patoloji-ders-notlari

Title

Serdar Balcı

SSS Tümörleri

Dr. Serdar BALCI

SSS Tümörleri

SSS Tümörlerinin Özellikleri

SSS Tümörleri

SSS Tümörleri Sınıflaması

Hücre orijinine göre sınıflama

Ayırıcı tanı için en önemli özellikler

Lokalizasyon, lokalizasyon, lokalizasyon…

Yaş

Radyolojik özellikler

Histomorfolojik özellikler

Genetik özellikler

SSS tümörlerinin lokalizasyonu

SSS tümörlerinin yayılımı

Beyin tümörlerinin diğer bölgelere yayılımı çok nadir

Spinal subaraknoid tümör yayılımı (ekilimi, seeding)

Autopsy Pathology: A Manual and Atlas

SSS tümörlerinin derecelendirilmesi

Derecelendirme histolojik alt tipe göre yapılır

Grade I tümörler

Grade II tümörler

Grade III tümörler

Grade IV tümörler

Benign, Resection is cure

Progression can be seen

GLIOMLAR

Gliomlar

Grading of CNS gliomas

Increasing tumor malignancy is associated with more cytologic anaplasia, increased cell density, necrosis, and mitotic activity

Grade I tumors

Grade II tumors

Grade III tumors

Grade IV tumors

Astrositomlar

Pilositik Astrositom, Derece I

Pilositik Astrositom

Autopsy Pathology: A Manual and Atlas

Pilositik Astrositom

Kistik

Kist duvarında mural nodül

Eğer solid ise, iyi sınırlı

Bipolar hücreler, ince saçsı uzantılar

GFAP-pozitif

Rozental lifler, eozinofilik granüler cisimcikler, mikrokist

Nekroz ve mitoz çok çok nadir

Diffüz astrositomlar

Diffuse Astrocytoma

Infiltrating pontine glioma (astrocytoma)

Autopsy Pathology: A Manual and Atlas

Diffüz infiltratif astrositom

Autopsy Pathology: A Manual and Atlas

İyi diferansiye astrositomlar belirsiz sınırlı, gri, infiltratif tümörlerdir. Ayrı bir kitle oluşturmadan infiltratif oalrak büyür ve normal dokuların yapısını ortadan kaldırır

Robbins Basic Pathology

Makroskopik olarak görünen sınır ötesine de uzanım mutlaka vardır

Kesit yüzü sert, yumuşak veya jelatinöz

Kistik dejenerasyon görülebilir

Robbins Basic Pathology

Clinical Neuropathology Text and Color Atlas

Practical Surgical Neuropathology

Brain Tumor Pathol (2011) 28:177–183

Diffüz astrositom

Brain Tumor Pathol (2011) 28:177–183

Defects in tricyclic carbon cycle

Isocitrate dehydrogenase mutasyonları yan ürünlerin birikimine neden olur

SSS tümörlerinde yakın zamanda bulunan en önemli mutasyon

Cancer Cell. 2010 Jan 19;17(1):7-9

N Engl J Med 2009;360:765-73.

İyi diferansiye astrositom

Glial hücre sayısında hafif orta artış

Değişken nükleer pleomorfizm

Zeminde ince, GFAP pozitif gliofibriler ağ

Normal doku ve tümör geçişi net değil

Neoplastik hücreler ana tümör kitlesinin santimetrelerce uzağında olabilir

Anaplastik astrositomlar, Derece III

Selülarite daha belirgin

Daha belirgin nükleer pleomorfizm

Mitotik figürlerin görülmesi

Practical Surgical Neuropathology

Glioblastom, Derece IV

Glioblastom

Glioblastom

Nekrotik, hemorajik, infiltratif kitle

Robbins Basic Pathology

Glioblastoma multiforme

Autopsy Pathology: A Manual and Atlas

Glioblastom

Selüler tümör, nekroz ve psödopalizatlanma dizilimi

Robbins Basic Pathology

Clinical Neuropathology Text and Color Atlas

Practical Surgical Neuropathology

Practical Surgical Neuropathology

OLIGODENDROGLIOM

Oligodendrogliom

Oligodendrogliom: Tümör hücreleri yuvarlak oval çekirdekli, sitoplazmik halo

Damarlar ince

Robbins Basic Pathology

1p ve 19q kromozomların kaybı

1p ve 19q kaybı tipik olarak birlikte olur

1p ve 19q kaybı gösteren tümörlerde kemoterapi ve radyoterapiye cevap çok daha iyi

İyi diferansiye oligodendrogliomlar (WHO grade II/IV)

Anaplastic oligodendroglioma (WHO grade III/IV)

More aggressive subtype

Higher cell density

Nuclear anaplasia

Mitotic activity

EPENDİMOM

Ependimom

4. ventrikül

Solid veya papiller kitle

Ventrikül tabanından köken alır

Ependymoma

Anaplastic ependymoma

Increased cell density

High mitotic rates

Necrosis

Less evident ependymal differentiation

Robbins Basic Pathology

NEURONAL TUMORS

Neuronal Tümörler

Santral Nörositom

Gangliogliom

Dysembryoplastic neuroepithelial tumor (DNET)

Central neurocytoma

Low-grade neoplasm

Within and adjacent to the ventricular system

Most commonly the lateral or third ventricles

Evenly spaced, round, uniform nuclei

Islands of neuropil

Ganglioglioma

Dysembryoplastic neuroepithelial tumor (DNET)

EMBRİYONAL (PRIMITIF) NEOPLAZİLER

Embryonal (Primitive) Neoplasms

Medulloblastom

En sık embriyonel tümör

Pediatrik beyin tümörlerinin %20’si

Serebellum

Küçük yuvarlak hücreli malign tümör

Located in the midline of the cerebellum

Lateral tumors occur more often in adults

Often well circumscribed, gray, and friable

May extend to the surface of the cerebellar folia and involve the leptomeninges

Robbins Basic Pathology

Autopsy Pathology: A Manual and Atlas

Extremely cellular

Sheets of anaplastic (small blue) cells

Small, with little cytoplasm

Hyperchromatic nuclei

Abundant mitosis

Robbins Basic Pathology

Focal neuronal differentiation

Homer Wright or neuroblastic rosette

Resembles the rosettes in neuroblastomas

Primitive tumor cells surrounding central neuropil

-delicate pink material formed by neuronal processes

Medulloblastom genetiği

Genetic of medulloblastoma

LENFOMALAR

Primary CNS Lymphoma

Primer SSS lenfoması

Çoğunlukla diffüz büyük B hücreli lenfoma

Primary CNS Lymphoma

GERM CELL TUMORS

Primary brain germ cell tumors

MENINGIOM

Meningioma

Benign

Araknoid meningotelyal hücre kökenli

Duraya yapışık

Meningioma

WHO grade I/IV

well-defined dura-based masses

compress the brain but do not invade it

Extension into the overlying bone may be present

Robbins Basic Pathology

Parasagittal meningioma

Autopsy Pathology: A Manual and Atlas

Intraosseous meningioma

Autopsy Pathology: A Manual and Atlas

Histologic patterns of Meningioma

Robbins Basic Pathology

Atypical meningiomas (WHO grade II/IV)

prominent nucleoli

increased cellularity

pattern-less growth

higher mitotic rate

More aggressive local growth

Higher rate of recurrence

Require therapy in addition to surgery

Anaplastic (malignant) meningiomas (WHO grade III/IV)

Highly aggressive tumors

May resemble a high-grade sarcoma or carcinoma

usually some histologic evidence of a meningothelial cell origin

METASTATIK TÜMÖRLER

Metastatic Tumors

Mostly carcinomas

1/4-1/2 of intracranial tumors

Most common primary sites

Lung, breast, skin (melanoma), kidney, and gastrointestinal tract

Form sharply demarcated masses

Often at the gray-white junction, and elicit edema

The boundary between tumor and brain parenchyma is sharp at the microscopic level as well, with surrounding reactive gliosis

Direct and localized effects

#

Metastatic spread of brain tumors to other regions of the body is rare

Brain is not protected against spread of distant tumors

Carcinomas are the dominant type of systemic tumors that metastasize to the nervous system

Metastatic melanoma

Metastatic lesions are distinguished grossly from most primary central nervous system tumors by their multicentricity and well-demarcated margins

The dark color of the tumor nodules in this specimen is due to the presence of melanin

Robbins Basic Pathology

Paraneoplastic syndromes

AILESEL TÜMÖR SENDROMLARI

Ailesel tümör sendromları

Tuberous Sclerosis (TSC)

von Hippel–Lindau Disease

Autopsy Pathology: A Manual and Atlas

Autopsy Pathology: A Manual and Atlas

Distribution of CNS Tumors

**A pilocytic astrocytoma of the cerebellum in a child. **

Glioma of the brainstem – pilocytic astrocytoma

GBM. Note the prominent vascularity as well as the area of necrosis at the left with neoplastic cells palisading around it (pseudopalisading necrosis*).

Here is an ependymoma arising from the ependymal lining of the fourth ventricle above the brainstem and bulging toward the cerebellum.

This horizontal (CT scan) section of the brain reveals a large ependymoma of the fourth ventricle.

Irregular posterior fossa mass (medulloblastoma) - near the midline of the cerebellum and extending into the fourth ventricle above the brainstem