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Brain tumors represent the second most frequent etiology in patients with focal seizure onset before 18 years of age and submitted to epilepsy surgery. Hence, this category of brain tumors, herein defined as low-grade, developmental, epilepsy-associated brain tumors (LEAT) is different from those frequently encountered in adults as (A): 77% of LEAT occur in the temporal lobe; (B): the vast majority of LEAT are of low malignancy and classified as WHO I°; (C): LEAT are often composed of mixed glial and neuronal cell components and present with variable growth patterns including small cysts or nodules; (D): LEAT do not share common gene driving mutations, such as IDH1 or 1p/19q co-deletions. Characteristic entities comprise the ganglioglioma (GG), the dysembryoplastic neuroepithelial tumor (DNT), the angiocentric glioma (AG), the isomorphic diffuse glioma (IDG) and the papillary glio-neuronal tumor (PGNT), representing 73.2% of 1680 tumors collected in a large German series of 6747 patients submitted to epilepsy surgery. In the realm of exciting discoveries of genetic drivers of brain tumors new genes have been also reported for LEAT. BRAF V600E mutations were linked to GG with CD34 expression, FGFR1 mutations to DNT, MYB alterations to AG and also IDG and PRKCA fusions to PGNT, suggesting the possibility to also develop a genetically driven tumor classification scheme for LEAT. Rare availability of LEAT in a single center is a challenging obstacle, however, to systematically unravel the neurobiological nature and clinical behavior of LEAT. Other challenges in need of clarification include malignant tumor progression of LEAT entities, seizure relapse in patients following bulk tumor resection and the controversial issue of associated focal cortical dysplasia as additional pathomechanism. In order to advance our understanding and promote reliable diagnostic work-up of LEAT, we recommend, therefore, international collaboration to achieve our goals.

[This corrects the article DOI: 10.1371/journal.pone.0209007.].

ObjectivesTo study the presentation, management and outcomes of symptomatic intracranial arachnoid cysts.DesignRetrospective records review.SubjectsAll cases coded as intracranial cysts.MethodsPatients admitted between Jan-2012 and Sep 2017. Of 56 cases, only 24 were arachnoid cysts. Median age 57 (range 8–81). Mean 49.1±5.0 years, 8 males and 16 females.ResultsMales were significantly younger (34.3±9.1 vs 56.5±5.2 year-old, p=0.03) and outnumbered females. Mean size of cysts was 58.8±6.1 mm (range 18.5–126 mm). Five were located frontally, one fronto-temporal and another fronto-parietal, two parietal and two parieto-occipital, two within third ventricle, one intraventricular, one supra sellar and one intra sellar while eight were posterior fossa. 12 patients had headache, five presented with cognitive and memory issues, five had visual problems, four with limb weakness and two had cerebellar signs. Eight had open fenestration, five endoscopic fenestration, four had aspiration with reservoir, two aspiration only, two marsupialisation, two received ICP monitor and one had no treatment. One reservoir was aspirated three times and another open fenestration had another surgical fenestration. Two had transient infection, two needed VP shunts and one had cysto-peritoneal shunt. mRS (0–2) improved significantly after the treatment (62.5% pre-surgery to 91.7% post-surgery, p=0.016).ConclusionsSymptomatic arachnoid cysts are more common in young males. In the literature, the most common location is middle cranial fossa which is not the case in symptomatic arachnoid cysts as in our series. Although intervention is variable, they are associated with very good outcomes.

ObjectivesThe first documented use of 5-ALA in the excision of an intraparenchymal endodermal cyst.DesignCase review.Subjects52 year old female presented with headaches, progressive right sided hemiparesis and anomic aphasia. She had a large cystic frontal lesion, adjacent of the frontal horn of the lateral ventricles cyst which did not show any nodular or solid component, and did not enhance with gadolinium.MethodsCase report and review of the literature.Results5-ALA assisted, image guided fenestration of the cystic lesion and insertion of a ventricular catheter connected to an Ommaya reservoir was performed. The cyst wall was observed under the microscope and it fluoresced with 5-ALA. Multiple biopsies were obtained from this tissue, which confirmed diagnosis of endodermal cyst. The patient had improvement in clinical symptoms and size of the cyst radiologically in both the immediate post-operative period and the 6 months follow up.ConclusionsPrevious reports described the use of 5-ALA to identify tumour cells within the solid component of cystic haemangioblastomas as well as in cystic ependymomas where the cystic component does not fluoresce. In our patient, the homogeneous fluorescence of the wall suggested that this was indeed a different entity from a haemangioblastoma or an ependymoma because of the different pattern of fluorescence observed. 5-ALA-assisted resection of cystic lesions should be considered when a tumoral origin is uncertain.

ObjectivesPineal cysts (PCs) may be incidentally found on intracranial imaging. They are typically quiescent, benign and asymptomatic. However, imaging alone cannot exclude an underlying pineal malignancy. This study reviewed our hospital’s experience of managing PCs in order to develop a clinical pathway to guide their investigation and follow-up.DesignSingle-centre retrospective review of cases.SubjectsPatients referred to our hospital with a PC.MethodsCase notes and imaging from patients referred to our hospital with a clinically-coded ‘pineal cyst’ between 2007 and 2017 were reviewed.Results43 cases were included. The most common indication for intracranial imaging was headache (12/43), followed by seizures (4/43). No symptoms were attributable to the PC. Follow-up imaging to monitor the PC was performed in 13/43 cases; in a further 10/43 cases, it was carried out for an indication unrelated to the PC. Follow-up was arranged by the neurosurgical team in 12 cases. Uncertainty about the diagnosis led to an endoscopic biopsy in one case, which confirmed a PC. There was no consistent clinical rationale underlying the decision to undertake follow-up imaging, nor its interval or duration.ConclusionsClinical practice varied widely for the management of incidental PCs. We propose that all PCs should be investigated with standardised MRI, reported by a neuroradiologist and repeated at 6–12 months. Further follow-up and its duration should be guided by the patient’s clinical condition and the radiological features of the PC.

Introduction: Pancreatic dermoidcyst is a benign germ cell tumor. It continues to be a rare entity with 50 cases described in the literature. Mostly, patients presented with vague symptoms. The pre-operative diagnosis is tough despite the imaging and EUS-guided aspiration. Accordingly, the surgical intervention is usually required for definite diagnosis. Case Description: A 50-year-old female presented with chronic epigastric abdominal pain for 6 months. The pain was worse with food. There was no change in bowel movement or weight. Ultrasound showed pancreatic head mass. CT scan showed solid mass contiguous with the lateral pancreatic neck. Other work-up showed elevated CA 19-9 of 1973, elevated chromogranin of 133, whereas CEA and CA-125 were within normal limit. MR showed 2.6 x 2.2 x 1.8 cm circumscribed mass arising exophytically from pancreas, hyperintensity on T1/T2/ diffusion-weighted images. EUS-guided aspiration showed squamousand lymphocytic-lined cyst (Figure 1). Patient underwent laproscopic removal of pancreatic mass. Pathology revealed benign cystic teratoma (Figure 2). The presence of a cyst filled with keratin and lined by mature keratinizing squamous epithelium supported by lymphoid tissue broadly establishes this lesion as a form of lymphoepithelial cyst; the presence of skin appendages and mature mesenchymal elements is diagnostic for cystic teratoma, no malignacy. Discussion: Cystic pancreatic lesions encompass a wide spectrum of non-neoplastic, benign, and malignant neoplasms. This lesion can be characterized by imaging and EUS - guided fine needle aspiration. However, often, the result was indeterminate as to whether the lesion was benign or malignant.Surgical resection remains the standard of therapy.

A 63-year-old female with a history of chronic back pain presented to the pulmonary clinic for evaluation of an occasional non-productive cough and shortness of breath. Conservative treatment, sclerotherapy, pleural and pericardial drainage, pleurectomy, radiation, chemotherapy, and embolization of lesions have been described.

Fluid analysis revealed high white blood cell count but negative culture for other infectious organisms. Infection with this organism is not commonly seen in the Western world but clinicians should remember to obtain a complete history and keep it in the differential diagnosis for high risk patients.