Improving care for central nervous system tumours: a mood for change (original) (raw)

An Overview of Central Nervous System Tumours

SciMedicine Journal, 2021

Central nervous system (CNS) tumours refer to tumours that occur in the tissues of the brain and/or spinal cord. These tumours arise as a result of abnormal growth of cells and may begin in different parts of the brain or spinal cord. There are many types of CNS tumours, which are further divided into subtypes. Despite decades of research conducted, CNS tumours remain among the deadliest of all cancers. It is most often challenging to treat these tumours, due to the risks involved, and biological characteristics associated with them. The classification, grading, and characterisation of CNS tumour plays a pivotal role in the management thereof. The current review provides an overview of CNS tumours, classification, grading and treatment, as well as their characterisation with specific focus on gliomas, ependymomas, oligodendrogliomas, meningiomas, medulloblastomas, schwannomas, gangliogliomas, and craniopharyngiomas. Doi: 10.28991/SciMedJ-2021-0304-8 Full Text: PDF

Tumors of the Central Nervous System

Annals of Internal Medicine, 1953

Tumors that present in infancy tend to be more insidious because of the nonspecifi c nature of the clinical symptoms, including vomiting, irritability, lethargy, macrocephaly, failure to thrive, and loss of, or delay in, attaining developmental milestones [4,6]. Older children may better communicate specifi c neurologic deficits. Additionally, signs and symptoms related to increased ICP, including headache, nausea, and vomiting (particularly upon awakening in the morning), frequently occur in this age group as well [4,6]. Supratentorial tumors produce signs and symptoms according to the area of the brain that is affected [4,6]. For example, cerebral hemispheric lesions may present with focal neurologic fi ndings or seizures, whereas tumors proximal to the optic chiasm and hypothalamus may produce vision loss, visual fi eld defects, or endocrine abnormalities [6]. Cerebellar tumors, on the other hand, frequently result in ataxia, gait disturbances, and signs of increased ICP secondary to obstruction of the fourth ventricle [4,6]. Brain stem tumors present with cranial nerve abnormalities and/or upper motor neuron signs [4,6]. Signs and symptoms associated with specifi c tumor types are discussed in more detail in the following sections. Gliomas Tumors of glial origin constitute approximately 50% of all primary brain tumors in children and are grouped based on histopathologic appearance into low-grade and high-grade gliomas [4,6,8,9]. These tumors are found throughout the central nervous system, and location is an important prognostic factor [3,10]. Low-grade gliomas are a heterogeneous group of tumors with an overall 10-year survival rate of greater than 80% with appropriate treatment [3,11]. The most frequent low-grade gliomas are posterior fossa and cerebral hemisphere astrocytomas. Most low-grade gliomas are classifi ed into two histopathologic types: pilocytic astrocytomas (World Health Organization, WHO grade I) and diffuse or fi brillary astrocytomas (WHO grade II) [3]. Pilocytic astrocytomas occur primarily in young children with a median age of 4 years [3]. These tumors can occur at all levels of the neuroaxis but occur most frequently in the cerebellum and the optic pathways [12]. On radiographic imaging, nearly all are brightly enhancing, well-circumscribed tumors that are clearly demarcated from surrounding brain tissue and have little surrounding edema; about half of them are cystic [3,12]. In contrast,

Tumors of the Central Nervous System: An Update

Cancers

The brain may be affected by a variety of tumors of different grade, which originate from different cell types at distinct locations, thus impacting on the brain structure and function [...]

R EV IE W The 2007 WHO ClassiWcation of Tumours of the Central Nervous System

The fourth edition of the World Health Organization (WHO) classiWcation of tumours of the central nervous system, published in 2007, lists several new entities, including angiocentric glioma, papillary glioneuronal tumour, rosette-forming glioneuronal tumour of the fourth ventricle, papillary tumour of the pineal region, pituicytoma and spindle cell oncocytoma of the adenohypophysis. Histological variants were added if there was evidence of a diVerent age distribution, location, genetic proWle or clinical behaviour; these included pilomyxoid astrocytoma, anaplastic medulloblastoma and medulloblastoma with extensive nodularity. The WHO grading scheme and the sections on genetic proWles were updated and the rhabdoid tumour predisposition syndrome was added to the list of familial tumour syndromes typically involving the nervous system. As in the previous, 2000 edition of the WHO 'Blue Book', the classiWcation is accompanied by a concise commentary on clinico-pathological characteristics of each tumour type. The 2007 WHO classiWcation is based on the consensus of an international Working Group of 25 pathologists and geneticists, as well as contributions from more than 70 international experts overall, and is presented as the standard for the deWnition of brain tumours to the clinical oncology and cancer research communities world-wide.

Glial differentiation predicts poor clinical outcome in primitive neuroectodermal brain tumors

Annals of Neurology, 1996

Primitive neuroectodermal tumors (PNETs) of the central nervous system, including medulloblastomas (PNET/MB), are the most common malignant brain tumor of childhood. These tumors often express proteins characteristic of glial differentiation (glial fibrillary acidic protein, GFAP), neuronal differentiation (neurofilament proteins, NFPs), and/or photoreceptor differentiation (retinal-s antigen). To identify biological factors of prognostic significance in PNETs, the expression of glial, neuronal, or photoreceptor antigens was evaluated in the tumor specimens of 86 patients with PNETs by immunohistochemistry after microwave antigen enhancement. Patterns of differentiation were then compared with patient relapse-free survival. Multivariate analysis of PNET immunohistochemistry and clinical variables indicated GFAP expression conferred a 6.7-fold greater risk of relapse than tumors that did not express GFAP or NFPs. Increased risk of relapse was directly related to the amount of GFAP expression. Tumors exhibiting clumps or sheets of GFAP-staining cells were associated with a 3.0-fold increased risk of relapse compared with tumors that did not express GFAP, irrespective of immunohistochemical evidence of other differentiation, while scattered GFAP staining was not associated with increased risk of relapse. These findings indicate that expression of GFAP in PNETs has prognostic power comparable with the most significant clinical factors currently used to predict clinical outcome. Janss AJ, Yachnis AT, Silber JH. Trojanowski JQ, Lee VM-Y, Sutton IAN9 Perilongo G, Rorkr LB, Phillips PC. Glial differentiation predicts poor clinical outcome in primitive neuroectodermal brain tumors. A n n Neurol 1396;39:481-489 Primitive neuroectodermal tumors (PNETs) of the central nervous system (CNS) represent an important group of small-cell embryonal tumors characterized clinically by their aggressive behavior [ 11. PNETs may differentiate along glial, neuronal, photoreceptor, ependymal, or other lines and may arise anywhere within the CNS. Accordingly, various classification schemes for these tumors have been proposed based on differentiation characteristics [ 2 ] , tumor location [ 3 , 41, or both [ 5 ] . PNETs of the cerebellum, also termed medulloblastoma (PNET/MB), constitute more than 20% of all pediatric brain tumors (6-81 and are the most common malignant brain tunior in children [9]. Supratentorial PNETs, including central neuroblastoma, ependymoblastoma, and pineoblastoma, may arise from different regions of the brain yet are often morphologically indistinguishable from PNET/MB [2-41. These tumors are considerably less common than PNETIMB and constitute approximately 2.5% of all childhood brain tumors. Supratentorial PNETs occur more frequently in infants and have a high rate of leptomeningeal tumor spread at diagnosis [4]. These clinical characteristics predict poor prognosis in PNET/ MB; however, the often-cited assertion that the supratentorial location, per se, confers a lower survival rate than PNET/MB [ l , 7, 101 is difficult to confirm because of the small number of supratentorial PNETs, variable treatment approaches, and inconsistent tumor staging [ 3 ] . Optimal strategies for initial PNET therapy are important since, at the present time, no effective salvage regimens for children with recurrent PNET have been identified. Unfortunately, intensive therapy may cause late treatment complications, including second malignancies, endocrine and/or growth dysfunctions, and cognitive impairments, thereby decreasing quality of life for many long-term survivors [ 1 1 -161. Identification of prognostic factors that distinguish patients at relatively low risk of tumor recurrence from those at

Changes in the Approach to Central Nervous System Tumors in Childhood

Pediatric Clinics of North America, 1992

The number of children who survive brain tumors has increased over the past 20 years because of advances in surgery, radiation, and possibly chemotherapy. Previously, minimal concern existed about possible adverse effects of these types of therapies because the number of long-term survivors was so limited. Today, 50% of children with all types of brain tumors may be expected to survive 5 years. The goals of neuro-oncology have broadened to include not only improved survival rates but also improved quality of life. In this article, we discuss both of these areas: (1) changes in therapy that have impacted survival rates and (2) changes in therapy as a consequence of complications of treatment. CHANGES IN THERAPY THAT HAVE IMPACTED ON SURVIVAL RATES Two tumors in which changes in therapy have impacted on survival rates are medulloblastomas and brain-stem gliomas. Perhaps the most remarkable improvement in survival has been in those children with medulloblastomas. Medulloblastomas Medulloblastomas represent 20% to 30% of all childhood brain tumors. Survival rates of children with these tumors have changed dramatically over the last 60 years. In 1930, Cushing reported that 1 of 61 children operated for

Central nervous system cancers

Journal of the National Comprehensive Cancer Network : JNCCN, 2013

Primary and metastatic tumors of the central nervous system are a heterogeneous group of neoplasms with varied outcomes and management strategies. Recently, improved survival observed in 2 randomized clinical trials established combined chemotherapy and radiation as the new standard for treating patients with pure or mixed anaplastic oligodendroglioma harboring the 1p/19q codeletion. For metastatic disease, increasing evidence supports the efficacy of stereotactic radiosurgery in treating patients with multiple metastatic lesions but low overall tumor volume. These guidelines provide recommendations on the diagnosis and management of this group of diseases based on clinical evidence and panel consensus. This version includes expert advice on the management of low-grade infiltrative astrocytomas, oligodendrogliomas, anaplastic gliomas, glioblastomas, medulloblastomas, supratentorial primitive neuroectodermal tumors, and brain metastases. The full online version, available at NCCN. or...

Retrospective histological analysis of CNS tumors - A 5 year study

International Journal of Medical Science and Public Health, 2014

Background: Cancers of the central nervous system (CNS) are considered to be among the most notorious of all cancers. The brain and spinal cord are complex & delicate organs that control the higher functions, the peripheral nervous system, and many of the voluntary and involuntary systems of the body. It has been found that about 1/3 of all cancers metastasize to the brain. Low-grade tumors have been found over time to progress to high grade tumors. Aims & Objectives: The objective of this article is to provide a current overview of the descriptive epidemiology of central nervous system tumors in our hospital set up. Our target was to study incidence of various lesion in light of WHO classification (2007) & study relevant statistics. Materials and Methods: A total of 65 cases of CNS tumors were retrieved from the archives of the Department of Pathology, M.G.M. Medical College, Indore from May 2009 to May 2014. The diagnoses in all the cases were made on hematoxylin & eosin stained slides of processed tissue. Results: In our study, meningioma was the most common lesion followed by astrocytoma. Out of total 65 cases, we came across 27 cases of meningioma and Astrocytoma was in 16 cases. 5 cases of ependymomas were seen. Conclusion: Males are at much higher risk of developing CNS lesion in comparison to females. WHO Grade I lesions were more common in our institutional set up. Astrocytic WHO Grade III lesion was more common in comparison to Grade I lesion indicating need for imaging & neurology consultation at grass root level.