Recurrent Primary Intrasellar Paraganglioma.

We describe a case of an 81-year-old male presenting with bitemporal visual field defects and blurry vision in the right eye. The patient was found to have a recurrent primary paraganglioma in the sellar and suprasellar region requiring a repeat transsphenoidal endoscopic resection. Immunohistochemical examination confirmed paraganglioma with the classic zellballen appearance which stained positive for chromogranin, synaptophysin, and S-100 in the periphery.

Ganglion cyst of the temporomandibular joint with intradural extension: case report.

Intracranial extension of temporomandibular joint (TMJ) ganglion cysts is very rare. Two previously reported cases presented clinically due to effects on cranial nerves and had obvious association with the TMJ on imaging. To the authors' knowledge, intracranial extension of a TMJ ganglion cyst presenting with seizures and mimicking a primary brain tumor has not been previously reported.

Detection of tau in Gerstmann-Sträussler-Scheinker disease (PRNP F198S) by [F]Flortaucipir PET.

This study aimed to determine the pattern of [F]flortaucipir uptake in individuals affected by Gerstmann-Sträussler-Scheinker disease (GSS) associated with the PRNP F198S mutation. The aims were to: 1) determine the pattern of [F]flortaucipir uptake in two GSS patients; 2) compare tau distribution by [F]flortaucipir PET imaging among three groups: two GSS patients, two early onset Alzheimer's disease patients (EOAD), two cognitively normal older adults (CN); 3) validate the PET imaging by comparing the pattern of [F]flortaucipir uptake, in vivo, with that of tau neuropathology, post-mortem. Scans were processed to generate standardized uptake value ratio (SUVR) images.

Cervical cancer metastasis to the brain: A case report and review of literature.

Background: Intracranial metastasis from cervical cancer is a rare occurrence.

Methods: In this study we describe a case of cervical cancer metastasis to the brain and perform an extensive review of literature from 1956 to 2016, to characterize clearly the clinical presentation, treatment options, molecular markers, targeted therapies, and survival of patients with this condition.

Results: An elderly woman with history of cervical cancer in remission, presented 2 years later with a right temporo-parietal tumor, which was treated with surgery and subsequent stereotactic radiosurgery (SRS) to the resection cavity.

Diffusion, Perfusion, and Histopathologic Characteristics of Desmoplastic Infantile Ganglioglioma.

We present a case series of a rare tumor, the desmoplastic infantile ganglioglioma (DIG) with MRI diffusion and perfusion imaging quantification as well as histopathologic characterization. Four cases with pathologically-proven DIG had diffusion weighted imaging (DWI) and two of the four had dynamic susceptibility contrast imaging. All four tumors demonstrate DWI findings compatible with low-grade pediatric tumors.

Morphological and molecular features of astroblastoma, including BRAFV600E mutations, suggest an ontological relationship to other cortical-based gliomas of children and young adults.

Background: Astroblastomas (ABs) are rare glial tumors showing overlapping features with astrocytomas, ependymomas, and sometimes other glial neoplasms, and may be challenging to diagnose.

Methods: We examined clinical, histopathological, and molecular features in 28 archival formalin-fixed, paraffin-embedded AB cases and performed survival analyses using Cox proportional hazards and Kaplan-Meier methods.

Results: Unlike ependymomas and angiocentric gliomas, ABs demonstrate abundant distinctive astroblastic pseudorosettes and are usually Olig2 immunopositive.

Disseminated choroid plexus papillomas in adults: A case series and review of the literature.

Choroid plexus papillomas (CPPs) are uncommon, usually intraventricular, low-grade tumors, accounting for less than 1% of all intracranial neoplasms and 2-4% of brain tumors in children. Dissemination of CPPs to multiple levels of the neuraxis has been seldom observed. Thus far, only 26 adult patients have been reported in the English language literature.

Ciliochoroidal ganglioneuroma in neurofibromatosis type 1: Report of a case and review of the literature.

Orbitofacial neurofibromatosis (OFNF) is considered a variant of neurofibromatosis type 1 (NF1). OFNF most often affects the eye, orbit and one side of the face. It is characterized by the development of relatively aggressive and disfiguring lesions, including plexiform and diffuse neurofibromas.

Clinical, Pathological, and Surgical Outcomes for Adult Pineoblastomas.

Introduction: Pineoblastomas are uncommon primitive neuroectodermal tumors that occur mostly in children; they are exceedingly rare in adults. Few published reports have compared the various aspects of these tumors between adults and children.

Methods: The authors report a series of 12 pineoblastomas in adults from 2 institutions over 24 years.

Epithelioid Glioblastomas and Anaplastic Epithelioid Pleomorphic Xanthoastrocytomas--Same Entity or First Cousins?

Epithelioid glioblastoma (eGBM) and pleomorphic xanthoastrocytoma (PXA) with anaplastically transformed foci (ePXA) show overlapping features. Eleven eGBMs and 5 ePXAs were reviewed and studied immunohistochemically. Fluorescence in situ hybridization for EGFR amplification, PTEN deletion and ODZ3 deletion was also performed, with Ilumina 450 methylome analysis obtained in five cases.

Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review.

Background: Chordomas are rare, slow-growing malignant neoplasms derived from remnants of the embryological notochord. Pediatric cases comprise only 5% of all chordomas, but more than half of the reported pediatric chordomas are intracranial. For patients of all ages, intracranial chordomas typically present with symptoms such as headaches and progressive neurological deficits occurring over several weeks to many years as they compress or invade local structures.

Intrasellar ependymoma: clinical, imaging, pathological, and surgical findings.

Ependymomas arising in the intrasellar compartment are extremely rare and most often are not included in the differential diagnosis of an intrasellar tumor mass. We review the literature to further advance awareness regarding unusual presentations of this type of tumor and present an illustrative case of an intrasellar cystic ependymoma that developed in an uncommon location. In our illustrative case, the patient had a 2 year history of hypopituitarism, but no headaches or visual disturbance.

Intrasellar pilocytic astrocytomas: clinical, imaging, pathological, and surgical findings.

A pilocytic astrocytoma is not usually considered in the differential diagnosis of an intrasellar tumor. An awareness of this tumor as primarily an intrasellar entity is important to avoid confusion during its diagnosis. We retrospectively examined the records of 631 patients treated at our institution between 2006 and 2010 who underwent transsphenoidal resection of pituitary tumors and identified those diagnosed with pilocytic astrocytoma.

Rathke's cleft cysts in twins with type 2C von Hippel-Lindau disease.

Von Hippel-Lindau disease (VHLD) is characterized by a spectrum of benign and malignant tumors in the CNS and visceral organs. Rathke's cleft cysts are benign, nonneoplastic sellar lesions that are often asymptomatic. The authors report the case of twin sisters with VHLD Type 2C with radiographically similar sellar lesions.

A case of natalizumab-associated progressive multifocal leukoencephalopathy with repeated negative CSF JCV testing.

The development of progressive multifocal leukoencephalopathy (PML) in patients treated with natalizumab is a well-known potential risk. Diagnosis of PML can be confounded in patients with multiple sclerosis (MS) if new demyelinating lesions develop, and the sensitivity of existing diagnostic tests is less than ideal. In the case presented here, four samples of cerebrospinal fluid tested negative for John Cunningham virus (JCV) DNA by polymerase chain reaction, yet brain biopsy eventually proved positive by immunohistochemistry.

T-cell lymphoblastic lymphoma/leukemia presenting as a pituitary mass lesion: a case report and review of the literature.

We present a rare case of primary T-cell lymphoblastic lymphoma of the pituitary gland. A 58-year-old woman presented with headaches, right-sided ptosis and cranial nerve III palsy. She subsequently developed polyuria, polydipsia, and hyperglycemia and was found to have hypopituitarism.

Intracranial Masson tumor: case report and literature review.

Intravascular papillary endothelial hyperplasia (IPEH) or Masson tumor has only been reported intracranially in 20 cases and can present as a congenital finding. This pathologic entity is an important diagnostic consideration when evaluating an infant with a congenital intracranial mass. We report a third case of a neonate who presented with the appearance of a metastatic brain tumor involving the orbit, sella, and cerebellum that was ultimately proven to be IPEH.

A 2-year-old boy with hemolytic uremic syndrome and pneumocephalus.

Clostridium septicum infection following hemolytic uremic syndrome is rare and carries a poor prognosis, especially when the brain is involved. We report a case of a previously healthy 2-year-old boy who presented with two days of anuria and bloody diarrhea. He was admitted to the local children's hospital with a diagnosis of hemolytic uremic syndrome, presumably secondary to E.

Angiographically visible and invisible arteriovenous malformation in the same patient.

A pathologically confirmed angiographically visible and invisible arteriovenous malformation in the same patient is described. The potential clinical significance of these observations is detailed and discussed.

Anaplastic astroblastoma presenting as massive, sudden-onset, intraparenchymal hemorrhage.

Astroblastoma is a rare primary glial tumor of children and young adults, typically located in the cerebral hemispheres and presenting as a well-circumscribed, nodular, often cystic enhancing mass. The existence of astroblastoma as a distinct clinicopathologic entity has long been debated but is recognized in the 2007 WHO classification of CNS tumors. The grading of these tumors remains unsettled.

Minimally invasive resection of an extrapelvic sciatic schwannoma.

Schwannomas associated with the sciatic nerve are relatively rare. Surgical excision of symptomatic sciatic schwannomas traditionally involves a posterior sciatic nerve approach with a large, open exposure and transection of the gluteal muscles. The authors provide a technical report of a minimally invasive approach for the resection of an extrapelvic schwannoma arising from the sciatic nerve.

Focal genomic amplification at 19q13.42 comprises a powerful diagnostic marker for embryonal tumors with ependymoblastic rosettes.

Ependymoblastoma (EBL) and embryonal tumor with abundant neuropil and true rosettes (ETANTR) are very aggressive embryonal neoplasms characterized by the presence of ependymoblastic multilayered rosettes typically occurring in children below 6 years of age. It has not been established whether these two tumors really comprise distinct entities. Earlier, using array-CGH, we identified a unique focal amplification at 19q13.

Glioblastoma with signet-ring morphology: a case report and review of the literature.

Primary central nervous system tumors with signet-ring morphology are exceedingly rare. We report an unusual case of glioblastoma with signet-ring cell features in an 81-year-old woman. Microscopic examination revealed a highly anaplastic tumor, with a prominent proportion of tumor cells exhibiting signet-ring appearance characterized by classic round cytoplasmic inclusions and eccentrically positioned nuclei.

Modified anterior capsulotomy technique and histopathology of the anterior capsule in cataracts after prolonged exposure to intravitreal silicone oil.

Cataract development is common in phakic eyes after pars plana vitrectomy and exposure to silicone oil. Anterior capsule rigidity is frequently encountered during cataract surgery in these cases. We report a capsulotomy technique to overcome capsule rigidity, as well as the histopathological findings of the anterior capsule after prolonged exposure to silicone oil.

Clear-cell ependymoma: a report of intracortical tumor with significant desmoplasia.

We report a case of clear-cell ependymoma in a 20-month-old girl who presented with seizures. The tumor had several uncommon features including a solid consistency, a purely intracortical location, and a remarkable degree of desmoplasia. Clinical, radiological, and pathological findings are presented.