10 Nervous Tumors

CENTRAL AND PERIPHERAL
NERVOUS TUMORS
Gliomas
General definition: Tumor arising from
astrocytes,
oligodendrocytes,
ependymal cells, and
plexus epithelial cells.
These tumors often exhibit a radial grouping

of cell nuclei. The term rosette is used to refer
to such a grouping around a nonvascular
lumen. A radial arrangement of nuclei around a
vascular structure or a virtual center is referred
to as a pseudorosette.
Gliomas
Gliomas present with the following general

clinical features:
The tumor is ill-defined due to its infiltrative

growth into the tissue of the brain.
Systemic metastases are extremely rare.
The tumor often distorts the topography of the
brain.
Complications include death from increased
intracranial pressure.
Gliomas
Gliomas are diagnosed by

immunohistochemical findings of expressed
GFAP (an acidic glial fiber protein).
astrocytoma
Well-differentiated astrocytoma. A, The right frontal tumor has

expanded gyri, which led to flattening (arrows).
B, Expanded white matter of the left cerebral hemisphere and
thickened corpus callosum and fornices.
Low-Grade-Astrocytoma
Pilocytic astrocytoma in the cerebellum

with a nodule of tumor in a cyst.
Pilocytic astrocytoma
(HE) x 100
Grade-III-Astrocytoma
Gemistocytic astrocytoma
(HE) x 100
Examination of crush preparation:

Cerebral masses whose malignancy status is unknown are located and
aspirated under stereotactic guidance. The needle biopsy obtained is flattened
between two slides. Once the proper stain is applied, the cells contained in the
specimen can be evaluated within a few minutes (C, D).
Oligodendroglioma
Occurrence: cerebrum.
Age of manifestation: adults.
Definition: Slowly growing tumor arising from
oligodendrocytes.
Morphology: Ill-defined tumor of small, densely
packed tumor cells (exhibiting a dark nucleus
in bright cytoplasm) that creates a honeycomb
pattern. Signs of regression include bleeding,
cysts, and calcification.
Oligodendroglioma
This rare tumor arises from cells that make up

the fatty substance called myelin that covers
the nerves like electrical insulation.
These tumors usually occur in the cerebrum.
They grow slowly and usually do not spread
into surrounding brain tissue like astrocytomas
do.
They are most common in middle-aged adults.
Oligodendroglioma
Clinical presentation : Patients exhibit

symptoms of epilepsy.
The tumor tends to evolve into less
differentiated forms.
Oligodendroglioma
ependymoma
ependymoma
Ependymoma. A, Tumor growing into the fourth ventricle,

distorting, compressing, and infiltrating surrounding
structures. B, Microscopic appearance of ependymoma.
Ependymoma, rosset
medulloblastoma
Medulloblastoma. A, CT scan showing a contrastenhancing midline lesion in the posterior fossa.
Medulloblastoma.
Sagittal section of brain showing medulloblastoma destroying
the superior midline cerebellum.
Medulloblastoma. C, Microscopic appearance of

medulloblastoma
Retinoblastoma
Retinoblastoma ( eye )
RETINOBLASTOMA
Retinoblastoma. A, Gross photograph of retinoblastoma. B, Tumor cells appear

viable when in proximity to blood vessels, but necrosis is seen as the distance
from the vessel increases. Dystrophic calcification (dark arrow) is present in the
zones of tumor necrosis. Flexner-Wintersteiner rosettes arrangements of a single
layer of tumor cells around an apparent "lumen"are seen throughout the tumor,
and one such rosette is indicated by the white arrow.
Retinoblastoma ( eye )
(HE) x 50
RETINOBLASTOMA
meningioma
meningioma
Definition: Benign arachnoid cell tumor

Morphology: Spherical or lobulated tumor
consisting of spindle-shaped tumor cells
(meningothelial cells of the arachnoid) that
tend to assume an arrangement resembling the
layers of an onion.
The tumor is located between the soft
meninges, successively leading to formation of
a capsule and reactive hyperostosis of the
skull.
Parasagittal multilobular meningioma attached to

the dura with compression of underlying brain.
beningn meningioma
meningioma
meningioma
Meningioma with a whorled pattern of cell growth and

psammoma bodies.
Psammomatous meningioma
Psammomatous meningioma exhibits dense

epithelial clusters of tumors cells forming
numerous corpuscles resembling the layers of
an onion, leading to psammomatous
calcification
Fibrous meningioma
Fibrous meningioma
Fibroblastic meningioma exhibits chains and

swirls of tumor cells rich in collagen fibers
with few onion-like corpuscles.
transisional meningioma
case
A 50-year-old woman presented with progressive

headaches, unstable gait, short-term memory
deficit, and mood swings.
Bladder and bowel function were unaffected. No
other focal deficits were noted.
The patient had no history of trauma.
Past medical history was significant for
rheumatoid arthritis treated with piroxicam and
hydroxychloroquine sulfate.
Serum electrolytes and white blood cell count

were within normal limits.
She was mildly anemic. Hemoglobin and
hematocrit values were 9.5 g/dL and 28%,
respectively.
A computed tomographic scan of the brain

demonstrated a large, enhancing left lateral sphenoid
wing tumor, measuring 5 to 6 cm in greatest
dimension with surrounding edema.
A follow-up magnetic resonance imaging/magnetic
resonance angiography study confirmed a large, left
frontotemporal, extra-axial tumor with generalized
enhancement and evidence of hemorrhage within the
tumor. No large vessels were noted to feed into the
tumor; however, the left middle cerebral artery was
markedly displaced. The patient was treated with
phenytoin and a craniotomy was performed.
Progressive Headaches in a 50-Year-Old Woman
Surgery yielded a 3.0 2.5 1.0-cm aggregate of

tan-pink, mucoid, focally hemorrhagic soft tissue
fragments. Histopathology revealed abnormal
trabeculae composed of vacuolated eosinophilic
cells in a myxoid background (Figure 1 ). Also
identified were small areas composed of whorled
epithelial cells (Figure 2 ).
What is your diagnosis?
Pathologic Diagnosis:
Chordoid Meningioma
Chordoid meningiomas feature a mixture of

epithelioid and spindled cells within a myxoid matrix.
15
The histologic appearance closely resembles a
chordoma.15 The tumor exhibits cytoplasmic
vacuolation and clustering or cords of tumor cells.2,5
Meningothelial foci are also usually present. In
addition, these tumors are often surrounded by a
heavy lymphocytic infiltrate, often showing follicles
and germinal centers; however, this feature is not
diagnostic.1,4
Most lymphocytic infiltrates in all meningioma types
are composed of T cells; however, chordoid
meningiomas of childhood are strongly associated
with B lymphocytes and plasma cells.4
neurofibroma
Type I neurofibromatosis (skin)
S-100: neurofibroma
(IH) x 75
Schwannoma
Schwannoma
These benign tumors arise from the neural crestderived Schwann cell and are associated with
neurofibromatosis type 2.
Symptoms are referable to local compression of the
involved nerve or to compression of adjacent
structures (such as brain stem or spinal cord).
Sporadic schwannomas are associated with mutations
in the NF2 gene on chromosome 22; there is usually
absence of the NF2 gene product by Western blotting
or immunostaining, even if there is no evidence of a
mutation in the gene.
Schwannoma.
A, Bilateral eighth
nerve schwannomas.
(Courtesy of Dr. K.M.
Earle.)
B, Tumor showing
cellular areas
(Antoni A),
including Verocay
bodies (far right),
as well as looser,
myxoid regions
(Antoni B).
Schwannoma
(HE) x 100
A 25 year old female patient presented to the ENT department of our institute
with a one year history of a gradually enlarging mass in the left infra auricular
region. There was no history of facial weakness or pain. Examination revealed a
2x2 cm firm, non-tender, mobile mass below the left pinna. Facial nerve function
along with other ENT examination was normal. FNAC was suggestive of a spindle
cell tumor: Intraoperatively, the main trunk of the facial nerve was normal.
Microphotograph showing verocay bodies

with nuclear palisading and a well
encapsulated
(A) Axial fat-suppressed T2-weighted spin echo image and

(B)Axial fat-suppressed T1- weighted spin echo image with
contrast enhancement.
(A) Photograph of the gross specimen and

(B)Histological photograph showing
schwannoma
METASTATIC TUMOR

10 Nervous Tumors

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CENTRAL AND PERIPHERAL

General definition: Tumor arising from

These tumors often exhibit a radial grouping

Gliomas present with the following general

The tumor is ill-defined due to its infiltrative

Gliomas are diagnosed by

Well-differentiated astrocytoma. A, The right frontal tumor has

Pilocytic astrocytoma in the cerebellum

Examination of crush preparation:

This rare tumor arises from cells that make up

Clinical presentation : Patients exhibit

Ependymoma. A, Tumor growing into the fourth ventricle,

Medulloblastoma. A, CT scan showing a contrastenhancing midline lesion in the posterior fossa.

Medulloblastoma. C, Microscopic appearance of

Retinoblastoma. A, Gross photograph of retinoblastoma. B, Tumor cells appear

Definition: Benign arachnoid cell tumor

Parasagittal multilobular meningioma attached to

Meningioma with a whorled pattern of cell growth and

Psammomatous meningioma exhibits dense

Fibroblastic meningioma exhibits chains and

A 50-year-old woman presented with progressive

Serum electrolytes and white blood cell count

A computed tomographic scan of the brain

Progressive Headaches in a 50-Year-Old Woman

Surgery yielded a 3.0 2.5 1.0-cm aggregate of

What is your diagnosis?

Chordoid meningiomas feature a mixture of

Type I neurofibromatosis (skin)

Microphotograph showing verocay bodies

(A) Axial fat-suppressed T2-weighted spin echo image and

(A) Photograph of the gross specimen and

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