trajectory endoscopic tumor biopsy and endoscopic cerebrospinal fluid diversion procedures in intra- and paraventricular tumors.

OBJECT: Intraventricular and paraventricular tumors resulting in hydrocephalus commonly require a CSF diversion procedure. A tumor biopsy can often be performed concurrently. Although the tissue samples obtained during endoscopic biopsy procedures are small, a diagnosis can be made in most cases. In the present study the authors analyzed the efficacy of concurrent endoscopic biopsy and CSF diversion procedures using a single bur hole and trajectory. METHODS: Eighty-seven patients with intraventricular and paraventricular tumors were treated with endoscopic biopsy and CSF diversion procedures using a rigid rod-lens endoscope or a rigid fiberscope during a 10-year period. All patients underwent a tumor biopsy and an endoscopic third ventriculostomy (ETV), aqueductal stenting (AS), or ventriculoperitoneal (VP) shunting, depending on the tumor location and site of obstruction. A single bur hole for both procedures was used in all patients. RESULTS: Among the 87 patients, the biopsy was diagnostic in 72 (83%) and merely suggestive in 7 (8%); in 8 patients (9%) the sample was nondiagnostic. Among the 22 patients who underwent an initial endoscopic biopsy and subsequent procedures, the specimen obtained at the second surgery was concordant with the initial endoscopic biopsy sample in 13 patients; it was somewhat similar in 4 patients. In the other 5 patients, either a microsurgical or stereotactic approach was used to correctly diagnose the pathology. Fifty-five patients were considered for endoscopic CSF diversion procedures; an ETV was performed in 52 patients and AS in 2. An ETV could not be performed in 3 patients for technical reasons. A VP shunt was inserted in 32 patients, with 25 undergoing shunt placement at the same time as the ETV and 7 at a later date. Significant bleeding was encountered in 3 patients during the tumor biopsy and in 1 patient during the ETV. The ETV failed in 1 patient during the follow-up, and a repeat ETV was required. CONCLUSIONS: Endoscopic biopsy sampling and a concurrent CSF diversion procedure through a single bur hole and trajectory can be considered for intraventricular tumors. The overall success rates of 83% for the biopsy procedure and 86% for the ETV indicate that the procedures are beneficial in the majority of cases. A concordance rate of 75% was found in patients who underwent an initial biopsy procedure and a subsequent microsurgical approach for tumor excision.

An endoscopically proven ventriculitis-type, cyst-like intraventricular primary lymphoma of the central nervous system.

Primary ventriculitis form of primary CNS lymphoma (PCNSL) is very rare and difficult to diagnose from radiological findings. A 55-year-woman presented with a cystic mass in the lateral ventricle without evidence of demonstrable malignant cells in the cerebrospinal fluid (CSF). The endoscopic biopsy showed a ventriculitis-like thin web on the surface of the ventricular wall around the foramen of Monro, resulting in obstruction. Pathological diagnosis was non-Hodgkin's lymphoma, B cell type. This case suggests that PCNSL should be considered as a differential diagnosis for a cystic ventricular lesion even with no malignant cells in the CSF.

Detection of craniospinal dissemination of intracranial germ cell tumours based on serum and cerebrospinal fluid levels of tumour markers.

Nineteen intracranial germ cell tumours treated during the past 11 years were evaluated retrospectively. The tumours were classified into three groups according to the level of alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (HCG) in serum and cerebrospinal fluid, and the optimal treatment for each group was determined. Group A consisted of patients with normal titres of AFP and HCG, group B of patients with relatively high titres (< 10 times normal), and group C comprised patients with higher titres ( 10 times normal levels). In group A, an appropriate field and dose of irradiation was necessary to prevent craniospinal dissemination. In group B, none of four patients who underwent total or subtotal resection of the tumour had craniospinal disseminatio n or tumour recurrence, whereas dissemination occurred in four further patients, including two who had received radiochemotherapy only, and two who had undergone partial resection of the tumour. While patients in group C had dissemination at the time of initial diagnosis, most patients in group B developed dissemination more than 10 months after initial treatment, suggesting that the type of treatment received as first line therapy is important in patients in this group. The prognosis of patients in group C, however, was unaffected by the priority given to either surgery, radiation or chemotherapy as first line treatment. Craniospinal dissemination can be prevented in patients with germ cell tumours who have a relative increase in levels of AFP and HCG by aggressive removal of the tumours as first line therapy, regardless of the type of adjuvant therapy given.

[Imaging of tumors of the third ventricle]. / Imagerie des tumeurs du troisième ventricule.

MR imaging now appears as the best tool for diagnosis and pre-therapeutic assessment of tumors arising in the third ventricle (V3), as MR images can be obtained in the axial, coronal, and sagittal planes. MRI makes certain the location of the tumor in the V3 and delineates the anatomical landmarks in order to plan the surgical approach. It also allows the evaluation of associated hydrocephalus. Colloid cysts, choroid plexus papillomas, cavernomas, subependymal giant cell astrocytomas and germ-cell tumors may exhibit specific radiological features.

Craniospinal dissemination of central neurocytoma. Report of two cases.

Central neurocytoma was first described in the literature in 1982 and has been noted to be a benign neuronal tumor usually located in the ventricular system. Of the more than 100 reported cases, only seven recurrences have been reported, all of which have been local. The authors report two cases of recurrent central neurocytoma that disseminated through the ventricular system with seeding to the spine, as evidenced by magnetic resonance images and positive cerebrospinal fluid cytology. The histological appearance of these two tumors was typical for the lesion and lacked evidence of malignant change. Central neurocytoma may not be as benign as previously thought, and the recognition of this more malignant behavior has implications for patient follow up and therapy.

Cerebrospinal fluid flow. III. Pathological cerebrospinal fluid pulsations.

Cardiac- and respiration-related movements of the cerebrospinal fluid (CSF) were investigated by MRI in 71 patients. In most patients with arteriosclerotic occlusive vascular disease CSF pulsations are normal. Decreased pulsatile flow is detectable in those with arteriovenous malformations, intracranial air and following lumbar puncture and withdrawal of CSF. Increased pulsatile flow in the cerebral aqueduct was found in 2 patients with large aneurysms, idiopathic communicating syringomyelia and in most cases of normal pressure hydrocephalus (NPH). CSF flow in the cervical spinal canal is, however, reduced or normal in NPH, indicating reduction of the unfolding ability of the surface of the brain and/or inhibition of rapid CSF movements in the subarachnoid space over its convexity.

Primary germinoma of the brain. Immunocytochemical demonstration of tumour cells in the cerebrospinal fluid.

A patient is presented, who developed a suprasellar tumour. Stereotactical biopsy of the tumour revealed the diagnosis of a dysgerminoma. Immunocytochemical examination of the CSF showed neoplastic cells staining for human chorionic gonadotropin and for alpha-fetoprotein. The authors stress the possibility to diagnose primary intracranial germ cell tumours without operation.

Etiology of ventriculomegaly in choroid plexus papilloma.

In order to test the hypothesis that overproduction of cerebrospinal fluid can cause the hydrocephalus seen in choroid plexus papillomas, adult mongrel dogs with and without hydrocephalus were subjected to high pressure intraventricular infusions of artificial cerebrospinal fluid (CSF). Pre- and postinfusion volumes were calculated, using a new method for on-line measurement of ventricular volume involving linear measurements from Conray ventriculograms. Ventricular volumes increased an average of 8% in 36 h. The dogs previously made hydrocephalic by the intracisternal injection of kaolin had a significant volume increase (34% in 36 h). Overproduction of CSF alone can produce hydrocephalus, but the presence of hydrocephalus in choroid plexus papillomas is more likely to be a result of the complex interaction of CSF overproduction and partial restriction of CSF flow.

Possible influence of cerebrospinal fluid pressure on the growth of a third ventricle choroid plexus papilloma.

A case of histologically proven benign choroid plexus papilloma of the third ventricle in a 4-month-old male child is reported. The clinical symptoms and the neuroradiologic findings were those of a supratentorial hydrocephalus. A shunting procedure was performed after refusal by the family of a direct surgical approach. Subsequently there was a four- to fivefold increase in volume of this tumor in less than 4 months under decreased cerebrospinal fluid pressure. The purpose of this report is first to add a case to 27 cases of papilloma of the choroid plexus of third ventricle reported in the medical literature. Secondly to underline a possible influence of intraventricular pressure on the growth of a histologically benign intraventricular papilloma. Subsequently, the question of radiation therapy may be raised, if total removal of the papilloma is not obtained, especially in view of decreased cerebrospinal fluid pressure provided by the previous shunting procedure.

Contribution to the background of Mollaret's meningitis.

An epidermoid cyst of the 3rd ventricle was verified by necropsy in a man of 60 with three episodes of Mollaret's aseptic leukocytic-endothelial meningitis. The authors accept the interpretation as macrophages of the "endothelial" cells. They bring forward evidence for the blastomatous nature of partly cornified epithelial cells present in their case. They suggest that cholesterin containing debris entering the subarachnoid space from the tumour cysts provoke an aseptic "chemical" meningitis of short course.

Lateral ventricle plexus papilloma and communicating hydrocephalus.

A choroid plexus papilloma was found in the right lateral ventricle of a five-month-old boy. Though it was associated with an important communicating hydrocephalus, it was seen at operation to be freely mobile and did not directly obstruct the flow of the cerebrospinal fluid. Computerized axial tomography was the most helpful para-clinical investigation for diagnostic purposes, and for follow-up study. Tumor removal alone was sufficient to cure the hydrocephalus, and no shunt was necessary. We feel that this case demonstrates that hyperproduction of CSF is sufficient alone to cause communicating hydrocephalus in patients with choroid plexus papilloma of the lateral ventricle.

Surgical removal of bilateral papillomas of the choroid plexus of the lateral ventricles with resolution of hydrocephalus. Case report.

The authors report a patient with bilateral papillomas of the choroid plexus of the lateral ventricles with documentation of cerebrospinal fluid (CSF) hypersecretion causing hydrocephalus. Special attention is given to the large volume of CSF produced by these tumors (removal of one tumor reduced CSF outflow by one-half) and to the fact that CSF diversion was not required after both tumors were removed. Since tumor removal alone was sufficient to stop the progression of hydrocephalus, we feel that this case supports the concept that elevated CSF production by itself is sufficient to cause hydrocephalus in patients with papillomas of the choroid plexus.

Concentration of homovanillic acid and 5-hydroxyindoleacetic acid in the ventricular cerebrospinal fluid of patients with obstructive hydrocephalus.

In 12 patients with different posterior fossa tumours the concentrations of homovanillic acid (HVA) and of 5-hydroxyindoleacetic acid (5-HIAA) were measured in cerebrospinal fluid from the lateral ventricles. All patients had obstructive hydrocephalus. Patients with a clear increase of 5-HIAA/HVA ratio in the ventricular CSF have died subsequently. This feature may have a diagnostic value, and indicates the prevalence of serotoninergic neurones in patients with obstructive hydrocephalus with fatal course after surgery.