Trigeminal nerve hemangioblastoma in the setting of undiagnosed von Hippel-Lindau disease: illustrative case.

Background: Von Hippel-Lindau disease (VHL) is an autosomal dominant tumor predisposition syndrome caused by mutations in the VHL gene. Patients with VHL are predisposed to developing numerous neoplasms, including central nervous system hemangioblastomas that typically arise within the cerebellum, brainstem, or spinal cord. The authors present the unusual case of a 69-year-old patient with a hemangioblastoma of the trigeminal nerve as his initial presentation of VHL.

Rare case of extracranial chordoid meningioma adjacent to the carotid sheath: illustrative case.

Background: Chordoid meningioma is a rare World Health Organization (WHO) grade 2 variant of meningioma with histological features resembling those of a chordoma. This tumor type is known for having an aggressive clinical course with a propensity for local recurrence. Most cases occur within the cranium, more specifically around the cerebral convexities.

Overlapping Immunohistochemical Features of Adenocarcinoma of the Nonpigmented Ciliary Body Epithelium and Renal Cell Carcinoma.

Purpose: To find immunohistochemical markers that distinguish adenocarcinoma of the nonpigmented ciliary epithelium (NPCE) from metastatic carcinoma, especially metastatic renal cell carcinoma.

Design: Retrospective case series.

Methods: Three cases of adenocarcinoma of the NPCE were examined histologically with hematoxylin-eosin stain and immunohistochemical stains including vimentin, AE1/AE3, Cam 5.

Follicular lymphoma presenting as scalp mass deformity: Case Report and Review of the literature.

Lymphoma presenting as a scalp mass is a rare but serious medical condition mandating aggressive treatment and neurosurgical intervention. We report a case of 53-year-old male who presented with a large right sided frontal scalp mass and a smaller mass located on the left frontal scalp. After discussion with the patient, it was decided to resect the larger mass for definitive diagnosis.

Ocular Human Papillomavirus Infections.

Context: - Human papillomavirus (HPV) has a well-known role in the pathogenesis of squamous cell carcinoma and precursor lesions of the cervix, anogenital region, and head and neck, but its role in the development of squamous neoplasms of the eye, particularly the conjunctiva, remains unclear.

Objective: - To review recent evidence implicating HPV in the pathophysiology of ocular lesions.

Data Sources: - Published articles obtained from a PubMed search of the English literature were the primary sources for this review.

Glioblastoma Survival Outcomes at a Tertiary Hospital in Appalachia: Factors Impacting the Survival of Patients Following Implementation of the Stupp Protocol.

Background: Glioblastoma is a fatal brain cancer with low median and yearly survival rates. Standard of care for treating glioblastoma is gross total resection (GTR) coupled with the Stupp protocol, but various factors influence the interventions undertaken and survival achieved. As health disparities exist in rural areas, survival in these areas needs to be assessed to understand which factors detract from the successes of these standard medical interventions.

Biallelic PMS2 Mutation and Heterozygous DICER1 Mutation Presenting as Constitutional Mismatch Repair Deficiency With Corpus Callosum Agenesis: Case Report and Review of Literature.

Constitutional mismatch repair deficiency syndrome is a cancer predisposition syndrome caused by autosomal recessive biallelic (homozygous) germline mutations in the mismatch repair genes (MLH1, MSH2, MSH6, and PMS2). The clinical spectrum includes neoplastic and non-neoplastic manifestations. We present the case of a 7-year-old boy who presented with T-lymphoblastic lymphoma and glioblastoma, together with non-neoplastic manifestations including corpus callosum agenesis, arachnoid cyst, developmental venous anomaly, and hydrocephalus.

A Review of Vascular Abnormalities of the Spine.

Patients with spinal vascular lesions present with unique symptoms and have important anatomical and physiologic changes that must be considered prior to treatment. In this mini-review, we provide an overview of normal spinal vascular anatomy and discuss several key spinal vascular lesions. We provide an overview of cavernous malformations, intradural arteriovenous malformations, perimedullary arteriovenous fistulas, and dural arteriovenous fistulas.

Neurosarcoidosis: unusual presentations and considerations for diagnosis and management.

Background: Sarcoidosis is a chronic, multisystem disease characterised by non-necrotising granulomatous inflammation of unknown aetiology. Most commonly, the lungs, lymph nodes, skin and eyes are affected in sarcoidosis; however, nervous system involvement occurs in approximately 5%-15% of cases. Any part of the nervous system can be affected by sarcoidosis.

Lipomatous meningioma: Case report and review of the literature.

Lipomatous meningiomas are a very rare form of brain meningiomas consisting of fat accumulation within the tumor. Magnetic resonance imaging (MRI) or computerized tomographic (CT) imaging can be utilized to visualize the fat accumulations, but histopathologic staining is necessary in order to make a definitive diagnosis. The key histopathologic feature is the identification of adipocyte-like cells within the tumor, but other markers have also been identified.

Giant calvarial intraosseous angiolipoma: a case report and review of the literature.

Intraosseous angiolipomas are very rare tumors occurring most commonly in the ribs and mandible. Only two cases with intracranial involvement have been reported in the literature. We report a case of a giant calvarial angiolipoma and its surgical treatment in a 30-year-old female who presented with a slowly expanding skull mass and discuss relevant radiological, histological and surgical findings.

An unusual form of listerial CNS infection.

Listeria monocytogenes is a known cause of meningitis, but cerebral abscess formation is exceedingly rare. We describe a patient who presented with an unsteady gait and a small ring-enhancing lesion in the right parietal lobe. Pathologic evaluation demonstrated an abscess with associated microglial nodules, and culture revealed Listeria.

West Nile virus infections.

Since its introduction to the United States in 1999, West Nile virus (WNV) has become endemic in North America and has emerged as the most common cause of epidemic meningoencephalitis in North America and the leading cause of arboviral encephalitis in the United States. West Nile virus is maintained in nature by cycling between mosquito vectors and bird hosts; humans are incidental hosts. Transmission to humans occurs predominantly after a bite from an infected mosquito but has also occurred via transfusion of blood products, via organ transplantation from infected donors, transplacentally, and percutaneously through occupational exposure.

Interobserver and intraobserver reproducibility in focal cortical dysplasia (malformations of cortical development).

Purpose: Malformations of cortical development (MCD) (cortical dysplasias) are well-recognized causes of intractable epilepsy. Although a histologic classification system for MCD has been proposed by Palmini et al. (Neurology; 2004; 62:S2), studies to date have not assessed reproducibility.

Vital signs: how early can resident evaluation predict acquisition of competency in surgical pathology?

Objectives: To determine whether a longitudinal, case-based evaluation system can predict acquisition of competency in surgical pathology and how trainees at risk can be identified early.

Design: Data were collected for trainee performance on surgical pathology cases (how well their diagnosis agreed with the faculty diagnosis) and compared with training outcomes. Negative training outcomes included failure to complete the residency, failure to pass the anatomic pathology component of the American Board of Pathology examination, and/or failure to obtain or hold a position immediately following training.

Systemic distribution of West Nile virus infection: postmortem immunohistochemical study of six cases.

Rare cases of West Nile virus (WNV)-associated inflammation outside the central nervous system (CNS) have been reported. We evaluated the systemic distribution of WNV in postmortem tissues during encephalitis in six patients using immunohistochemistry. WNV antigens were detected in neurons of CNS (all 6 cases), kidney (4 cases), lungs (2 cases), pancreas (2 cases), thyroid (2 cases), intestine (2 cases), stomach (1 case), esophagus (1 case), bile duct (1 case), skin (1 case), prostate (1 case) and testis (1 case).

Intranasal administration of the growth-compromised HSV-2 vector DeltaRR prevents kainate-induced seizures and neuronal loss in rats and mice.

Identification of targets and delivery platforms for gene therapy of neurodegenerative disorders is a clinical challenge. We describe a novel paradigm in which the neuroprotective gene is the herpes simplex virus type 2 (HSV-2) antiapoptotic gene ICP10PK and the vector is the growth-compromised HSV-2 mutant DeltaRR. DeltaRR is delivered intranasally.

Newly defined central nervous system neoplasms.

In recent years, numerous new entities or variants of recognized central nervous system tumors have been described in the literature, and the morphologic spectrum of these neoplasms is delineated incompletely. The accurate diagnosis and classification of these lesions is important to ensure that patients receive adequate therapy and prognostic information. The clinicopathologic features and differential diagnosis of 4 new entities, including the chordoid glioma of the third ventricle, cerebellar liponeurocytoma, atypical teratoid/rhabdoid tumor, and papillary glioneuronal tumor, are discussed in this review.

Temporal lobe intraparenchymal retained foreign body from remote orbital trauma.

We present the case of a 35-year-old man with a new onset of seizure disorder who was found to harbor an intraparenchymal retained foreign body related to remote orbital trauma. Imaging revealed a rim-enhancing anterior medial temporal lobe mass. Histologic evaluation of the resected mass showed evidence of acute and chronic inflammation with associated foreign material.