Tumores SNC - Aula
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Serviço de anatomia patológica HU-UFJF
Janaína Le Sann Nascimento
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Anatomia
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Sistema nervoso central:
• Encéfalo;
• Constituintes neurais do
sistema fotorreceptor;
• Medula espinhal;
Sistema nervoso periférico:
• Nervos;
• Gânglios nervosos;
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Substância
branca
Substância
cinzenta
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Corpos celulares
(neurônios e neuroglia);
Prolongamentos
celulares;
Prolongamentos
celulares (mielina);
Células da Glia;
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Meninges
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Neurônios
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Células da glia
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• Sustentação; • Controle composição iônica/molecular extracelular; • Barreira hematoencefálica; • Protoplasmáticos; • Fibrosos;
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• Produzem a bainha de
mielina do SNC;
• Corresponde à célula de
Schwann no SNP;
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• Células mononucleares
fagocitárias;
• Inflamação;
• Reparação;
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• Revestem
ventrículos e canal
central da medula;
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Epidemiologia
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10 a 17 casos por 1000 pessoas (intracraneanos);
1 a 2 casos por 1000 pessoas (intramedulares);
½ a ¾ tumores primários do SNC;
20% dos cânceres na infância;
5ª a 6ª décadas (astrocitomas e glioblastomas de
hemisférios cerebrais, metástases, meningiomas,
shwannomas e adenomas hipofisários);
< 10 anos (astrocitomas do cerebelo e tronco
cerebral, meduloblastomas e ependimomas).
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Topografia:
• Ependimomas: IV ventrículo; medula;
• Oligodendrogliomas: cérebro
(hemisférios)
• Astrocitomas: cerebelo e ponte; medula,
hemisférios.
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Padrões de crescimento:
• Expansivo;
• Infiltrativo;
Disseminação liquórica:
• Meduloblastomas;
Não dão metástases para fora do SNC;
Malignidade x benignidade: • Potencial de ressecção;
• Infiltração;
• Localização;
• Histologia tumoral.
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Classificação
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Neuroectodérmicos (glia e neurônios):
• Astrócitos (astrocitomas, glioblastomas);
• Oligodendrócitos (oligodendrogliomas);
• Epêndima (ependimomas);
• Neurônios imaturos (meduloblastomas);
Mesodèrmicos (fibroblastos meníngeos ):
• Meningiomas
Metastáticos
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Neuroectodérmicos (glia e neurônios):
• Astrócitos (astrocitomas, glioblastomas);
• Oligodendrócitos (oligodendrogliomas);
• Epêndima (ependimomas);
• Neurônios imaturos (meduloblastomas);
Mesodèrmicos (fibroblastos meníngeos )
• Meningiomas;
Metastáticos
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Critérios para classificação (OMS):
• Atipias nucleares;
• Atividade mitótica;
• Proliferação vascular;
• Necrose;
Graus de I/IV a IV/IV;
Bem diferenciados (I e II/IV),
anaplásicos (III e IV/IV);
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Origem neuroendócrina
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Astrocitomas
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Astrocitomas:
• Fibrilar;
• Glioblastoma;
• Astrocitoma pilocítico;
• Xantoastrocitoma pleomórfico;
Oligodendrogliomas;
Ependimomas:
• Mixopapilar;
• Convencional;
• Anaplásico;
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Astrocitomas difusos e glioblastoma:
•4ª a 6ª décadas;
•80 % tumores primários SNC(adulto);
• Hemisférios cerebrais;
• Sinais e sintomas relacionados à topografia
(cefaleias, convulsões, déficits focais);
• Substância branca;
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Macroscopia:
• Substância branca, hemisfério cerebral;
• Mal delimitados, infiltrativos, coloração branca
ou acinzentada; consistência firme ou até
gelatinosa;
• Cistos de conteúdo líquido, áreas de necrose
ou hemorragia;
• Poucos centímetros até lesões que substituem
o hemisfério;
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Microscopia:
• Multinucleação;
• Pleomorfismo nuclear variável;
• Trama de finas expansões celulares
(aparência fibrilar);
• Transição imprecisa entre tecido neoplásico e
normal;
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Grau II de malignidade (OMS);
Constituídos por células que lembram astrócitos
normais ou patológicos:
• Fibrilares;
• Protoplasmáticos;
• Gemistocíticos;
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Grau III de malignidade (menos diferenciado);
Maior densidade celular à microscopia;
Maior pleomorfismo;
Frequente atividade mitótica;
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Macroscopia:
• Aparência macroscópica varia;
• Pode aparecer bem demarcado do tecido
nervoso adjacente;
• Há infiltração microscópica;
• Edema peritumoral;
• Efeito de massa;
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Microscopia:
• Atipias celulares (cél. gigantes multinucleadas,
células pequenas);
• Mitoses;
• Proliferação vascular :
Aumento número capilares com proliferação das
células endoteliais (pseudoglomérulos);
• Necrose (coagulativa, em pseudo paliçada);
• Mal delimitados microscopicamente (diferenciar
de metastáses)
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Clínica:
• Grave, evolução rápida;
• Hipertensão intracraneana;
• Sinais de localização;
• Convulsão de início tardio (investigar para
tumores cerebrais);
Tratamento:
• Cirúrgico, paliativo;
• Radio adjuvante; quimio tem pouco efeito;
Prognóstico sombrio (sobrevida de 6 a 12 meses);
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Benigno;
Evolução lenta, passíveis de ressecção cirúrgica;
Predomínio na infância e adultos jovens;
Cerebelo; assoalho e paredes ventriculares (III),
nervos ópticos;
Macroscopia:
• Cístos de conteúdo amarelado com área mais
espessa em sua parede;
• Não formam cápsula;
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Microscopia:
• Células bipolares com extensões “pilosas”;
• Fibras de Rosenthal (estruturas hialinas com
formato de cenoura) localizam-se entre os
filamentos celulares;
• Corpos granulares eosinofílicos e microcistos
podem estar presentes (padrão protoplasmático);
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CRITÉRIO
HISTOLÓGICO TIPO
GRADUAÇÃO
(OMS) SOBREVIDA
CRESCIMENTO
LENTO
Astrocitoma
pilocítico I
> 5 anos
(cura)
ATIPIA
(geralmente)
Astrocitoma difuso
baixo grau II > 5 anos
ATIPIA +
MITOSES
Astrocitoma
anaplásico III 2 a 5 anos
3 ou + critérios Glioblastoma
multiforme IV < 1 ano
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Oligodendrogliomas
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5 a 15% dos tumores gliais;
4ª e 5ª décadas;
Anos de queixas neurológicas;
Convulsões;
Substância branca dos hemisférios cerebrais;
Macroscopia:
• Massas acinzentadas, bem delimitadas,
gelatinosas;
• Cistos, hemorragia focal;
• Calcificações;
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Microscopia:
• Massas de células pequenas (oligodendrócitos)
de núcleos esféricos e citoplasma claro (“ovo
frito”);
• Vasos em “tela de galinheiro”;
Tendem a recidivar;
Respondem à radio e quimioterapia;
Sobrevida média de 5 a 10 anos;
Grau II/IV (oms);
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Oligodendroglioma anaplásico:
• Aumento da densidade celuar;
• Anaplasia nuclear;
• Mitoses;
• Necrose;
• Grau III/IV;
• Pior prognóstico;
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Ependimomas
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5 a 15% dos tumores cerebrais nas 1ª e 2ª
décadas;;
Origem epitélio ependimário dos ventrículos e
canal medular (adultos);
Tumores medulares frequentes na
neurofibromatose tipo 2;
Grau II/IV (OMS);
Difícil remoção completa devido localização
(núcleos pontinos e bulbares);
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Manifestações clínicas:
• Hidrocefalia,
• Crescimento lento;
Prognóstico:
• Sobrevida de 4 anos após cirurgia e radioterapia;
• Medulares tem melhor prognóstico devido a
melhor ressecabilidade;
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Macroscopia:
• IV ventrículo;
• Massas acinzentadas papilares (forma de
couve-flor) ou sólidas, originadas no
assoalho ventricular;
• Bem demarcados;
• Infiltram tecido cerebral adjacente;
• Intramedulares;
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Microscopia:
• Núcleos regulares, arredondados ou ovais;
• Cromatina granular abundante;
• Fundo fibrilar;
• Estruturas glanduares alongadas ou arredondadas
(rosetas de Flexner, canais) com extensões longas e
delicadas protruindo para a luz;
• Pseudorosetas perivasculares formam halo
anucleado ao redor dos vasos (aspecto em pele de
leopardo) ;
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Ependimomas anaplásicos:
• Densidade celular aumentada,
• Altas taxas mitótica;
• Áreas de necrose;
• Perda da diferenciação ependimária;
• Mais agressivas (grau III/IV);
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Ependimomas mixopapilares:
• Filo terminal da medula;
• Elementos papilares em fundo mixóide, células
ependimoma símile;
• Células cubóides, citoplasma claro circundam os
eixos papilares delimitando áreas preenchidas por
tecido conjuntivo e vasos sanguíneos;
• Podem estender-se ao espaço subaracnóide
circundando a cauda equina (fator recorrência)
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Manifestações clínicas:
• Dependentes da localização;
• Dores em MMII, hipotonia, alterações
esfincterianas;
Prognóstico: depende da ressecção cirúrgica;
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Meduloblastomas
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Pobremente diferenciados ou embrionários (retém
características de células indiferenciadas primitivas);
20% dos tumores cerebrais em crianças (pico 10
anos);
Exclusivamente cerebelar;
Crianças: vermis; Adultos: hemisférios cerebelares;
Rápido crescimento, malignos;
Sensível à radioterapia;
Excisão total e irradiação (inclusive de cérebro e
medula): sobrevida em 5 anos: 75%;
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Macroscopia:
• Branco ou róseo acinzentado, bem circunscrito,
friável;
• Estende-se às leptomeninges;
Microscopia:
• Muito celular (“folhas” de células anaplásicas:
• Células pequenas, pouco citoplasma, núcleos
hipercromáticos, alongados ou em forma de
crescente;
• Mitoses abundantes;
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Origem mesodérmica
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Meningiomas
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Benignos (grau I/IV);
Adultos (45 anos);
Mulheres 3:1 Homens;
Originam-se das células meningoteliais da
aracnóide (fibroblastos);
Localizam-se ao longo das superfícies externas do
cérebro;
Origem das células aracnóides estromais do plexo
coroide (interior do sistema ventricular);
![Page 142: Tumores SNC - Aula](https://reader036.fdocuments.net/reader036/viewer/2022062304/55cf946e550346f57ba1fa6f/html5/thumbnails/142.jpg)
Macroscopia:
• Convexidade cerebral (ao longo do seio sagital
superior metade anterior); base do crânio, goteira
dos nervos olfatórios; sela turca;
• Massas esféricas ou achatadas;
• Aderidos à dura máter;
• Comprimem o cérebro deslocando-o sem invasão;
• Superfície lobulada (aspecto em “couve flor”);
• Friáveis ou firmes (calcificação)
• Sem cápsula
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Superfície de corte esbranquiçada, exibindo
corpos psamomatosos (calcificações)/
Podem infiltrar a dura máter e seios venosos;
Associa-se a alterações reativas hiperostóticas;
No canal medular são intradurais,
extramedulares;
Ausência de necrose ou hemorragias;
![Page 144: Tumores SNC - Aula](https://reader036.fdocuments.net/reader036/viewer/2022062304/55cf946e550346f57ba1fa6f/html5/thumbnails/144.jpg)
Microscopia:
• Células uniformes, núcleos redondos ou
alongados, cromatina frouxa, citoplasma róseo,
abundante, limites imprecisos;
• Padrões histológicos (disposição das células):
- Sincicial, fibroblástico, transicional,
psamomatoso, secretório, microcístico;
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Meningiomas atípicos:
• Grau II/IV;
• Maior taxa de recorrência;
• Crescimento local mais agressivo;
• Índice mitótico elevado;
• Atipias celulares (celularidade aumentada,
aumento relação N/C, nucléolos proeminentes,
necrose);
• Terapia adicional à cirurgia;
• Células claras; Cordóides;
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Meningioma anaplásico:
• Grau III/IV;
• Taxas mitóticas extremamente elevadas;
• Aspecto geral de sarcoma;
• Subtipos papilar e rabdóide;
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Outras origens
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Metastáticos
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Via hematogênica;
O tumor metastático pode se manifestar antes
do tumor primário (15% dos casos);
Pulmões > mama > pele (melanoma) > rins >
TGI;
Massas bem demarcadas tanto macro quanto
microscopicamente; edema peritumoral
Características microscópicas do tumor primário
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Referências bibliográficas:
Robbins and Cotran Pathologic Basis Disease, 8th
Edition, Elsevier-Saunders, 2008.;
Rosai and Ackerman's surgical pathology – 10th ed.;
http://anatpat.unicamp.br/
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Obrigada [email protected]