Erdheim-Chester disease of the brain: cytological features and differential diagnosis of a challenging case.

Erdheim-Chester disease (ECD) is an uncommon, systemic xanthogranulomatous disorder, with distinct clinicopathological features, that is rarely expected preoperatively. We describe a case that presented in the brain of a 26-yr-old male patient and clinically mimicked the appearance of a neoplasm. The final diagnosis was a surprise.

From the archives of the AFIP: pilocytic astrocytoma: radiologic-pathologic correlation.

Pilocytic astrocytoma is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor. This tumor has a noteworthy benign biologic behavior that translates into an extremely high survival rate-94% at 10 years-that is by far the best of any glial tumor. Most patients present in the first 2 decades, and clinical symptoms and signs are usually of several months duration and directly related to the specific location of the tumor.

Human cerebral infarct: a proposed histopathologic classification based on 137 cases.

We studied the microscopic features of 137 cases of human cerebral infarct. In each case, the age of the lesion was determined by measuring the time elapsed between initial clinical presentation and date of surgery or death. Multiple microscopic variables were analyzed on hematoxylin and eosin-stained sections.

Erdheim-Chester disease mimicking a primary brain tumor. Case report.

Erdheim-Chester disease (ECD) is a rare systemic histiocytic disease. The authors present a case report detailing the presentation and treatment of a 26-year-old man diagnosed with seizures and a well-circumscribed temporoparietal mass that had been demonstrated on imaging studies. Both preoperative and intraoperative diagnoses were consistent with a low-grade astrocytic neoplasm.

Basic pathology of the peripheral nerve.

Peripheral nerve pathology encompasses a complex array of disease processes that are poorly understood. This article provides a substrate for communication between pathologists and radiologists who are involved in the diagnosis and treatment of patients with peripheral neuropathy. The article is organized into sections on normal histology, routine morphologic techniques used in the study of peripheral nerve, and the basic disease patterns, followed by a brief discussion of selected neuropathies.

Neuropathology of the brain and spinal cord in human West Nile virus infection.

Objective: To describe the histopathology of the brain and spinal cord in human West Nile virus (WNV) infection.

Materials And Methods: Single case report, including premortem clinical and laboratory findings, and autopsy.

Results: An 83-year-old female presented with acute confusion, high fevers, dysarthria and generalized subjective weakness, with decreased deep tendon reflexes and weakness on physical examination.

From the archives of the AFIP: medulloblastoma: a comprehensive review with radiologic-pathologic correlation.

Medulloblastoma is the most common pediatric central nervous system malignancy and the most common primary tumor of the posterior fossa in children. This highly malignant neoplasm occurs more frequently in males and usually before 10 years of age. Clinical symptoms and signs are generally brief, typically less than 3 months in duration, and reflect the strong predilection of this tumor to arise within the cerebellum, most often in the vermis.

Alpha-internexin expression in medulloblastomas and atypical teratoid-rhabdoid tumors.

Objective: To determine whether medulloblastomas and atypical teratoid/rhabdoid tumors express alpha-internexin, an intermediate filament protein that is expressed in normal neurons undergoing maturation and differentiation.

Materials And Methods: 28 medulloblastomas and 5 atypical teratoid/rhabdoid tumors were examined for the immunohistochemical expression of alpha-internexin, as well as the neuronal markers peripherin and synaptophysin, and glial fibrillary acidic protein.

Results: Overall, 21 of 28 medulloblastomas (75%) expressed alpha-internexin.

Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy.

We describe a case of anaplastic astrocytoma in a 14-year-old boy arising at the site of a dysembryoplastic neuroepithelial tumor (DNT) 3 years after combined radiation and chemotherapy. The subtotally excised superficial right temporoparietal tumor was originally diagnosed as mixed oligoastrocytoma in 1974; the patient was treated with radiation therapy postoperatively. One year later he underwent a craniotomy to remove cyst fluid and no change was reported in the size of the residual tumor.

Spinal cord malignant astrocytomas. Clinicopathologic features in 36 cases.

Background: Malignant astrocytomas of the spinal cord are uncommon neoplasms with a dismal prognosis. To the authors' knowledge, little information has been published to date regarding the prognostic impact of clinicopathologic factors.

Methods: The authors studied 36 cases to investigate the prognostic effect of the World Health Organization (WHO) tumor grade, tumor localization, cell proliferative activity, p53 expression, and therapy.

Widely invasive solitary fibrous tumor of the sphenoid sinus, cavernous sinus, and pituitary fossa.

Solitary fibrous tumor is a spindle cell tumor first described in the pleura, but also found in multiple extrathoracic sites including the meninges, orbit, nasal and paranasal sinuses. No cases have been previously reported in the cavernous sinus or pituitary fossa. We present the case of a 54-year-old woman who presented with progressive amaurosis.

Pediatric oligodendrogliomas: a study of molecular alterations on 1p and 19q using fluorescence in situ hybridization.

Oligodendrogliomas (OGs) are rare in children and have not been well characterized from a molecular viewpoint. In adults, losses on chromosomes 1p and/or 19q are common in "oligodendroglial" neoplasms and are highly associated with chemosensitivity and greater length of survival, especially in the anaplastic category. We have analyzed the 1p/19q status of pediatric OGs and compared it with similar alterations in adult OGs.

Infections of the nervous system.

This article surveys common causes and pathologic features of nervous system infections within the general population. Special consideration is given to infections in people with the HIV virus.

Ablation of NF1 function in neurons induces abnormal development of cerebral cortex and reactive gliosis in the brain.

Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology.

Characterization of initiated cells in N-methylnitrosourea-induced carcinogenesis of the CNS in the adult rat.

Glial tumors may originate from the malignant transformation of multipotent glial progenitor cells, but tools to study malignant transformation leading to gliomas are limited by the lack of biological systems that represent early stages of this disease in adult animals. In order to characterize the initiated cells that give rise to gliomas, we have employed the N-methylnitrosourea (MNU) model for induction of brain tumors in adult rats (Rushing et al., 1998).

Recurrent (nonfamilial) hemangioblastomas involving spinal nerve roots: case report.

Objective And Importance: Spinal nerve root hemangioblastomas are rare and are reported mainly in patients with von Hippel-Lindau (VHL) syndrome. The pathogenesis of so-called nonfamilial lesions is virtually unknown. We discuss, mainly from a molecular perspective, a unique patient with sporadic, recurrent hemangioblastomas restricted to spinal nerve roots.

Proliferative activity in craniopharyngiomas: clinicopathological correlations in adults and children.

Background: Craniopharyngiomas are slow-growing, locally invasive intracranial tumors that can generate considerable morbidity, and recurrences are often difficult to manage. Because reliable morphologic criteria for accurately predicting the clinical outcome of these tumors are lacking, we evaluated the growth potential of craniopharyngiomas by measuring their proliferative activity based on MIB-1 immunostaining for the Ki-67 antigen, which is expressed during all phases of the cell cycle except G(0.)

Methods: Paraffin sections from 37 cases of craniopharyngiomas were immunostained with the monoclonal antibody MIB-1, and a labeling index was derived in each case from an the with the highest labeling.

Prognostic value of proliferation index and expression of the RNA component of human telomerase (hTR) in papillary meningiomas.

Papillary meningioma is a rare subtype of meningioma that often behaves aggressively. In order to characterize factors that may influence this behavior, we chose to compare MIB-1 labeling index (LI) and telomerase RNA localization (hTR) in papillary meningiomas, meningiomas, and atypical meningiomas. LI is now often used to supplement histologic grade in the evaluation of these lesions.

Alpha-synuclein expression in central nervous system tumors showing neuronal or mixed neuronal/glial differentiation.

Alpha-synuclein (alpha-synuclein) is a member of a family of cytoplasmic proteins found predominantly and abundantly in the brain, and concentrated in pre-synaptic nerve terminals, near vesicles. We hypothesized that an antibody to alpha-synuclein could be a useful marker of neuronal differentiation in central nervous system (CNS) tumors. Twenty tumors known to have neuronal or mixed neuronal/glial differentiation ( 11 gangliogliomas, 2 anaplastic gangliogliomas, 5 gangliocytomas, and 2 ganglioneuroblastomas), 5 central neurocytomas, and 1 dysembryoplastic neuroepithelial tumor (DNET) were immunostained with a mouse monoclonal antibody raised against human alpha-synuclein.

Mouse tumor model for neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by increased incidence of benign and malignant tumors of neural crest origin. Mutations that activate the protooncogene ras, such as loss of Nf1, cooperate with inactivating mutations at the p53 tumor suppressor gene during malignant transformation. One hundred percent of mice harboring null Nf1 and p53 alleles in cis synergize to develop soft tissue sarcomas between 3 and 7 months of age.

Radiation change versus recurrent astrocytoma: diagnostic utility of the proliferation index?

Differentiation of recurrent glioma from radiation damage can be a challenge to neurologists, neurosurgeons, neuroradiologists, and even neuropathologists. We hypothesized that by evaluating sections of recurrent lesions with proliferation markers we might objectively differentiate between radiation damage gliosis and recurrent astrocytoma. We compared the labeling indices of radiation damage and recurrent neoplasm immunohistochemically, using an antibody to MIB-1, a monoclonal antibody to the Ki-67 proliferation marker.

Multidisciplinary approach to traumatic intracranial aneurysms secondary to shotgun and handgun wounds.

Background: Traumatic intracranial aneurysms (TICAs) may develop following gunshot injuries to the head. Management of these lesions often combines various aspects of microneurosurgical and endovascular techniques to safely repair or obliterate vessel defects.

Methods: We reviewed our experience over the last 18 years and identified five cases of intracranial aneurysms following gunshot and handgun wounds that were treated surgically and/or endovascularly.

Death due to a ruptured berry aneurysm in a 3.5-year-old child.

Berry aneurysms in childhood are rare and have certain characteristics that distinguish them from their adult counterparts. A case of death caused by a ruptured berry aneurysm in a child is presented, and the topic is reviewed.