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Central Nervous System 9. Tumors

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1 Central Nervous System 9. Tumors

2 Clinical Presentation of brain tumors
Headaches Seizures Nausea & vomiting Loss of consciousness Cognitive dysfunction Neurological dysfx- weakness, sensory loss, aphasia, visual spatial dysfunction

3 Types of Brain Tumors Meninges: meningioma, hemangiopericytoma
Glia: astrocytoma, oligodendroglioma, ependymoma, choroid plexus papilloma.. Vascular: hemangioblastoma. Primitive cells: neuroblastoma, germinoma, medulloblastoma, pineoblastoma, retinoblastoma Neuronal: ganglioglioma, gangliocytoma Pituitary: adenoma, craniopharyngioma Nerves: schwannoma, neurofibroma, MPNST

Primary or Metastatic Occur with equal frequency in adults, but in children primary tumors are far more common. Primary ICTs account for ~2% of cancers in adults 20% of all cancers in children. In children 70% of ICTs arise in  Posterior fossa (infra-tentorial). as In adults 70% of ICTs arise in  Supra-tentorial. Because of its location, a benign ICT may have fatal “malignant” effects. Malignant ICTs spread by: Direct infiltration of adjacent tissues May disseminate within the CNS via CSF. Gliomas account for 60% of primary ICTs Meningiomas for 20% & all others 20%. All CNS tumors behave as malignant clinically . Limited space Less than 5 years small round cell tumors eg wills, neuroblastoma leukemia's ets between 5 to 14 lymphomas and CNS tumors

5 Normal Anatomy of Brain (MRI)
Supratentorial compartment: Cerebral hemispheres Basal ganglia Thalamic nuclei Lateral ventricles Hypothalamus Corpus callosum Infratentorial compartment: Cerebellum Brain stem (MB/P/MO) 4th ventricle Sagittal Axial INTP - PPO, PHO, IAP. P2 – 6/27

6 The unique features of CNS tumors – “ICP”
1. CNS tumors- < 2% of all malignant tumors. They grow in a unique environment: the intracranial space. 2. The intracranial contents - incompressible Brain and blood contained within a rigid unyielding bony structure. 3. Intracranial pathologies (tumors, abscess, hematoma, infarction, edema, etc.) eventually produce life threatening increase of the intracranial-pressure: ICP.

7 Cytologic origin of CNS tumors
Neuro-ectodermal – most important are the Gliomas; Mesenchymal – most frequent ones are the Meningiomas; Ectopic tissues – from tissues „displaced” during embryogenesis: Ex., Dermoid cyst; Retained embryonal structures: various cysts – Paraphyseal cyst; Metastases: Lung, Breast, Melanoma, etc. in 50% of cases

8 Neuro – ecto - dermal tumors
Glial cells: astrocytes (A) - Astrocytoma Oligodendroglial cells - Oligodendroglioma Ependymal cells – Ependymoma Neurons - Gangliocytoma

9 WHO Grading System (evolves)
Low-grade WHO Grade I i.e., Juvenile Pilocytic Astrocytoma WHO Grade II i.e., Diffuse Astrocytoma High-grade WHO Grade III i.e., Anaplastic Astrocytoma WHO Grade IV i.e., Glioblastoma Multiforme

10 ASTROCYTOMAS Account for ~ 80% of primary ICTS in adults
MC in the cerebral hemispheres MC Symptoms: headaches, seizures, focal neurologic deficits ( usually in the anterior or middle) Low-grade Astrocytomas: Gross: Poorly defined gray-white infiltrative tumors. Histology: Hypercellularity; astrocytic nuclei of mild degree of atypia & astrocytic processes  fibrillary background = fingers of astrocytes

11 Low-grade Astrocytomas
Pilocytic Astrocytomas: MC in the cerebellum of children & young adults; and less commonly in the optic nerve, hypothalamic region or cerebral hemispheres Morphology: Cystic, with a tumor nodule in the wall of the cyst. Composed of bipolar astrocytes, with long hair-like processes, Rosenthal fibers & Micro-cysts+ calcification = good prognosis Grow very slowly (some patients have survived for >40 yrs after incomplete resection) & have an Excellent prognosis DD ;- not to confuse with low grade Fibrillary Astrocytoma

12 Grade I. tumor: pilocytic astro~

13 Pilocytic Astrocytoma
NB fibrially back ground

14 Pilocytic Astrocytoma

15 Rosenthal fibers

16 Gr. II. Astrocytoma Hypercellularity + nuclear atypia

17 Low-Grade Astrocytoma (Grade II)
Characteristics Slow growing Rarely spreads to other parts of the CNS Borders not well defined Common among men and women in their 20s-50s Treatment Treatment depends on the size and location of the tumor. The doctor will most likely perform a biopsy or surgery to remove the tumor. Partial resections or inoperable tumors may be treated with radiation. Recurring tumors may require additional surgery, radiation and/or chemotherapy.

18 Gr. II. astrocytoma

19 Gr. III. astrocytoma

20 Gr. III. astrocytoma

21 Gr. III. Astrocytoma Increased cellularity + nuclear atypia+ mitotic figures

22 Anaplastic Astrocytoma (Grade III)
Characteristics   Grows faster and more aggressively than grade II astrocytomas Tumor cells are not uniform in appearance Invades neighboring tissue Common among men and women in their 30s-50s More common in men than women Accounts for four percent of all brain tumors Treatment Treatment depends on the location of the tumor and how far it has progressed. Surgery and radiation therapy, with chemotherapy during or following radiation, are the standard treatments. If surgery is not an option, then the doctor may recommend radiation and/or chemotherapy. Many clinical trials (experimental treatments) using radiation, chemotherapy, or a combination are available for initial and recurrent anaplastic astrocytomas

23 Gr. IV. astro ~ = GBM Grade + giant cells

24 Glioblastoma Multiforme (GBM ) (Grade IV )
Characteristics   Most invasive type of glial tumor Commonly spreads to nearby tissue Grows rapidly May be composed of several different kinds of cells (i.e., astrocytes, oligodendrocytes) May have evolved from a low-grade astrocytoma or an oligodendroglioma (see below) Common among men and women in their 50s-70s More common in men than women Accounts for 23 percent of all primary brain tumors

25 Treatment Standard treatment is surgery followed by radiation therapy. If surgery is not an option, the doctor may administer radiation therapy. Chemotherapy is sometimes given during or after radiation therapy or if the tumor recurs. Many clinical trials (experimental treatments) using radiation, chemotherapy, or a combination are available for initial and recurrent GBM.

26 glioblastoma multiforme

27 Gr. IV. astro ~ = GBM

28 Gr. IV. astro ~ = GBM

29 GBM: necrosis/pseudo-palisade
Pseudo pallisding central necrosis with perpendicular cells

30 GBM: pleomorphic cytology

31 Gr. IV. Astro ~ = GBM Die directly

32 OLIGODENDROGLIOMA Comprise ~ 5 -15% of Gliomas
Arise in the cerebral white matter MC in the 4th & 5th decades Gross: Well circumscribed, gelatinous, gray masses, with foci of hemorrhage & calcification. Histology: Sheets of cells with rounded nuclei surrounded by a halo of clear cytoplasm (fried egg appearance). There is often a delicate network of capillaries & scattered foci of calcification (psammoma bodies)( seen in thyroid, CNS, kidneys etc). Grows slowly, presents commonly with seizures, prognosis is better than Astrocytoma, average survival is 5-10 yrs (with modern therapeutic approaches

33 Oligodendroglioma

34 Oligodendroglioma

35 Oligodendroglioma

36 Oligo-Astrocytoma

37 EPENDYMOMA Arise from the Ependymal lining of the ventricles or the central canal of the spinal cord Arise in the Fourth ventricle in children & young adults Spinal cord in the middle aged. Morphology: Highly cellular, tumor cells have regular nuclei May exhibit epithelial features with formation of “rosettes” (Flexner…) or “canals”, also perivascular pseudo-rosettes (homer …) Most tumors are well differentiated 4th ventricle tumors: May cause hydrocephalus, usually can’t be completely removed CSF dissemination may occur Average survival is ~ 4 yrs

38 Myxo-papillary Ependymomas
Arise in the filum terminale of the spinal cord Prognosis depends on completeness of surgical excision

39 Ependymoma “Rosettes” & perivascular Pseudo-rosettes

40 MEDULLOBLASTOMAS Second MC ICT of childhood (after Astrocytomas).
Occurs exclusively in the cerebellum. Derived from fetal external granular layer of cerebellum. Grows rapidly & occludes CSF flow  hydrocephalus. Seeds through CSF  implants around the spinal cord & cauda equina (need irradiation of the whole Neuraxis). Histology: Extremely cellular, anaplastic, small round or carrot-shaped cells with hyperchromatic nuclei,  N/C, may form Homer-Wright pseudo-rosettes Highly malignant, yet radiosensitive & 5-yr survival 75%.

41 Medulloblastoma

42 Medulloblastoma Homer-Wright pseudo-rosettes carrot-shaped cells

43 Usually Benign slow-growing tumors of adults, F/M 3:2
MENINGIOMAS Usually Benign slow-growing tumors of adults, F/M 3:2 Originate from meningothelial cells of the arachnoid. Usually solitary ( multiple meningiomas  NF2 ) Morphology: Firm rounded masses, adherent to the dura and compressing the underlying brain (no infiltration). Histologic variants include: Syncytial, fibroblastic, transitional, Psammomatous & papillary ( propensity to recur). Malignant Meningioma is very rare Infiltrates the underlying brain, shows marked nuclear atypia,  mitoses, & foci of necrosis. Other rare sarcomas of meninges include: Hemangiopericytoma, malignant fibrous histiocytoma & Fibrosarcoma.

44 Meningioma Easily removed with little neurological complication rarely

45 Meningioma

46 Meningothelial whorls

47 Meningioma Syncytial Psammomatous Epithelial Membrane Antigen

48 NERVE SHEATH TUMORS 1. Schwannomas: Consist of
Benign tumors of Schwann cells MC in the vestibular branch of the VIII CN at the cerebello-pontine angle (acoustic neuroma)  tinnitus & hearing loss Also involve branches of the trigeminal nerve & dorsal nerve roots Tumors are encapsulated, attached to one side of the nerve; axons do not pass through the tumor Consist of Antoni -A areas of high cellularity Nuclei form palisades “Verocay bodies” Antoni -B myxoid areas

49 Schwannoma/Acoustic neuroma

50 Antony A Antony B

51 Schwannoma Antoni – A hyprecellular Antoni –B Sparsely cellular

52 2. Neurofibromas: Benign tumors composed predominately of Schwann cells, but also containing fibroblasts & perineural cells May involve single or multiple dorsal spinal nerve roots (multiple in patients with von Ricklinghausen's disease - NF1) CN involvement is extremely rare May present as Localized fusiform enlargement of a nerve or Extensively infiltrate along the nerve  “ropy enlargement” of the nerve & it’s branches (plexiform Neurofibroma) Plexiform neurofibromas are usually part of NF1, excision is very difficult Histology: Wavy spindle shaped cells, myxoid collagenous stroma with interspersed nerve fibers

53 Neurofibromatosis - Von Recklinghausen
Dominant inheritance Multiple neurofibromas Central - CNS peripheral nerves Increased incidence of: meningioma glioma schwannoma - bilateral VIII N. Cafe-au-lait (melanosis) in skin Elephantiasis: increased connective tissue

54 Von Recklinghausen’s Disease
Café-au-lait spots Multiple neurofibromas

55 „Acoustic Neurinoma” (Schwannoma)
USAULLY bilaterally

56 METASTATIC ICTS 50% of ICTs. Common Primaries: Sites of metastases:
Broncho-genic small cell undifferentiated (oat cell) ca., Breast ca., Malignant melanoma, RCC. & Colon ca. Sites of metastases: Cerebral cortex 80%; Rest are in the cerebellum & brain stem. 50% are multiple; at the junction between the gray & white matter. Vertebral column is a common site for metastases of Breast & Prostatic carcinomas Thoracic spine 60%, Cervical 20% & Lumbar 20% Treatment : Radiotherapy

57 Metastasis Tumor material different from brain tissue

58 Brain Metastasis - (lung)

MC in children  Arising from the lateral ventricles In adults they are found MC in the 4th ventricle Present with Hydrocephalus Due to either over-production of CSF or to obstruction of the ventricular system. Consist of papillae with fibrovascular stalks covered with a cuboidal or columnar ciliated epithelium, recapitulating the structure of the normal choroid plexus.

60 COLLOID CYST OF THE THIRD VENTRICLE A non-neoplastic cystic lesion
Morphology: Having a thin fibrous capsule, a lining of Cuboidal to columnar epithelium & containing gelatinous Proteinaceous material. Attached to the roof of the third ventricle at the foramina of Munroe & may cause sudden obstruction of the CSF flow  acute non-communicating hydrocephalus  brain herniation & death Symptoms: headaches (often positional), “drop attacks”, incontinence Goblet cells are confirmatory

Pinealomas: True pineocytomas are extremely rare, may also have pineoblastomas Germinomas: MC in the pineal & suprasellar regions in adolescents & young adults Closely resemble testicular Seminomas & ovarian Dysgerminomas Other GCTs (Teratomas & Choriocarcinomas) also occur Not clear how GCTs arise within the CNS Craniopharyngiomas: Benign cystic tumors of children & adolescents Develop in the suprasellar region  Hypopituitarism Originate from remnants of Rathke’s pouch & contain squamous & columnar epithelium, calcifications are common.

62 CNS LYMPHOMA Primary CNS lymphomas Secondary CNS lymphomas
Account for ~1% of ICTs MC CNS neoplasm in AIDS & other immunosuppressed patients often arise deep within the cerebral hemispheres & are commonly bilateral Lymphoma cells exhibit an angiocentric distribution Usually are B-cell lymphomas & many appear to be EBV-related. Secondary CNS lymphomas Lymphomas arising outside of the CNS rarely involve the brain parenchyma May involve the meninges, intradural spinal nerve roots & epidural space

63 Phakomatosis (Neurocut. dysplasia)
Neurologic abnormalities + defects of skin or retina (ectodermal). Neurofibromatosis (von Recklinghausen) Tuberous Sclerosis Sturge-Weber Sy (Encephalofacial Angiomatosis) von Hippel-Lindau Disease Neurocutaneous Melanosis

Hamartomas & Neoplasms Esp. involving the nervous system & skin Mutations in tumor suppressor genes 1. Neurofibromatosis Type 1 (NF1): Neurofibromas, Neurofibro-sarcomas Optic nerve Gliomas Pigmented cutaneous macules (café au lait spots) Pigmented nodules of iris (Lisch nodules) 2. Neurofibromatosis Type 2 (NF2): Bilateral Schwannomas of CN VIII Multiple meningiomas Spinal cord Ependymomas

65 Seizures & mental retardation Extra CNS findings:
3. Tuberous Sclerosis Hamartomas (“tubers”) in the cerebral cortex, Sub-Ependymal hamartomas (“candle drippings”)  Sub-Ependymal giant cell Astrocytomas Seizures & mental retardation Extra CNS findings: Kidney (Angiomyolipoma), Heart (Rhabdomyoma MCC in kids, adult = mixomas ), skin (Angiofibroma)

66 4. von Hippel-Lindau disease
Hemangioblastomas of the cerebellum, retina, brain stem & spinal cord Cysts of liver, kidney & pancreas  incidence of RCC, may be bilateral ~ 10% of Hemangioblastomas  polycythemia

67 Peripheral nerve tumors
Schwannoma Schwann cells Compress the nerve trunk Encapsulated Easily resectable without nerve damage Microscopic: Antony A and B fibers Verocay bodies Neurofibroma Schwann cells, neurites, fibroblasts Fusiform and involves nerve trunk Not encapsulated Not resectable without sacrificing nerve Micro- Intermingled cells with wavy nuclei

68 Embryonal tumors (Primitive neuroectodermal tumors)
Neuroblastoma - cerebral hemispheres Neuroblastic (neuronal) differentiation Medulloblastoma - cerebellum Neuronal and glial differentiation Ependymoblastoma – ventricles Ependymal differentiation Pineoblastoma- pineal region Retinoblastic differentiation All commonly known as Primitive neuroectodermal tumor (PNET)

69 Histologic patterns: definitions
Whorls: onion-skinning pattern of tumor cells Psammoma bodies: laminated calcium Pseudopalisading: lining up of the tumor cells around a central necrotic area Palisade: lining up of tumor cells around their own cytoplasmic processes. No necrosis. Pseudorosette: tumor cells around blood vessels, cells equidistant from vessel walls. Rosettes: tumor cells around central lumen or fibrillary area of cellular processes

70 Tumors of Nerve Roots and Peripheral Nerves
1. Schwannoma viii Cranial nerve (Acoustic sch.) Spinal roots, posterior Peripheral nerves 2. Neurofibroma Spinal Roots, rare Peripheral nerves 3. Malignant variants Malignant peripheral nerve sheath tumor (MPNT) Rare

71 Metastatic brain tumors
Most common brain tumor in adults. Common primary sites: melanoma, lung, breast, GI tract, kidney. Most are in cerebrum (MCA territory). In gray-white junctions due to rich capillarity Discrete, globoid, sharply demarcated tumors. Amenable to surgical resection. Single or multiple. Brain edema frequent.

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