Tumors found in kids?
What is a central Neurocytoma?
Intravenctricular --> LATERAL VENTRICLE tumor with neuronal differentiation
see small benign looking cells that stain with Synaptophysin
von Hippel Lindau presentation
Hemangioblastomas of CNS!!!!
Renal Cell CArcinoma
Microcytic Adenomas of Pancreas!!!!
If you see hemangioblastoma in younger patients, check kidneys and adrenals
Presentation of Oligodendrogliomas and appearance?
Typically adult patients and likes to grow in frontal lobes and White Matter and can cause seizures
Grows in White Matter, Calcifications!
See: Rounded cell contours with perinuclear halos = Fried Eggs
See: Plexiform background network = Chicken wire
What is a Craniopharyngioma? PResentation and cytology?
More common in children but Bimodal Distribution
Benign tumor from remnants of Rathke's Pouch with both Cystic and Solid portions = Pharyngeal invaginations
Tx: Surgery Trans-sphenoid gives higher GTR
Radiation therapy after sub-total resection
What the HECK is a Dysembryopastic Neuroepithelial Tumor (DNT)?
Mixed Neuronal and Glial Tumor
Commonly presents in younger patients with Long history of partial complex seizures
*Young ppl with epilepsy --> Check Temporal lobe
What are the features of a Medulloblastoma? How is it staged? Treatment?
See granular cells of the cerebellum - smal, round, blue cells
Homor Write Pseudorosette with fibrillary processes into the lumen
Grade 4 tumor that can be cured
Staging is either "average" or "high risk"
Treatment for both stages is Resection and External bean radiation therapy
can also add chemotherapy
What are the different types of pituitary adenomas?
Prolactinomas - most common
GH-producing adenomas (Somatotrophs)
ACTH- producing adenomas (somatotrophs)
TSH-producing adenomas (thyrotrophs)
Treatment for Prolactinoma?
Bromocriptine and Cabergoline = Da Agonists and radiation therapy
What is a grade 1 astrocytome? who gets it? what you do see?
Pilocytic Astrocytoma - not diffuse!
Benign tumor in children and young adults that presents in the Cerebellum
Cysts with Mural Nodule
Rosenthal Fibers - protenacious accumulations of intermediate filaments in astrocites (thick eosinophilic processes, GFAP+)
Biphasic PAttern - loose textured microcytic material next to dense eosinophilic material with hair-like processes
What are the different treatment options for meningioma?
Observation if asymptomatic
Surgery - GTR if possible
+ Post-op RT for progression free survival if sub-total resection
What are the gross and microscopic findings in Glioblastoma Multiforms?
Circumscribed appearance (from necrosis and hemorrhage) that is an ENHANCING tumor on MRI with surrounding edema (mass effect)
Glioblastoma with Butteryfly Pattern crossing the Corpis Collosum
Vascular Proliferation and Necrosis with Pseudopalisading
What are the microscopic indications for Meningioma?
What are the genes?
see Whorls and Psammoma Bodies
Loss of 22q12 gene for Merlin/Schwannomin Tumor Suprpresor
Von Hippel Lindau Disease inheritance and genetics?
Autosomal Dominant loss of tumor suppressor gene on Chromsome 3p25.3 (involved in HIF1alpha metabolism)
What is the most common source for mets to the brain? Where do Mets go? what do they look like?
Mets are usually multiple and live at the Gray-White Junction
see Circumscirbed lesions with Vasogenic Edema
What are treatments for Brain Mets?
Whole Brain RT + resection if single mets or need emergent decompression
[Chemotherapy limited bc poor BBB permeability]
Treatment for ACTH producing tumors?
Mitotane, Ketoconazole, Metapyrone
NF2 inheritance and genes, presentation?
Autosomal Dominant loss of chromosome 22q12.2 Merlin/Schwannomin tumor suppressor gene
Bilateral CN 8 Schannomas (tx Bevacizumab)
Also associated with Ependymomas and meningiomans
What is a myxopapillary ependyoma? who gets it? how does it present?
Grade 1 Ependyoma
Benign and typically seen in young adults in the Filum Terminale
*Back Pain in young adults
TX: Surgery curative
Can see ECM and ground substance in the tumor
What is the genetic mutation in pilocytic astrocytoma?
7q34 Duplication producing BRAF fusion
All kids are going to be
What is the mutation sequence for gliomas?
First TP53 mutation
Then IDH-1 Mutation which is low grade 2/3 tumors of astrocytes or oligos and indicates favorable prognosis and marker for late benefit from chemotherapy
1p/19q = Oligodendrogliomas (predictive and prognostic marker)
ATRX mutation = Astrocytomas
Primary vs Secondary Glioblastoma?
Primary (de novo) = Older patients and increased EGFR gene amplifications; tend to be Grade 4 at presentation
Secondary = younger patients and have progression from low grade to high grade (TP53 --> IDH1)
What is a Schwannoma? Presentaiton?
Benign tumor of vestibular nerve sheath
*Unilateral hearing loss/ringing/tinnitis in adults!!!
Grow eccentrically around CN8 - acoustic schwannoma or neuroma
If Bilateral = Think NF2!!!
PRognostic/Predictive variable in Glioblastoma?
MGMT Methylation is Prognostic - patients do poorer if unmethylated no matter treatment
and Predictive - patients do better if methylated with treatment of Temozolomide
What is a Hemangioblastoma? Who gets it?
Where does it occur? what does it look like?
Adult tumor in Cerebellum (*May present with polycythemia - from EPO production)
either sporadic or familial with Von-Hippel Lindau
see Cyst with mural nodule in cerebellum
Lipidized stromal cells with Foamy Cytoplasm
What are the treatments for Glioblastoma? What genetics are predictive?
Methylation of MGMT = convers improved progression free survival in pts with radiation and Temozolomide (alkylating agent)
-If tumor has methylated MGMT then it can't function properly to remove DNA damage from Temozolomide and it's more effective
GTR almost impossible so Tx with Radiation + Temozolomide
New Therapy Novo TTFields - low amplitude electric currents disrupts interface with cell division (similar to microtubule poison)
Genetics of Tuberous Sclerosis
Autosomal Dominant mutations in TSC1 (Hamartin) or TSC2 (tuberin)
Chromosomes 9 and 16
NF1 genetics, Inheritance and presentation?
Autosomal Dominant loss of chromosome 17q11.1 (neurofibromin down regulates p21ras)
See Plexiform Neurofibroma - tumor that expands in the nerve in sheets and fascicles
Associated LEsions: cafe au lait spots, Pheochromocytoma, gliomas of optic nerve and hypothalamus
Differential Dx for blob tumor in ventricle?
Choroid Plexus Tumor
What are the treatment options for schwannoma?
Observation if growing/shrinking
Surgery but preservation of facial nerve and hearing is difficult
Radiosurgery of tumors
Bevacizumab used for bilateral tumors to reduce size and preserve hearing
PRognostic and Predictive Markers for Oligodendrogliomas?
1p/19q Codeletion = Prognostic Marker (improved progression free survival)
= Predictive Marker (chemosensitivity - esp grade 3 tumors)
Codeletion is a molecular marker and you do better if you have the deletion no matter what!!!
What is a Meningioma? Who gets it? Where does it occur?
Meningioma is the most common benign primary brain tumor in adults
Grade 1 are majority and Grade 2 or 3 are rare
Adults get it - incidence increases with Age
FEMALES > Males (hormone receptors on tumor to E/P - can grow in pregnancy)
seen in the dural attachment but derived from meningothelial cells in arachnoid "arachnoid cap cells"
Well-circumscribed mass in dura
What are the tumors of the choroid plexus?
Choroid plexus papilloma = young patients, low grade benign; Intraventricular
Choroid plexus Carcinoma = higher grade, can be seen in infancy
Presentation of Tuberous Sclerosis
Skin lesions, Seizures, Mental REtardation
Skin findings ( adenoma, sebaceous, macules and patches etc)
Renal Angiomyolipomas, Pulmonary lymphangiomyomatosis and cardiac rhabdomyomas
aka Kindey, Lyng, and Heart tumors
See Subependymal Nodules = Candle Wax
Cortical Tubers = potato cortex that causes seizuers
What is an Ependymoma? What is it's presentation? What do you see in it? Genetics?
Tumor of Ependymal cells --> Grade 2 or 3
Presentation: KIDS in 4th ventricle and get hydrocephalus or Adults in Spinal Cord
*Deletion of Chromosome 22
See: True Ependymal rosettes (true lumen) and Perivascular Pseudorosettes
Tumors in the Cerebellum
What is a medulloblastoma? Who gets it? Where does it occur?
Primitive Neuroectodermal Tumor (PNET) of the Cerebellum = Neuroectoderm Granular cells of the cerebellum turn into a tumor
Malignant tumor in Kids (men>women, Whites>blacks)
Cerebellum and can seed from 4th ventricle to spine = Drop Mets wihch are bad!!!!
Treatment for GH-producing ademonas?
Somatostatin analogs like Ocreotide and Lanreotide
Pregvisomant - IGF inhibiotr $$$$
Who gets pituitary ademonas? How do they present?
Females > MAles ages 45-55
Majority are benign; occur midline in the Sella Turcica
Diplopia is the most common presentation bc they press on the optic chiasm
ALL are anterior pituitary - no posterior lobe tumors
Can be Microadenomas Macroademoas >10mm or Giant Adenomas >40mm
What is the cytology of a Schwannoma? What are the genetics?
Antoni A with Verocay Bodies (pink with palisading organization of cells) and Antoni B (pale staining)
If bilateral, think NF2 inactivating mutations of Tumor Sppressor gene on 22q12 = MERLIN protein
What tumors present with Cysts with Mural Nodules?
PCA - kids in cerebellum; chrom 7 duplication BRAF, Biphasic, rosenthal fibers, benign
Hemangioblastoma - adults sporadic or Von Hippel, lipiized stromal cells with Foamy cytoplasm, increase EPO - polycythemia,
What is the difference between a Grade 2 and Grade 3 Astrocytoma?
What is the difference between a Grade 3 and Grade 4?
Grade 2 = can be treated with Maximum Safe resection and Adjuvant ChemoRT to prolong progression free survival
Grade 3 = Increased Mitotic Activity!!!! Increased cellularity and pleomorphism
Grade 4 = Vascular Proliferation and Necrosis - see necrosis and pseudopalisading areas; enhancing tumor on MRI with surrounding edema (mass effect)