Spontaneous involution of a large pineal cyst: Case report and narrative review.

OBJECTIVE: To illustrate the possibility of spontaneous involution of a pineal cyst, justifying an initial observation strategy in patients without evident mass effect. BACKGROUND: Pineal cysts are frequent radiological findings, with a reported overall prevalence from 0.6% to 40%. Historically, surgery has been reserved for patients with symptoms attributable to a mass effect of the cyst. Despite the high prevalence of pineal cysts, the clinical approach to patients with non-specific symptoms remains controversial. METHODS: We report on the spontaneous involution of a large pineal cyst in a 26-year-old female who presented in our outpatient clinic with transient symptoms of headache and nausea. PubMed and Web of Science databases were scrutinized using a predefined search strategy in accordance with the Population, Intervention, Comparison and Outcome (PICO) set-up using "pineal cyst" and "surgery" as search terms. Only peer-reviewed publications were considered eligible. Titles and abstracts of 1513 manuscripts were screened for relevance. After excluding 1420 publications evaluating non-relevant pathology, the eligibility of the remaining 93 full-text records was further assessed and included if they reported patients with pineal cysts presenting with intractable symptoms without hydrocephalus or Parinaud syndrome, and if they documented on their symptomatology and clinical management. CONCLUSION: Recent case series and reviews report favorable results of surgery in patients with pineal cysts but also a potentially high complication rate. However, the evidence offered by these reports is limited and a placebo effect cannot be ruled out. Therefore, surgery cannot be unequivocally advocated in these patients. Instead, an initial surveillance strategy is advocated. We concur with a previously propounded surveillance strategy of a single follow-up magnetic resonance imaging at 12 months. This case report demonstrates the importance of an initial observation strategy since pineal cysts may also show a spontaneous involution.

The SAFE-trial: Safe surgery for glioblastoma multiforme: Awake craniotomy versus surgery under general anesthesia. Study protocol for a multicenter prospective randomized controlled trial.

BACKGROUND: Surgery of GBM nowadays is usually performed under general anesthesia (GA) and resections are often not as aggressive as possible, due to the chance of seriously damaging the patient with a rather low life expectancy. A surgical technique optimizing resection of the tumor in eloquent areas but preventing neurological deficits is necessary to improve survival and quality of life in these patients. Awake craniotomy (AC) with the use of cortical and subcortical stimulation has been widely implemented for low-grade glioma resections (LGG), but not yet for GBM. AC has shown to increase resection percentage and preserve quality of life in LGG and could thus be of important value in GBM surgery. METHODS/DESIGN: This study is a prospective, multicenter, randomized controlled trial (RCT). Consecutive patients with a glioblastoma in or near eloquent areas (Sawaya grading II/III) will be 1:1 randomized to awake craniotomy or craniotomy under general anesthesia. 246 patients will be included in neurosurgical centers in the Netherlands and Belgium. Primary end-points are: 1) Postoperative neurological morbidity and 2) Proportion of patients with gross-total resections. Secondary end-points are: 1) Health-related quality of life; 2) Progression-free survival (PFS); 3) Overall survival (OS) and 4) Frequency and severity of Serious Adverse Effects in each group. Also, a cost-benefit analysis will be performed. All patients will receive standard adjuvant treatment with concomitant chemoradiotherapy. DISCUSSION: This RCT should demonstrate whether AC is superior to craniotomy under GA on neurological morbidity, extent of resection and survival for glioblastoma resections in or near eloquent areas. TRIAL REGISTRATION: Clinicaltrials.gov: NCT03861299 Netherlands Trial Register (NTR): NL7589.

Loss of endoplasmic reticulum calcium pump expression in choroid plexus tumours.

AIMS: Sarco/Endoplasmic Reticulum Calcium ATPase-type calcium pumps (SERCA enzymes) control cell activation by sequestering calcium ions from the cytosol into the endoplasmic reticulum. Although endoplasmic reticulum calcium signalling plays an important role in the regulation of choroid plexus epithelial function, SERCA expression in the choroid plexus has not been investigated so far. METHODS: In this work we investigated the expression of the SERCA3-type calcium pump in choroid plexus epithelial cells grown in vitro, and in normal and hyperplastic choroid plexus tissue, in choroid plexus papillomas displaying various degrees of atypia, and in choroid plexus carcinoma by immunohistochemistry in situ. RESULTS: Whereas normal choroid plexus epithelial cells express SERCA3 abundantly, SERCA3 expression is strongly decreased in papillomas, and is absent in choroid plexus carcinoma, while expression in hyperplastic epithelium is high, similarly to normal epithelium. SERCA3 expression was detected also in normal primary choroid plexus epithelial cells grown in vitro, and expression was markedly enhanced by short-chain fatty acid-type cell differentiation inducing agents, including valproate. CONCLUSION: These observations show that SERCA3 is a new phenotypic marker of normal choroid plexus epithelial differentiation, and that SERCA3 constitutes an early tumour marker 'by loss of expression' in the choroid plexus that may be useful to distinguish hyperplastic processes from papillomas. Endoplasmic reticulum calcium homeostasis becomes anomalous, due to loss of SERCA3 expression, already in benign neoplastic lesions of the choroid plexus epithelium.

Endoscopic coagulation of choroid plexus hyperplasia.

Hydrocephalus is a clinical disorder resulting from an imbalance between the production of CSF and its resorption, of which the latter is mostly a disadvantage. In rare cases of choroid plexus papilloma or carcinoma, hydrocephalus is due to an overproduction of CSF. Choroid plexus hyperplasia (CPH) is a distinct clinicopathological entity in which the enlarged choroid plexus produces large amounts of CSF. Historically, patients with CPH were treated by shunt procedures or by microsurgical removal of the choroid plexus, which is associated with a high complication rate. In this paper the authors show that endoscopic plexus coagulation can result in restoring the equilibrium of the intracranial fluid volumes, resulting in shunt independency. In this way, both the shunt-related complications and the bleeding risks of microsurgical plexectomy are avoided. In instances of hydrocephalus, thorough efforts should be made to demonstrate the underlying pathophysiology to choose the optimal treatment, of which shunt procedures should receive the least priority.

Intracranial dural arteriovenous fistula successfully treated by combined open-endovascular procedure.

Intracranial dural arteriovenous fistulas constitute a rare though potentially devastating disease. Because the arterial (high-pressure) blood flow drains directly into the low-pressure venous system, there is a high risk of bleeding and associated neurological deficit. The classifications by Borden and Cognard underline the correlation between bleeding risk and venous drainage pattern of the fistula. There are different treatment options for this vascular pathology, which always poses a challenge for the physicians involved to offer the optimal treatment for an individual patient. This case report illustrates how combining forces between the neurosurgical and endovascular team benefits outcome. Simultaneously, this contributes to the growing amount of evidence that a new endovascular technique with transarterial ONYX embolisation enables complete obliteration of the vascular malformation.