It is
group of tumors with similar histological appearance that are thought to
arise from germinal or matrix cells of the primitive embryonal neural
tube.
Ependymoblastomas, rare, malignant, tumors with distinct ependymal
differentiation, are included together with medulloepitheliomas,
neuroblastomas, ganglioneuroblastoma, pineoblastoma, and medulloblastomas
in the group of embryonal tumors.
In the past, these tumors, along with
medulloblastoma, were included in 'Primitive Neuroectodermal tumors (PNET)'.
Recently,
WHO reserved the term
PNET only to Medulloblastoma, irrespective of location.
The
majority of these embryonal tumors are found infratentorially in the form
of cerebellar
medulloblastomas.
Other
embryonal tumors are predominantly large tumors, often involving the deep
supratentorial structures. The children are most commonly affected. In
children, they account for 2.5% to 5% of all primary brain tumors.
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They have a well known propensity to disseminate, occasionally doing
so systemically.
They resemble each other histologically.
Histologically, they are composed of a sheet like pattern of
undifferentiated cells containing dark, oval to irregular nuclei
surrounded by minimal amounts of cytoplasm.
Variable degrees of neuronal and glial differentiation may be
observed.
Naming of the individual tumors within this category depends on
histology.
In
cases of neuronal differentiation (Homer Wright rosettes), it is
'cerebral neuroblastoma'.
In
cases of ganglion like morphology, it is 'ganglionneuroblastoma'.
In
cases with differentiation along multiple cell lines, it is 'mixed
malignant tumor'. |
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Ependymoblastoma-MRI |
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In
cases with ependymal differentiation (perivascular pseudorosettes and true
rosettes), it is named 'ependymoblastoma'..
Seizures and focal neurological deficits, with features of raised ICT are
the symptoms at presentation.
CT and
MRI reveal a well differentiated, heterogenous, markedly enhancing
hemispheric mass with varying degree of cystic or necrotic changes.
Evidence of CSF dissemination is typically seen as focal or diffuse
enhancement within the subarchnoid space and ventricular system.
Aggressive multimodality approach, similar to the management of
medulloblastomas, is recommended.
Complete resection, and post operative craniospinal radiation is commonly
used.
Chemotheray have taken a greater role in children.
The
prognosis is poor, with few survivors beyond three years.
Medulloepithelioma:
This is an extremely
rare tumor, possibly derived from the primitive medullary plate and neural
tube. Rubinstein considers it the most primitive and
multi-potential neoplasm in neuro-oncology and a truly ‘embryonic tumor’.
The tumor is
essentially cerebral in location and is generally encountered early in
life.
It is usually soft,
friable and hemorrhagic.
Microscopically it
presents a papillary and almost tubular arrangement of medium or tall
columnar cells, recalling the structure of the primitive medullary
epithelium. These cells are bounded by an internal limiting membrane and
may be arranged in a single layer and show slight stratification. Neither
cilia nor blepharoplasts can be demonstrated and the nuclei are large,
oval and vesicular. The papillae and occasional solid cords of tumor
cells rest on a prominent vascular connective tissue stroma which might be
so proliferated as to create the impression of an additional vasoformative
tumor.
Generally, these
primitive tumors do not exhibit any cellular maturation, but occasionally
there may be evidence of a neuroblastic transformation in the majority of
tumors. However, tissue culture experiments have shown that
medulloblastomas are uniformly neuroblastic in virto.
Neuroblastoma:
Neuroblastomas are rare
tumors occurring essentially during the first year of life. Many occur as
congenital tumors. These arise from immature neurons and
characteristically, therefore, possess the ability to mature into adult
neurons. Another feature is their ability to undergo arrest of maturation
and involution.
They may arise in two
situations, viz., cerebral and olfactory. Though extracranial examples are
more common than cerebral forms, the histological features are similar in
both regions.
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Cererbral
neuroblastomas are found most frequently in children, under 5,
and situated deep in the brain often in the forntoparietal lobes,
where they form a defined mass. Histologically, it consists of a
pattern less proliferation of small cells often engaged in
Homer-Wright resettes similar to those observed in medulloblastoma,
but glial differentiation is not seen.
Olfactory neuroblastoma (Esthesio
neuroblastomas) are peculiar tumors that arise from the olfactory bulb
or more frequently from the roof of the nasal cavity. They spread
locally along the paranasal sinuses and may invade the frontal lobe.
The treatment consist of surgery and radiation. They are highly
radiosenstive.They tend to recur. Long term survival is possible. |
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Esthesio
neuroblastoma |
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The histological
features are comparable to those of cerebellar medulloblastoma. Generally,
a highly cellular neoplasm is seen and this consists of fairly uniform
cells resembling the cerebellar medulloblastoma, with scanty cytoplasm and
a large deep-staining nucleus. Homer-Wright rosettes may also be seen in
up to 50 per cent of cases. In many areas, islands of cells may be
separated by strands of connective tissue. Silver stains may show the
presence of neurofibrillary material and in some areas, cells suggestive
of immature neurons may be seen. Secondary changes such as necrosis,
hemorrhage and calcification are rare. The undifferentiated variety has
sheets of small tumor cells with dense circular nuclei. With greater
differentiation vaguely defined rosettes or small clusters of cells may be
seen in places. In either event, the tumor shows a considerable amount of
connective tissue, specially a rich reticulin framework, which reaches up
to the surface arachnoid mater. On this account, the cerebral
neuroblastoma, like the medulloblastoma, may be confused with a meningeal
sarcoma. Parts of the tumor may show some differentiation towards the
formation of neurons.
The tumors are of low
grade malignancy and the olfactory examples are of relatively slow
growth. Though highly radiosensitive, they are liable to recur and spread
to adjacent regions. The olfactory neuroblastoma (esthesioneuroblastoma)
may possibly have its origin in the olfactory bulb and present as a
paramedian frontal space occupying lesion.
Recurrence after
surgical removal and changes in histological features after radiation have
been described.
Medulloblastomas:
discussed elsewhere.
Pineoblastomas: discussed
elsewhere.