NEURO INTRO (1) ONCOLOGY (BRAIN - GENERAL) ............................................................................................................. 1 EPIDEMIOLOGY ...................................................................................................................................... 2 DIAGNOSIS ............................................................................................................................................. 3 PATHOLOGY ........................................................................................................................................... 4 GENETICS ............................................................................................................................................... 5 IMMUNOHISTOCHEMISTRY TUMOR MARKERS / STAINS .......................................................................... 6 TREATMENT ........................................................................................................................................... 6 PROGNOSIS ............................................................................................................................................ 8 NEUROLOGICAL PARANEOPLASTIC SYNDROMES ................................................................................... 8 ONCOLOGY (BRAIN – TUMOR TYPES) .................................................................................................... 8 METASTASES ......................................................................................................................................... 8 PRIMARY CNS LYMPHOMA .................................................................................................................. 11 HISTIOCYTIC TUMORS .......................................................................................................................... 13 ASTROCYTOMA .................................................................................................................................... 13 GLIOMATOSIS CEREBRI ........................................................................................................................ 18 OLIGODENDROGLIOMAS ...................................................................................................................... 18 EPENDYMOMA ..................................................................................................................................... 20 CHOROID PLEXUS PAPILLOMA ............................................................................................................. 21 PNET, MEDULLOBLASTOMA ................................................................................................................ 22 ATYPICAL TERATOID/RHABDOID TUMOUR (WHO GRADE IV) .............................................................. 24 TUMORS OF NEURAL CREST CELLS (NEURON TUMORS) ........................................................................ 24 VASCULAR TUMORS ............................................................................................................................. 32 PITUITARY ........................................................................................................................................... 35 PINEAL TUMORS .................................................................................................................................. 38 GERM CELLS ........................................................................................................................................ 40 (EPI)DERMOIDS ................................................................................................................................... 42 COLLOID CYSTS ................................................................................................................................... 42 ARACHNOID CYSTS .............................................................................................................................. 42 NEOPLASTIC MENINGITIS ..................................................................................................................... 42 MENINGIOMA ....................................................................................................................................... 43 CHORDOMA.......................................................................................................................................... 45 SKULL TUMORS .................................................................................................................................... 46 IDIOPATHIC (BENIGN) INTRACRANIAL HYPERTENSION = PSEUDOTUMOR CEREBRI .............................. 47 ONCOLOGY (SPINE) ............................................................................................................................... 47 INTRAMEDULLARY ............................................................................................................................... 47 INTRADURAL EXTRAMEDULLARY ........................................................................................................ 52 EXTRADURAL / VERTEBRAL ................................................................................................................. 52 ONCOLOGY (PNS, CRANIAL NERVES) ................................................................................................... 60 SCHWANOMA ....................................................................................................................................... 60 NEUROFIBROMA ................................................................................................................................... 62 MALIGNANT PERIPHERAL NERVE SHEATH TUMOR ................................................................................ 62 PARAGANGLIOMAS .............................................................................................................................. 63 ONCOLOGY (BRAIN - GENERAL) ≈ 1/3 brain tumors can be called BENIGN (mainly extra-axial tumors - meningiomas, acoustic neuromas). tumor mass of 30-60 g (3-6 × 1010 cells) usually produces neurologic symptoms. brain cancer is lethal when tumor + edema reaches 100 g (vs. ≈ 1000 g in systemic cancers). immune system per se can kill only ≈ 0.0001 g, or 1 × 105 glioma cells. KARNOFSKY performance scale - objective measurement of functional ability: 100 – Normal (no evidence of disease) NEURO INTRO (2) 90 – Minor symptoms (able to carry on normal activity) 80 – Some symptoms (normal activity with effort) 70 – Unable to carry on normal activity (cares for self) - justifies aggressive therapy! 20 – Active supportive treatment needed (very sick) 10 – Moribund WHO performance scale EPIDEMIOLOGY 1-2% of all cancers. 13% of all cancer deaths. 6th most common cancer in adults. after leukemia, 2nd most common cancer in children [20% pediatric tumors]! two peaks of incidence: small peak in childhood (predominance of embryonal CNS neoplasms and relative absence of gliomas) → much higher peak in 60-80 years (predominance of supratentorial gliomas) men ≥ women (except meningiomas ← women : men = 2:1). HEREDITARY SYNDROMES → see here >> !!!!!! WHO CLASSIFICATION see Onc1 >> FREQUENCIES Most common primary CNS tumors in adults: 1. Glioblastoma multiforme (50-60%) 2. Meningioma 3. Astrocytoma 4. Pituitary adenoma, Vestibular schwannoma Most common primary CNS tumors in children: 1. Medulloblastoma (10-30%) - propensity to dissemination via CSF! exquisitely radio & chemosensitive 2. Astrocytoma (esp. cerebellar pilocytic astrocytoma) (20%) NEURO INTRO (3) 3. Glioblastoma multiforme (20%) 4. Ependymoma, Craniopharyngioma Most common primary CNS tumors in children (< 2 yrs): 1. Medulloblastoma 2. Ependymoma 3. Low-grade gliomas (esp. midline pilocytic astrocytomas) Childhood cancers: 1) leukemias 2) CNS tumors 3) lymphomas 4) neuroblastomas LOCATION – adults 70% supratentorial – children (2-12 yrs) 70% infratentorial – infants (< 2 yrs) and adolescents (> 12 yrs) 50/50% Age Posterior Fossa Meninges Adulthood Metastases, Meningioma, hemangioblastoma CN8 schwannoma, metastases, lymphoma Childhood Medulloblastoma!!!, Leukemia, lymphoma ependymoma, cerebellar pilocytic astrocytomas, brain stem astrocytoma, choroid plexus tumor Intraventricular tumors – see here >> Sellar and parasellar tumor – see here >> Tumors that spread via CSF: HIGH-GRADE GLIOMAS (10-25%) PRIMITIVE NEUROECTODERMAL TUMORS, incl. MEDULLOBLASTOMAS (10-20%) EPENDYMOMAS (12%) CHOROID PLEXUS CARCINOMAS OLIGODENDROGLIOMAS (1%) PINEAL GERMINOMAS (rare). Tumors that tend to bleed – see here >> DIAGNOSIS Oral boards: Do physical exam + labs (do not jump to imaging!) Spend money – ask for MRI!!!!! T1 - well-demarcated area of low density. T1 with gadolinium - most precise way to image brain tumor! NEURO INTRO (4) T2 - brightness in more extensive region (signal of surrounding brain edema); tumors that are hypointense on T2: METASTATIC MELANOMA (paramagnetic properties of melanin) DERMOID (due to fat) COLLOID CYSTS INTRATUMORAL HEMORRHAGE MENINGIOMAS are usually isointense on all image sequences!!! DW-MRI - tumors show diffusion restriction! (due to hypercellularity and proteinaceous stroma) PW-MRI: – markedly increased rCBV - excess vascularization (growth of high-grade tumors); – increased rCBV - low-grade tumors; – decreased rCBV - vasogenic edema or radiation necrosis. contrast enhancement is sign of malignancy! (exceptions exist). Tumors that enhance strongly: benign tumors (meningiomas, CN neuromas, pilocytic