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Abramson Cancer CenterNCI CANCERLIT® Search: Tuberous Sclerosis - August 2002
National Cancer Institute®
Last Modified: August 1, 2002
- Gangliogliomas: an intriguing tumor entity associated with focal epilepsies.
- Tuberous sclerosis complex: a review of neurological aspects.
- Ethical approval for research involving geographically dispersed subjects: unsuitability of the UK MREC/LREC system and relevance to
Table of Contents
1
UI - 12125736
AU - Blumcke I; Wiestler OD
TI -
Gangliogliomas: an intriguing tumor entity associated with focal
epilepsies.
SO - J Neuropathol Exp Neurol 2002 Jul;61(7):575-84
AD - Department of Neuropathology, University of Erlangen, Germany.
Gangliogliomas represent the most frequent tumor entity in young
patients suffering from chronic focal epilepsies. In a series of 326
gangliogliomas collected from the University of Bonn Epilepsy Surgery
Program and other departments of neuropathology in Germany, Austria, and
Switzerland, epidemiological findings and histopathological hallmarks of
gangliogliomas are systematically reviewed. The majority of these tumors
occur within the temporal lobe and reveal a biphasic histological
architecture characterized by a combination of dysplastic neurons and
neoplastic glial cell elements. However, gangliogliomas exhibit a
considerable variability in their histopathological appearance.
Immunohistochemical studies are an important tool to discriminate these
neoplasms from other tumor entities. Almost 80% of gangliogliomas reveal
immunoreactivity for CD34, a stem cell epitope not expressed in normal
brain. Immunohistochemical reactions for MAP2 or NeuN can be employed to
characterize the dysplastic nature of neurons in those areas difficult
to discriminate from pre-existing brain parenchyma. Less than 50% of the
cases display binucleated neurons. With the frequent finding of
"satellite" tumor clusters in adjacent brain regions, gangliogliomas are
microscopically less circumscribed than previously assumed. The
distinction from diffusely infiltrating gliomas is of considerable
importance since tumor recurrence or malignant progression are rare
events in gangliogliomas. Only little is known about the molecular
pathogenesis of these glioneuronal tumors. Our findings support a
dysontogenic origin from a glioneuronal precursor lesion with
neoplastic, clonal proliferation of the glial cell population. Candidate
genes appear to associate with neurodevelopmental signaling cascades
rather than cell cycle control or DNA repair mechanisms. The reelin
signaling and tuberin/insulin growth receptor pathways have recently
been implicated in ganglioglioma development. Powerful new molecular
genetic and biological tools can now be employed to unravel the
pathogenesis of these intriguing lesions.
2
UI - 11993952
AU - Curatolo P; Verdecchia M; Bombardieri R
TI -
Tuberous sclerosis complex: a review of neurological aspects.
SO - Eur J Paediatr Neurol 2002;6(1):15-23
AD - Department of Neurosciences-Section of Paediatric Neurology, Tor Vergata
University of Rome, Italy. curatolo@uniroma2.it
Tuberous sclerosis complex is characterized by hamartomatous lesions
involving skin, brain, kidneys, eyes and heart. Pathologically, tuberous
sclerosis is a disorder of cell migration, proliferation and
differentiation. Cell lineage and cell migration disorders in the
developing cortex of tuberous sclerosis complex patients might produce
very different neurological phenotypes including epilepsy, cognitive
impairment and autism. Cortical tubers constitute the hallmark of the
disease and are pathognomonic of cerebral tuberous sclerosis. Epilepsy
is the most common neurological feature, occurring in 96% of patients.
Seizures often begin in the first months of life and are frequently
severe and intractable. The treatment of seizures has recently benefited
from the advent of the new anti-epileptic drugs. Selected drug-resistant
patients with tuberous sclerosis complex could be considered for
surgical treatment. Clear localization of the most active epileptogenic
focus and the zone of the cortical abnormality may lead to tuberectomy
and improved seizure control in selective drug-resistant patients. The
finding of multiple areas of cerebral involvement should not
automatically preclude epilepsy surgery in a child with intractable
seizures and a well defined seizure origin.
3
UI - 11579194
AU - Lewis JC; Tomkins S; Sampson JR
TI -
Ethical approval for research involving geographically dispersed
subjects: unsuitability of the UK MREC/LREC system and relevance to
uncommon genetic disorders.
SO - J Med Ethics 2001 Oct;27(5):347-51
AD - Institute of Medical Genetics, University of Wales College of Medicine,
Heath Park, Cardiff, Wales, UK.
OBJECTIVES: To assess the process involved in obtaining ethical approval
for a single-centre study involving geographically dispersed subjects
with an uncommon genetic disorder. DESIGN: Observational data of the
application process to 53 local research ethics committees (LRECs)
throughout Wales, England and Scotland. The Multicentre Research Ethics
Committee (MREC) for Wales had already granted approval. RESULTS:
Application to the 53 LRECs required 24,552 sheets of paper and took two
months of the researcher's time. The median time taken for approval was
39 days with only seven (13%) of committees responding within the
recommended 21 days. In at least nineteen cases (36%) a subcommittee
considered the application. Thirty-three committees (62%) accepted the
proposal without amendments but, of the remainder, four (8%) requested
changes outside of the remit of LRECs. DISCUSSION: Difficulties still
exist with the system for obtaining ethical approval for studies
involving a single centre but with patients at multiple sites, as is
often required for genetic observational research. As such studies
differ from true multicentre studies, it may be advantageous to develop
a separate and specific process of application to ensure that resources
are not unnecessarily expended in the quest for ethical approval.
The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.
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