10 Nervous Tumors

7/23/2019 10 Nervous Tumors

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CENTRAL AND PERIPHERALCENTRAL AND PERIPHERAL

NERVOUS TUMORSNERVOUS TUMORS


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GliomasGliomas

General definition: Tumor arising fromGeneral definition: Tumor arising from astrocytes,astrocytes,

oligodendrocytes,oligodendrocytes,

ependymal cells, andependymal cells, and

plexus epithelial cells.plexus epithelial cells.

These tumors often exhibit a radial groupingThese tumors often exhibit a radial grouping

of cell nuclei. The term rosette is used to referof cell nuclei. The term rosette is used to refer

to such a grouping around a nonvascularto such a grouping around a nonvascular

lumen. A radial arrangement of nuclei around alumen. A radial arrangement of nuclei around a

vascular structure or a virtual center is referredvascular structure or a virtual center is referred

to as a pseudorosette.to as a pseudorosette.


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GliomasGliomas

Gliomas present with the following generalGliomas present with the following general

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

growth into the tissue of the brain.growth into the tissue of the brain.

Systemic metastases are extremely rare.Systemic metastases are extremely rare.

The tumor often distorts the topography of theThe tumor often distorts the topography of the

brain.brain. omplications include death from increasedomplications include death from increased

intracranial pressure.intracranial pressure.


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Gliomas are diagnosed byGliomas are diagnosed by

immunohistochemical findings of expressedimmunohistochemical findings of expressed

G!A" #an acidic glial fiber protein$.G!A" #an acidic glial fiber protein$.

GliomasGliomas


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astrocytoma


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%ell-differentiated astrocytoma.A, The right frontal tumor has

expanded gyri, which led to flattening (arrows).

B, &xpanded white matter of the left cerebral hemisphere and

thic'ened corpus callosum and fornices.


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(ow-Grade-Astrocytoma


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"ilocytic astrocytoma in the cerebellum

with a nodule of tumor in a cyst.


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"ilocytic astrocytoma"ilocytic astrocytoma

(HE) x 100(HE) x 100


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Grad!III!As"ro#$"omaGrad!III!As"ro#$"oma


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Gemistocytic astrocytomaGemistocytic astrocytoma

(HE) x 100(HE) x 100


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Exami%a"io% o& #r's rara"io%*Exami%a"io% o& #r's rara"io%*

Cr+ral masss ,os mali-%a%#$ s"a"'s is '%.%o,% ar lo#a"d a%dCr+ral masss ,os mali-%a%#$ s"a"'s is '%.%o,% ar lo#a"d a%d

asira"d '%dr s"ro"a#"i# -'ida%#/ T %dl +ios$ o+"ai%d is &la""%dasira"d '%dr s"ro"a#"i# -'ida%#/ T %dl +ios$ o+"ai%d is &la""%d

+",% ",o slids/ O%# " ror s"ai% is alid " #lls #o%"ai%d i% "+",% ",o slids/ O%# " ror s"ai% is alid " #lls #o%"ai%d i% "

s#im% #a% + al'a"d ,i"i% a &, mi%'"s (C D)/s#im% #a% + al'a"d ,i"i% a &, mi%'"s (C D)/


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Oli-od%dro-liomaOli-od%dro-lioma

)ccurrence: cerebrum.)ccurrence: cerebrum.

Age of manifestation: adults.Age of manifestation: adults.

*efinition: Slowly growing tumor arising from*efinition: Slowly growing tumor arising from

oligodendrocytes.oligodendrocytes.

+orphology: ll-defined tumor of small, densely+orphology: ll-defined tumor of small, densely

pac'ed tumor cells #exhibiting a dar' nucleuspac'ed tumor cells #exhibiting a dar' nucleus

in bright cytoplasm$ that creates a honeycombin bright cytoplasm$ that creates a honeycomb

pattern. Signs of regression include bleeding,pattern. Signs of regression include bleeding,

cysts, and calcification.cysts, and calcification.


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This rare tumor arises from cells that ma'e upThis rare tumor arises from cells that ma'e up

the fatty substance called myelin that coversthe fatty substance called myelin that covers

the nerves li'e electrical insulation.the nerves li'e electrical insulation.

These tumors usually occur in the cerebrum.These tumors usually occur in the cerebrum.

They grow slowly and usually do not spreadThey grow slowly and usually do not spread

into surrounding brain tissue li'e astrocytomasinto surrounding brain tissue li'e astrocytomas

do.do.

They are most common in middle-aged adults.They are most common in middle-aged adults.


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linical presentation : "atients exhibit

symptoms of epilepsy. The tumor tends to evolve into less

differentiated forms.



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%d$moma%d$moma


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%d$moma%d$moma


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&pendymoma.A, Tumor growing into the fourth ventricle,

distorting, compressing, and infiltrating surrounding

structures.B, +icroscopic appearance of ependymoma.


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E%d$moma ross"E%d$moma ross"


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md'llo+las"omamd'llo+las"oma


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+edulloblastoma.A, T scan showing a contrast-

enhancing midline lesion in the posterior fossa.


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+edulloblastoma.

Sagittal section of brain showing medulloblastoma destroying

the superior midline cerebellum.


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+edulloblastoma. C, +icroscopic appearance of

medulloblastoma


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etinoblastomaetinoblastoma


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etinoblastomaetinoblastoma ( $ )( $ )


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RETINO2LASTOMARETINO2LASTOMA


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etinoblastoma.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. *ystrophic calcification (dark arrow) is present in the

ones of tumor necrosis. !lexner-%intersteiner rosettes arrangements of a singlelayer of tumor cells around an apparent /lumen/0are seen throughout the tumor,

and one such rosette is indicated by the white arrow.


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etinoblastomaetinoblastoma( $ )( $ ) (HE) x 30(HE) x 30


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RETINO2LASTOMARETINO2LASTOMA


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m%i%-iomam%i%-ioma


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*efinition: 1enign arachnoid cell tumor +orphology: Spherical or lobulated tumor

consisting of spindle-shaped tumor cells

#meningothelial cells of the arachnoid$ thattend to assume an arrangement resembling thelayers of an onion.

The tumor is located between the softmeninges, successively leading to formation ofa capsule and reactive hyperostosis of thes'ull.

m%i%-iomam%i%-ioma


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"arasagittal multilobular meningioma attached to

the dura with compression of underlying brain.


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+%i%-% m%i%-ioma+%i%-% m%i%-ioma


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m%i%-iomam%i%-ioma


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m%i%-iomam%i%-ioma


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+eningioma with a whorled pattern of cell growth and

psammoma bodies.


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Psammoma"o's m%i%-ioma

"sammomatous meningioma exhibits dense

epithelial clusters of tumors cells forming

numerous corpuscles resembling the layers of

an onion, leading to 2psammomatous3calcification


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4i+ro's m%i%-ioma4i+ro's m%i%-ioma


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4i+ro's m%i%-ioma4i+ro's m%i%-ioma


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!ibroblastic meningioma exhibits chains and

swirls of tumor cells rich in collagen fibers

with few onion-li'e corpuscles.


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"ra%sisio%al m%i%-ioma"ra%sisio%al m%i%-ioma


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#as#as


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A 45-year-old woman presented with progressiveA 45-year-old woman presented with progressive

headaches, unstable gait, short-term memoryheadaches, unstable gait, short-term memory

deficit, and mood swings.deficit, and mood swings.

1ladder and bowel function were unaffected. 6o1ladder and bowel function were unaffected. 6o

other focal deficits were noted.other focal deficits were noted. The patient had no history of trauma.The patient had no history of trauma.

"ast medical history was significant for"ast medical history was significant for

rheumatoid arthritis treated with piroxicam andrheumatoid arthritis treated with piroxicam and

hydroxychloro7uine sulfate.hydroxychloro7uine sulfate.


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Serum electrolytes and white blood cell countSerum electrolytes and white blood cell count

were within normal limits.were within normal limits.

She was mildly anemic. 8emoglobin andShe was mildly anemic. 8emoglobin and

hematocrit values were 9.4 gd( and ;


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A computed tomographic scan of the brainA computed tomographic scan of the brain

demonstrated a large, enhancing left lateral sphenoiddemonstrated a large, enhancing left lateral sphenoid

wing tumor, measuring 4 to > cm in greatestwing tumor, measuring 4 to > cm in greatestdimension with surrounding edema.dimension with surrounding edema.

A follow-up magnetic resonance imagingmagneticA follow-up magnetic resonance imagingmagnetic

resonance angiography study confirmed a large, leftresonance angiography study confirmed a large, leftfrontotemporal, extra-axial tumor with generaliedfrontotemporal, extra-axial tumor with generalied

enhancement and evidence of hemorrhage within theenhancement and evidence of hemorrhage within the

tumor. 6o large vessels were noted to feed into thetumor. 6o large vessels were noted to feed into the

tumor? however, the left middle cerebral artery wastumor? however, the left middle cerebral artery wasmar'edly displaced. The patient was treated withmar'edly displaced. The patient was treated with

phenytoin and a craniotomy was performed.phenytoin and a craniotomy was performed.


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Pro-rssi Hada#s i% a 30!5ar!Old 6oma%Pro-rssi Hada#s i% a 30!5ar!Old 6oma%


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Surgery yielded a @.5 ;.4 B.5-cm aggregate ofSurgery yielded a @.5 ;.4 B.5-cm aggregate of

tan-pin', mucoid, focally hemorrhagic soft tissuetan-pin', mucoid, focally hemorrhagic soft tissue

fragments. 8istopathology revealed abnormalfragments. 8istopathology revealed abnormal

trabeculae composed of vacuolated eosinophilictrabeculae composed of vacuolated eosinophiliccells in a myxoid bac'ground #cells in a myxoid bac'ground #!igure B!igure B $. Also$. Also

identified were small areas composed of whorledidentified were small areas composed of whorled

epithelial cells #epithelial cells #!igure ;!igure ; $.$.


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6a" is $o'r dia-%osis76a" is $o'r dia-%osis7


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Pa"olo-i# Dia-%osis*Pa"olo-i# Dia-%osis*Cordoid M%i%-iomaCordoid M%i%-ioma


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hordoid meningiomas feature a mixture ofhordoid meningiomas feature a mixture ofepithelioid and spindled cells within a myxoid matrix.epithelioid and spindled cells within a myxoid matrix.

BC4BC4 The histologic appearance closely resembles aThe histologic appearance closely resembles a

chordoma.chordoma.BC4BC4The tumor exhibits cytoplasmicThe tumor exhibits cytoplasmicvacuolation and clustering or cords of tumor cells.vacuolation and clustering or cords of tumor cells.;,;,44

+eningothelial foci are also usually present. n+eningothelial foci are also usually present. naddition, these tumors are often surrounded by aaddition, these tumors are often surrounded by aheavy lymphocytic infiltrate, often showing folliclesheavy lymphocytic infiltrate, often showing folliclesand germinal centers? however, this feature is notand germinal centers? however, this feature is notdiagnostic.diagnostic.B,B,DD

+ost lymphocytic infiltrates in all meningioma types+ost lymphocytic infiltrates in all meningioma typesare composed of T cells? however, chordoidare composed of T cells? however, chordoidmeningiomas of childhood are strongly associatedmeningiomas of childhood are strongly associatedwith 1 lymphocytes and plasma cells.with 1 lymphocytes and plasma cells.DD


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neurofibroma

T fib i ( .i )


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Type neurofibromatosis (s.i%)

S B55 &i+


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S-B55: %'ro&i+roma

(IH) x 83


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SchwannomaSchwannoma


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These benign tumors arise from the neural crest-derived Schwann cell and are associated withneurofibromatosis type ;.

Symptoms are referable to local compression of theinvolved nerve or to compression of adEacentstructures #such as brain stem or spinal cord$.

Sporadic schwannomas are associated with mutationsin theNF2 gene on chromosome ;;? there is usuallyabsence of theNF2 gene product by %estern blottingor immunostaining, even if there is no evidence of amutation in the gene.



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S#,a%%oma/A 2ila"ral i-"

%r s#,a%%omas/(Courtesy of Dr. K.M.Earle.)

B T'mor so,i%-

#ll'lar aras(A%"o%i A)i%#l'di%- Vro#a$+odis (far right)

as ,ll as loosrm$xoid r-io%s(A%"o%i 2)/

S hS h


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(HE) x 100(HE) x 100


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A 93 $ar old &mal a"i%" rs%"d "o " ENT dar"m%" o& o'r i%s"i"'"A 93 $ar old &mal a"i%" rs%"d "o " ENT dar"m%" o& o'r i%s"i"'"

,i" a o% $ar is"or$ o& a -rad'all$ %lar-i%- mass i% " l&" i%&ra a'ri#'lar,i" a o% $ar is"or$ o& a -rad'all$ %lar-i%- mass i% " l&" i%&ra a'ri#'lar

r-io%/ Tr ,as %o is"or$ o& &a#ial ,a.%ss or ai%/ Exami%a"io% rald ar-io%/ Tr ,as %o is"or$ o& &a#ial ,a.%ss or ai%/ Exami%a"io% rald a

9x9 #m &irm %o%!"%dr mo+il mass +lo, " l&" i%%a/ 4a#ial %r &'%#"io%9x9 #m &irm %o%!"%dr mo+il mass +lo, " l&" i%%a/ 4a#ial %r &'%#"io%

alo%- ,i" o"r ENT xami%a"io% ,as %ormal/ 4NAC ,as s'--s"i o& a si%dlalo%- ,i" o"r ENT xami%a"io% ,as %ormal/ 4NAC ,as s'--s"i o& a si%dl#ll "'mor* I%"raora"il$ " mai% "r'%. o& " &a#ial %r ,as %ormal/#ll "'mor* I%"raora"il$ " mai% "r'%. o& " &a#ial %r ,as %ormal/

Mi " i + diMi " i + di


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Mi#roo"o-ra so,i%- ro#a$ +odisMi#roo"o-ra so,i%- ro#a$ +odis

,i" %'#lar alisadi%- a%d a ,ll,i" %'#lar alisadi%- a%d a ,ll

%#as'la"d%#as'la"d


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(A) Axial &a"!s'rssd T9!,i-"d si% #o ima- a%d(A) Axial &a"!s'rssd T9!,i-"d si% #o ima- a%d

(2)Axial &a"!s'rssd T1! ,i-"d si% #o ima- ,i"(2)Axial &a"!s'rssd T1! ,i-"d si% #o ima- ,i"

#o%"ras" %a%#m%"/#o%"ras" %a%#m%"/

( & i d


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((A) Po"o-raA) Po"o-rao& " -ross s#im% a%do& " -ross s#im% a%d

(2)His"olo-i#al o"o-ra so,i%-(2)His"olo-i#al o"o-ra so,i%-

s#,a%%omas#,a%%oma


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METASTATIC TUMORMETASTATIC TUMOR

10 Nervous Tumors

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