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      Pilocytic Astrocytoma Presenting as an Orbital Encephalocele: A Case Report

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          We describe the case of a 29-year-old male who presented with new-onset seizures. He was subsequently found to have an orbital encephalocele containing a focus of pilocytic astrocytoma. We believe that this is the first report of a pilocytic astrocytoma located within the orbit that did not originate from the optic pathway. It is also the first case of a pilocytic astrocytoma completely contained within an encephalocele. This case suggests a close pathological examination of encephaloceles for underlying diseases.

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          Most cited references 14

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          Frequent recurrence and progression in pilocytic astrocytoma in adults.

          Most pilocytic astrocytomas (piloA) are benign growths (World Health Organization [WHO] grade 1) of the deep midline structures, the brainstem, and the cerebellum. To the authors' knowledge, the literature contains only scarce data regarding piloA in adults. Between 1995 and 2005, 44 patients (26 women and 18 men) underwent surgery for a primary or recurrent piloA at the authors' institution. All patients were aged > 16 years (mean +/- standard deviation: 31 +/- 14 years) at the time of their first surgery. The histopathologic diagnoses were reviewed, and relevant clinical information was obtained through a chart review and telephone interviews. The mean follow-up was 76 +/- 59 months (range, 1-227 months). There were 20 patients (45%) with supratentorial lobar piloA (including 10 temporal/temporomesial tumors, 5 parietal tumors, 3 insular tumors, 1 frontal tumor, and 1 occipital tumors), 12 patients with cerebellar piloA, 7 patients with brainstem piloA, 2 patients with opticochiasmatic PiloA, 1 patient with intramedullary piloA, and 2 patients with piloA of the basal ganglia. All but 1 patient with a lobar tumor presented with epilepsy. In 6 of 44 patients (14%), increased proliferative activity was revealed. WHO grade 3 primary anaplastic piloA was diagnosed in 2 patients (5%), and WHO grade 3 secondary anaplastic piloA was diagnosed in 4 patients (9%). Tumor recurrence or disease progression was observed in 13 of 44 patients (30%). Eight of 44 patients (18%) died from their disease. Histologic grading and extent of surgical resection proved to be important predictors of survival. PiloA in adult patients, surprisingly, often was not a benign disease. The degree of surgical resection was found to be of major importance for the patient's further clinical course; therefore, an aggressive surgical resection should be attempted whenever possible.
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            Traumatic encephalocele related to orbital roof fractures: report of six cases and literature review.

            Orbital roof fractures after blunt injury are rare. Traumatic encephaloceles in the orbital cavity are even rarer, with only 15 cases published to date. The clinical, radiological, and surgical findings of 6 cases of traumatic encephalocele treated at our institution from June 1998 to January 2000 are presented. They are also compared with previously published series. In contrast to other published cases, 5 out of 6 patients in our series were adults. The most common cause of trauma was road traffic accident. Ecchymosis and preoperative exophthalmos/proptosis were frequent. In all of our patients a coronal CT scan (3 mm increments with bone windows) was obtained. It demonstrated the extension of the orbital roof fractures and a possible encephalocele in 4 cases. Associated frontal brain contusions were seen in 5 cases. An MRI was performed in 3 patients (and only in 2 previously published cases); it showed the extension of the brain herniation into the orbital cavity. Surgical treatment via a fronto-basal approach with evacuation of the contused herniated brain tissue and orbital roof reconstruction was performed. The outcome at 6 months was good recovery in five patients with one patient still in a persistent vegetative state. Postoperatively the ocular disturbances improved in 5 cases. A review of the other published cases confirmed recovery of normal ocular function in the vast majority of the cases. Whenever orbital roof fractures associated with frontal contusions are identified in an acute brain injured patient, an orbital encephalocele should be suspected. In our opinion MRI is the investigation of choice in such patients. If the encephalocele is confirmed, a surgical approach via the subfrontal route is indicated with resection of herniated contused brain tissue, dural closure, and orbital roof reconstruction. Good results in regard to the orbital symptoms (mainly exophthalmos) can be expected.
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              A 10 year retrospective study of surgical outcomes of adult intracranial pilocytic astrocytoma.

              Pilocytic astrocytomas (PA) are benign neoplasms commonly located in the cerebellum with a peak incidence in the first two decades of life. PA occurrence in adults is rare and very little information is available in the literature about tumour characteristics in this population. This study retrospectively identified 20 adults with PA. The characteristics of the tumour, treatment modalities and patient outcomes are discussed, as well as identifying factors that may be associated with worse prognosis. The mean age at diagnosis was 27 years. The majority of PA were located in the posterior fossa. Other tumour locations included the cerebral hemispheres, brainstem, tectal plate and optochiasmatic region (optic chiasm, hypothalamus and third ventricle). All patients in this study underwent surgery, two received adjuvant chemotherapy and one received adjuvant radiotherapy. Tumour recurrence occurred in six patients and two eventually died from the disease. When achieved, complete tumour resection was found to be curative. Tumour location affects extent of surgical resection; tumours in inaccessible locations were associated with higher rates of recurrence. Overall survival and progression free survival rates were 87% and 60% respectively. The degree of surgical resection and tumour location were found to affect prognosis. Unfavourable outcomes were observed in these adults with PA compared to those expected for a younger population, suggesting a possible association between age and outcome.

                Author and article information

                Case Reports in Neurology
                S. Karger AG
                January – April 2015
                23 April 2015
                : 7
                : 1
                : 90-94
                aVirginia Commonwealth University, Richmond, Va., bMayo Clinic and Departments of cNeurosurgery and dAnatomic Pathology, Mayo Clinic, Rochester, Minn., USA
                Author notes
                *Jamie J. Van Gompel, Department of Neurosurgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905 (USA), E-Mail
                381943 PMC4448056 Case Rep Neurol 2015;7:90-94
                © 2015 S. Karger AG, Basel

                Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (, applicable to the online version of the article only. Distribution permitted for non-commercial purposes only. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                Figures: 3, References: 10, Pages: 5
                Published: April 2015


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