Neurosurgery: Neoplastic Disease Flashcards
Approach to:
Inferior frontal lobe and parasellar region
Bicoronal incision with unilateral/bilateral subfrontal approach
Pterional approach
Approach to:
Sellar region
Transsphenoidal
Bicoronal incision with unilateral/bilateral subfrontal approach
Pterional approach
Approach to:
Frontal lobe
Frontal craniotomy (linear, curved, or horseshoe incision)
Approach to:
Anterior temporal lobe
Temporal craniotomy (linear incision)
Approach to:
Posterior temporal lobe
Temporal craniotomy (linear, reverse question mark, or Isle of Mann incision)
Approach to:
Parietal lobe
Parietal craniotomy (linear or horseshoe incision)
Approach to:
Occipital lobe
Occipital craniotomy (linear or horseshoe incision)
Approach to:
Trigone of lateral ventricle
Appropriate craniotomy for superior parietal, middle temporal gyrus, lateral tempero-occipital, or transoccipital approach (linear or horseshoe incision)
Approach to:
Anterior third ventricle
Frontal parasagittal craniotomy and interhemispheric/transcallosal or transcortical approach
Interforniceal or transchoroidal approach once within
Approach to:
Posterior third ventricle/pineal region
Suboccipital transtentorial
Supracerebellar infratentorial approach
Interhemispheric transcallosal (splenium) approach
Transcortical parietal approach (rarely used)
Approach to:
Midline posterior fossa/fourth ventricle
Suboccipital craniotomy (linear incision)
Approach to:
Lateral posterior fossa/CPA
Retrosigmoid craniotomy (linear incision)
Approach to:
Upper clivus
Subtemporal approach and anterior petrosectomy (linear or horseshoe approach)
Approach to:
Middle and lower clivus
Combined retrosigmoid posterior temporal craniotomy and posterior petrosectomy (curvilinear incision)
What is located two finger breadths above the zygomatic arch and one thumb’s breadth behind the frontal process of zygomatic bone?
Pterion
What landmark does the asterion represent?
Asterion is on skull over the lower half of the transverse/sigmoid sinus junction
Asterion: junction of lamboid, occipitomastoid, and parietomastoid sutures
What lays at the junction of the lamboid and sagittal suture?
Lambda
What lays at the junction of the coronal and sagittal sutures?
Bregma
Where is the inion located?
At indentation below external occipital protuberance
How can you roughly estimate the location of the Sylvian fissure? (Stepwise approach)
1) Draw a line connecting the nasion and inion, find the 75% point on the line (closer to inion)
2) Mark frontozygomatic point
3) Connect the two points and the Sylvian fissure travels along this axis
- Pterion is located ~3cm behind the frontozygmatic point
How can you approximate location of Rolandic fissure (aka central sulcus)? (Stepwise approach)
1) Find upper Rolandic point (2cm posterior from 50% point on a line connecting nasion and inion)
2) Lower Rolandic point is at junction of line from upper rolandic to midzygomatic arch and sylvian fissure line (Lower Rolandic point is 2.5 cm behind pterion along Sylvian line)
How far behind the coronal suture is the motor stip usually located?
4 to 5.4 cm
What is a rough estimate of the location of the Angular gyrus (which is part of Wernicke’s area)?
Just above pinna of ear
Astrocytic tumors may be graded II, III, or IV. What are these specific types typically called?
Grade II: Low grade astrocytoma
Grade III: Anaplastic astrocytoma
Grade IV: GBM
What is standard treatment for astrocytomas?
Surgical resection (partial or complete) followed by external beam radiation and temozolomide
What are common locations for pilocytic astrocytomas?
Cerebellar
Optic glioma type
Hypothalamic hemisphere
Optic gliomas are common in what genetic condition?
What type of tumors are these typically?
NF1
Pilocytic astrocytomas
What is tx of choice for cerebellar pilocytic astrocytomas?
Surgical resection
They often have an enhancing mural nodule; if the mural nodule (contrast-enhancing) is removed the cyst wall doesn’t need to be completely taken
What is treatment of choice for pilocytic astrocytomas of optic nerve or hypothalamus?
If sparing resection can be completed then removal but if resection risky then biopsy with chemo and radiation
How do oligodendrogliomas often present?
Seizures and/or hemorrhage
What is unique about oligodendrogliomas on CTH?
They may have associated calcifcation
What chromosomal loss is associated with better response to treatment in oligodendroglioma?
Loss of 1p and 19q
Where do ependymomas often arise from?
Floor of 4th ventricle
What is preferred treatment of ependymomas?
Should you aim for full resection?
Surgical resection
Maximal resection is associated with improved survival
Given proximity to CSF space. What adjunctive test should be done in patients with ependymoma?
LP (cytology) and spinal MRI to look for subarachnoid mets
In what age group do choroid plexus papillomas often present? How do they present? What is mgmt?
<2 yo
HCP
Resection followed by chemo (radiation if carcinoma)
What are the different types of pediatric brainstem glioma (3)?
Tectal glioma
Focal tegmental mesencephalic
Diffuse pontine glioma
What is general treatment of choice for each of following types of pediatric brainstem glioma:
Tectal giioma
Focal tegmental mesencephalic
Diffuse pontine glioma
Tectal glioma: CSF diversion due to HCP risk
Focal tegmental mesencephalic: Resection, chemo/rad
Diffuse pontine glioma: radiation, experimental chemo, palliative
Angiocentric glioma
Rare, slow-growing low-grade glial often in kids/young adults with seizures. Resection often curative
Choroid glioma third ventricle
Rare low-grade glial often with HCP/chiasm compression/hypothalamic dysfunction often cured by resection
Astroblastoma
Rare low-grade glial tumor often in kids/young adults and surgically resected with adjuvant chemo/rad
What is dysplastic cerebellar gangliocytoma also known as?
What syndrome is it associated with?
How does it present and how is it managed?
Lhermitte-Duclos Disease
Cowden Syndrome (multiple hamartomas)
Inc’d ICP/HCP, cerebellar signs, slow progression
Resection
Desmoplastic infantile ganglioglioma presents in what age group?
How does it present?
What is it’s survivability?
What is optimum treatment?
<2 yo (peak 3-6 months)
Bulging fontanelles, inc’d head size, paresis, seizures
>75% survive at 15 years
Resection curative; chemo if anaplasia
In what age group do dysembryonic neuroepithelial tumors (DNETs) present?
Are they fast or slow growing lesions?
What will they commonly present with?
What is treatment?
Children and young adults (<20 yo)
Slow growing, benign
Epilepsy
Surgical resection +/- epileptogenic foci
In what age group do gangliogliomas/gangliocytomas present?
What is typical presentation?
What is mgmt?
<30 yo (peak 11 yo)
Epilepsy; slow growing, benign lesions
Resection +/- chemo if anaplastic
In what location do central neurocytomas often present?
What is presentation?
What is survivability?
What is mgmt?
Intraventricular
ICP rise and HCP; seizures
5 yr survival > 80%; benign and slow-growing
Resection; SRS/chemo for recurrence
Central liponeurocytoma often presents in what age group and what location?
What is appropriate management?
Adults; posterior fossa
Resection; possible radiation to prevent recurrence
How common is papillary glioneuronal tumor?
What is presentation?
What is mgmt?
Rare adult tumor
Seizures; slow-growing, benign tumors
Resection
How do rosette-forming glioneuronal tumors of the 4th ventricle often present?
What is mgmt?
Rising ICP and HCP with ataxia
(In young adults often)
Resection usually curative
Paraganglioma at the carotid bifurcation is known as …
Carotid body tumor
Paraganglioma at the superior vagal ganglion is known as …
Glomus jugulare tumor
Paraganglioma at the auricular branch of vagus is known as …
Glomus tympanicum
Paraganglioma at the inferior vagal ganglion is known as …
Glomus intravagale
Paraganglioma of the adrenal medulla and sympathetic chain is known as …
Pheoochromocytoma
How rapidly do paragangliomas grow and what is appx 5 year survivability?
Slow; 90%
How are paragangliomas managed?
If catecholamine secreting (i.e. pheochromocytoma) then alpha and beta blockers to prevent BP lability and arrhythmias
Resection; radiation is nonresectable
Preop embolization can reduce blood loss
What is the peak age incidence for pineocytomas? How do they present? What is survivability? What is treatment? What treatment is high risk?
10-20 yo ICP issues; Parinaud's syndrome 90% at 5 years Resection SRS is a high risk procedure for these tumors
What age group do pineoblastomas arise in?
How do they present?
What is survivability?
What is treatment?
Peak incidence 2 yo
ICP issues; Parinaud’s syndrome
Median survival 2 years
Resection + chemo + rad if > 3 yo
Why is pineoblastoma associated with worse survival than pineocytomas?
Pineoblastoma is a PNET type of tumor with associated CSF seeding in ~50% of patients
What is appropriate treatment of a papillary tumor of the pineal region?
Surgical resection followed by radiation
What is a key distinguishing feature of embryonal/primitive neuroectodermal tumors (PNETs) regarding their spread?
CSF seeding/dissemination in many patients
What are types of PNET tumors?
Medulloblastomas
CNS PNET/Supratentorial PNET
ATRT
Pineoblastomas
At what age and at what location do medulloblastomas often present?
5 years old median Posterior fossa (1/3)
How quickly do medulloblastomas present and with what signs?
Rapidly
ICP rise, HCP, cerebellar signs
If there is no metastasis and there is a gross total resection then what is the 5 yr survivability of medulloblastomas? (Hint: it depends on a genetic factor)
If ERBB-2 tumor protein negative then near 100%
If ERBB-2 tumor protein positive then 54%
What is the 5 year survival of medulloblastomas which exhibit metastasis or if there is residual postresection?
~20%
What are variants of CNS PNET tumors?
CNS neuroblastoma (including esthesioneuroblastoma) Ganglioneuroblastoma Medulloepithelioma Pineoblastoma Ependymoblastoma
What is the appx 5 year survival of CNS PNET tumors?
What factors increase chances of survival?
~ 30%
Complete resection, no metastasis, age > 2, heavily calcified lesion
What is appropriate treatment of CNS PNET tumors?
Aggressive surgical resection (since extent of resection impacts prognosis in most cases) + chemo + rads if > 3 yo
At what age do atypical teratoid-rhabdoid tumors present?
How do they present?
What is prognosis?
What is treatment?
< 3 yo
ICP elevation, developmental regression, seizures, torticollis
Median survival is 6 months
Gross total resection
How does the presentation of schwannoma differ based on age?
<30 yo more likely to be parnechymal with seizure/epilepsy presentation
> 30 yo more likely vestibular schwannoma with sensorineural hearing loss, tinnitus, and dizziness
How often do schwannomas recur?
They’re slow growing and only recur in ~ 10% of cases post-resection
For schwannomas < 3cm in diameter what are options for management?
May follow symptoms, audiology, and scans every 6 month. SRS is an option for these. Surgery can also be an acceptable option but the risk of CN VII damage or hearing loss may be high relative to risk of the small lesion
Approximately what is the chance of hearing preservation in removing a schwannoma > 3cm?
40%
Chances of hearing preservation decrease as the size of the tumor increases
There are 4 approaches to consider for removing a vestibular schwannoma. What are they and which is best and worst for hearing preservation?
Translabyrinthine (sacrifices hearing but may better preserve CN VII)
Suboccipital (may best preserve hearing)
Retromastoid
Subtemporal
Neurofibromas (including the plexiform type) often arise from where?
What condition are they associated with?
In the orbit (V1), scalp, parotid (CN VII)
NF1
Are neurofibromas fast or slow growing?
Do they recur often?
Slow growing but 2-12 % degenerate into malignant nerve sheath tumors
Recurrence is high
What is the main indication for surgical resection of neurofibromas? How likely is complete resection?
Neural compression
Complete resection nearly impossible
What is a perineurioma?
Rare, slow-growing, benign lesions growing on perineurial cells often treated with surgical removal (may be plexiform)
In cases of malignant peripheral nerve sheath tumors what adjunct to resection is there?
Chemo and radiation
What are the two most important risk factors for recurrence of a meningioma? What is the 20 year rate of recurrence?
Atypical histology
Extent of resection
20-50%
Risk of meningiomas is higher in what condition?
NF
What are examples of benign mesenchymal tumors and tumor-like lesions?
Do these need to be resected?
Lipoma, angiolipoma, rhabdomyoma, chondroma, leiomyoma, osteochondroma, benign fibrous hisiocytoma, osteoma, solitary fibrous tumor, and hemangioma
No. Sometimes they are for cosmetic reasons
Unlike benign types, malignant mesenchymal tumors and tumor-like lesions often require resection. What are some types?
What is median survival generally? (Hint: there is one exception, what is it?)
Rhabdomyosarcoma, chondrosarcoma, Ewing tumor, osteosarcoma, leiomyosarcoma, Kaposi sarcoma, liposarcoma, epithelioid hemangioendothelioma, angiosarcoma, and malignant fibroid histiocytoma
Aggressive and median survival 6-24 months
Exception is hemangiopericytoma which is 80% at 5 years
Hemangioblastoma is associated with what condition?
Hemangioblastoma makes up ____ % of posterior fossa tumors
Von Hippel Lindau Syndrome
10%
What is survivability of hemangioblastoma?
What is treatment?
85% at 10 years (slow growing)
Resection +/- preop embolization
How are primary malignant melanomas of meninges treated? What is the prognosis?
Surgical resection with chemo and radiation
Poor prognosis
What other operation may a patient with diffuse melanosis/meningeal melanomatosis require and why?
Shunt placement for HCP
If a patient presents with primary CNS lymphoma and is 30 how will your management differ than if they were 60? What if they were 10?
The younger the patient the more likely an acquired or inherited immunodeficiency state. If 10 yo consider an inherited immunodeficiency, if 30 think acquired (e.g. AIDS)
With what predominant symptom may primary CNS lymphomas often present?
Neuropsychiatric changes
How does median survival in primary CNS lymphoma change depending on treatment?
1-4 months if left untreated
1-4 years if treated
What is the treatment for primary CNS lymphoma?
No surgery usually.
High dose methotrexate for young patients
Steroid response is helpful and dramatic but may be short-lived
Where do plasmacytomas arise and why?
What is treatment and what must be ruled out and how?
Often skull due to plasma cells in marrow
Complete surgical resection but need to rule out multiple myeloma with urine and serum protein electrophoresis. There is a high risk of developing multiple myeloma
In what age group does granulocytic sarcoma arise and associated with what other malignancy?
What is median survival?
What is first line treatment?
Pediatrics, usually associated with AML
2-20 months median survival
Chemo/Rad therapy first line; surgical resection reserved for emergent mass effect
What are the 3 types pf astrocytes?
Fibrillary - in WM, PTAH, silver, GFAP pos.
Protoplasmic - in GM, larger nucleous, less citoplasm
gemistocytic - swollen, active, near injury
Circumscribed astrocytic tumors
low gr, good prognosis, frequently cystic
1. Juvenile pilocytic astr.
2. pleomphic xantoastrocytoma
3. Subependimal gian cell astr.
General attributes of pilocystic astrocytomas
2nd most common ped. brain tumor
location: cerebellum, brainstem, optic pathway!, infundibulum
age: 10 yrs
morphology: red-tan nodule, 60% cystic, nodule enhance, 10% contain calcium
survival: 5y: 86-100% 20y 70%
Histology of pilocystic astrocytomas
Growth pattern
Predominantly solid / circumscribed; often limited peripheral infiltration
Frequent extension into subarachnoid space
Biphasic appearance
Compact fibrillar portions: elongated nuclei, bipolar piloid processes, Rosenthal fibers
Loose microcystic portions: round to oval nuclei, cobweb-like processes, eosinophilic granular bodies
What is Rosenthal fiber? What pathology?
A Rosenthal fiber is a thick, elongated, worm-like or “corkscrew” eosinophilic (pink) bundle that is found on staining of brain tissue in the presence of long-standing gliosis, occasional tumors, and some metabolic disorders.
Pilocytic astr.
Pleomorphic xanthoastrocytoma general attributes
low grade astrocytoma
age: 7-25
Majority of tumors occur supratentorially, most commonly in temporal lobes (seizures!)
Often superficially located with involvement of the overlying leptomeninges
Many patients present with long history of epileptic seizures
occasionly transform to GBM
Pleomorphic xanthoastrocytoma histology
bizarre pleomorphic cells
xanthomatous fat cells
multinucleoted cells
Pleomorphic appereance histology
Subependymal giant cell astrocytoma (SEGA)
Benign, slowly growing tumor typically arising in wall of lateral ventricles and composed of large ganglioid astrocytes
Usually associated with tuberous sclerosis
Near foramen Monroe –> hydrocephalus!
Tuberous sclerosis complex
seizures, retardation, sebaceous adenomas
TSC1 Ch9 = hamartin
TSC2 Ch16= tuberin
subependymal calcification
Diffuse gliomas
fibrillary (most frequent)
protoplasmic
gemistocytic (worst prognosis
mixed
Grade: anaplasia, cellualrity, nucl. plomorffism, endothel. prolif., necrosis, pseudopalisading
- Mutations in IDH1/2, ATRX, TERT, and TP53 and co-deletion
of 1p/19q correlate better with tumor biological and clinical
behavior than do stratifications based only on histological
features
Gliomas 2021 WHO changes
Standardization with other fifth edition WHO classification systems (Neuro Oncol 2021;23:1231)
Switch from Roman to Arabic numeral system
The term “type” replaces “entity” and “subtype” replaces “variant”
Shift toward within tumor type grading
- Removal of modifier terms, such as anaplastic
- IDH mutant astrocytic gliomas were 3 separate entities under the 2016 WHO (diffuse astrocytoma, anaplastic astrocytoma and glioblastoma) but now fall under the single tumor type astrocytoma, IDH mutant, with CNS WHO grade ranging from 2 to 4
- Oligodendroglioma, IDH mutant and 1p / 19q codeleted, is assigned a CNS WHO grade of 2 or 3
- Pleomorphic xanthroastrocytoma is assigned a CNS WHO grade of 2 or 3
- Terms that are no longer recommended include diffuse astrocytoma, anaplastic astrocytoma, glioblastoma IDH mutant, anaplastic oligodendroglioma and anaplastic pleomorphic xanthoastrocytoma
Gliomas WHO 2021 new subgroups
New tumor type groupings
- Adult type diffuse gliomas
- Pediatric type diffuse low grade gliomas
- Pediatric type diffuse high grade gliomas
- Circumscribed astrocytic gliomas
New glioma types
- Diffuse astrocytoma, MYB or MYBL1 altered
- Polymorphous low grade neuroepithelial tumor of the young
- Diffuse low grade glioma, MAPK pathway altered
- Diffuse hemispheric glioma, H3 G34 mutant
- Diffuse pediatric type high grade glioma, H3 wildtype and IDH
wildtype
- Infant type hemispheric glioma
- High grade astrocytoma with piloid features
Adult type diffuse gliomas astrocytoma, IDH mutant
IDH1 / IDH2 mutated,diffusely infiltrating glioma, most often with concurrent TP53 or ATRX mutations and without 1p / 19q codeletion
Can be graded CNS WHO grade 2, 3 or 4
IDH1 codon 132 or IDH2 codon 172 mutated, diffusely infiltrating glioma without 1p / 19q codeletion and usually with TP53 or ATRX mutations
In the absence of 1p / 19q codeletion, a component that morphologically resembles oligodendroglioma is compatible with this diagnosis
Can be designated CNS WHO grade 2, 3 or 4 depending on presence of mitotic activity, nuclear atypia, pleomorphism, necrosis, microvascular proliferation or CDKN2A / CDKN2B homozygous deletion
Significant proliferative activity is consistent with a CNS WHO grade 3 diagnosis
Presence of either necrosis, microvascular proliferation or CDKN2A / CDKN2B homozygous deletion is consistent with a CNS WHO grade 4 diagnosis
Infiltrating gliomas histology
Typical histologic features of infiltrating gliomas. In diffuse astrocytomas (a), cellularity is increased due to infiltrating neoplastic astrocytes with irregular, hyperchromatic nuclei and scant associated cytoplasm. Immunohistochemistry for IDH1 R132H mutant protein (A, inset) can be helpful when infiltrating cells are sparse or rare. b Anaplastic astrocytoma is distinguished by mitotic activity (black arrow). Note the infiltrating tumor cells around a non-neoplastic neuron (white arrowhead). c Palisading necrosis (left) and endothelial proliferation (upper right) are histologic features of glioblastoma, though neither feature is absolutely specific.
Gemiostocytic astrocytoma
worst prognosis
Gemistocytes are polygonal
cells with peripherally
displaced nuclei and glassy
cytoplasm, as well as coarse
processes. Despite the tumor’s
aggressive behavior, mitoses
are usually hard to find
Genetic alteration in gliomas
Oligodendroglioma, (IDH mutant, 1p / 19q codeleted)
CNS WHO 2021 definition: diffusely infiltrating glioma with IDH1 or IDH2 mutation and codeletion of chromosome arms 1p and 19q (CNS WHO grade 2 or 3)
Essential features
Diffusely infiltrating glial neoplasm with IDH1 or IDH2 mutation and 1p / 19q whole arm codeletion (both features are required for diagnosis)
Morphology resembles nonneoplastic oligodendrocytes with round monotonous nuclei and perinuclear halos
Chicken wire vasculature, microcalcifications and microcysts are characteristic
Median overall survival: 11.6 years; 10 year overall survival rate: 51 - 63%
Unfavorable features:
Contrast enhancement on MRI
CNS WHO grade 3 histology
CDKN2A / CDKN2B homozygous deletion
Local recurrence and malignant transformation are common
Classic oligodendroglioma features
perinuclear halo = fried egg (= citoplasma retractio –> artefitial! not in frozen sample)
present with seizure
higher frequency of hemorrhage
have calcification
Greenberg:
● slow growing tumor that frequently presents with seizures
● occur primarily in adults, predilection for the frontal lobes (F>T)
● by definition: a diffusely infiltrating glioma with codeletion of BOTH chromosome arms 1p AND
19q, AND mutation of IDH1 OR IDH2
● histology: classic features of “fried egg” cytoplasm (on permanent pathology) & “chicken wire” vasculature are unreliable. Calcifications are common
● recommended treatment: as for WHO grade II astrocytic tumors
Gliosarcoma
Rare, classic variant of glioblastoma (GBM), WHO grade 4
Biphasic glial and mesenchymal differentiation
2% of GBM, 40-60y, temporal lobe, dural invasion
GBM component GFAP stains
intra-extracranial metastasis
Sarcoma: from vascular structures
Median overall survival with treatment (both primary and secondary gliosarcoma): 17.5 months (J Neurooncol 2015;125:401)
Median overall survival with treatment: 24.7 months
Optic glioma
Relatively rare
Slow growing tumor within orbital segment of optic nerve
Usually ages 0 - 9 years with symptoms of minimal exophthalmos, optic nerve atrophy or papilledema
Associated with neurofibromatosis type 1
Glioblastoma subtypes
- giant cell GBM
- gliosarcoma
- epitheloid GBM
Glioblastoma, IDH wild type
An aggressive, infiltrating, astrocytic glioma that lacks mutations in IDH1, IDH2 and histone H3 genes and is:
Histologically defined by brisk mitotic activity and microvascular proliferation or necrosis
Or molecularly defined by the presence of TERT promoter mutation, EGFR gene amplification or copy number changes in the form of combined gain of chromosome 7 and loss of chromosome 10
TERT, EGFR, +7/-10 no IDH!
Necrosis in GBM
GBM favorable factors
- KPS >80
- younger age
- IDH1 mut
- MGMT promoter methylation (unmethlyated medisan OS 12.2 m; methy.: median OS 18.2m)
worst: IDH wild, MGMT unmeth., biopsy, 50y older
RANO criteria
RANO criteria is a set of guidelines used to assess treatment response in patients with glioblastoma, a type of brain cancer. The criteria include four categories: complete response, partial response, stable disease, and progressive disease. These categories are based on changes in tumor size, enhancement, and clinical status.
MR spectroscopy
ChoCrNALaLi
CHOCNALALI
lactate: endproduct of anaerob glycolysis (hypoxia)
N-acetyl-aspartate: neuronal marker, tallest peak (csökkent in ALL abnormalities)
cholin: marker of membrane synthesis (stroke is low)
GLIOMAS:
higher lactate, lipid, cholin, lower NAA (higher the choloin, higher the grade!)
ABCESS: low NAA, Cr, CHo; atypical paeks (from baci –> succinate, acetate…), lavetate can be elevated
Glioma MRS
csökk NAA; növ: lactate, lipid, cholin
Ependymomas (of post. fossa) key concepts
● usually benign tumors, often fibrillary with epithelial appearance. Perivascular pseudorosettes or
ependymal rosettes may be seen
● most often occur in the floor of the 4th ventricle, presenting with hydrocephalus (increased ICP)
and cranial nerve VI & VII palsies
● evaluation: includes imaging the entire neuraxis (MRI with and without enhancement: cervical,
thoracic, lumbar & brain) because of potential for seeding through CSF
● worse prognosis the younger the patient (especially age < 24 months)
● treatment: the best outcomes are associated with gross total removal (no enhancing tumor on
post-op MRI) followed by XRT. XRT may be withheld for age < 3 years due to side effects
● do LP ≈ 2 weeks post-op to send ≈ 10 cc of CSF for cytology for prognostication
Ependymomas general
Essential features
Well circumscribed tumor of ependymal differentiated cells that occurs in the supratentorium, posterior fossa and spinal cord
Bimodal age distribution of children and adults, occurring equally between genders
Is classified and prognosticated by location, histology and molecular and methylation studies
Supratentorial ependymomas can have ZFTA fusions or YAP1 fusions; posterior fossa ependymomas are split into posterior fossa group A (PFA) and posterior fossa group B (PFB) by methylation profile; spinal ependymomas can have MYCN amplification
Specific ependymoma types
- supratentorial
- supratentorial ZFTA-fusion + (ZFTA fused w/ RELA)
- supratentorial YAP1 fusion +
- posterior fossa
- posterior fossa Group A
- posterior fossa Group B
- spinal ependymoma
- spinal ependymoma MYCN amplified
- myxopapillary ependymoma
- subependymoma
Ependymoma types 2021 WHO
Overview of key characteristics and diagnostic criteria of the distinct ependymoma tumor types as proposed by the 2021 WHO Classification of CNS tumors. Range of age (in years) at onset of disease is indicated in black, the median age of onset is highlighted with a red triangle. PFS and OS are rated on a scale of green (very low progression rate and good OS), orange (intermediate PFS and OS) to red (high progression rate and dismal survival prognosis). For SE, we highlighted that tumors with supratentorial or spinal location have a low progression rate. In contrast, SE of the PF have a higher tendency to progress. Typical molecular features described to date are indicated for each tumor type. Obligatory criteria for the diagnosis of all ependymoma types are morphological and immunohistological features of ependymoma. Additional obligatory criteria are listed for each tumor type. Also, the 2021 WHO Classification provides desirable criteria for the diagnosis of each tumor type that can support the diagnosis. CNP, copy number profile; MPE, myxopapillary ependymoma; NEC, not elsewhere classified; NOS, not otherwise specified; OS, overall survival; PF, posterior fossa ependymoma; PF-A, group A posterior fossa ependymoma; PF-B, group B posterior fossa ependymoma; PFS, progression free survival; SE, subependymoma; SP-EPN, spinal ependymoma; SP-MYCN, MYCN-amplified spinal ependymoma; ST, supratentorial ependymoma; ST-YAP1, YAP1-fusion positive ependymoma; ST-ZFTA, ZFTA-fusion positive ependymoma
Ependymoma mutations and locations
Ependymal rosettes
true rosettes
perivascular rosettes
Choroid plexus papilloma
Rare intracranial tumor arising in the ventricle, mainly occurring in children
3 histological grades (WHO grade 1, 2, 3): choroid plexus papilloma (CPP), atypical choroid plexus papilloma (aCPP), choroid plexus carcinoma (CPC)
Histological classification is based on architecture (preservation of papillary pattern), cellular density, cytology (nuclear pleomorphism), proliferation (mitoses) and necrosis / brain invasion
Diagnostically, transthyretin (TTR), KIR7.1, cytokeratin and Ki67 immunohistochemistry are most helpful
Based on methylation profiling, these tumors are now categorized in to 3 subtypes
In line with other CNS tumors, integrated phenotype genotype diagnosis is preferred, which will guide appropriate management
Choroid plexus papilloma histology
Choroid plexus papilloma are benign tumors with preserved papillary or finger-like architecture, with fibrovascular cores, lined by single layer of cuboidal to columnar epithelium, almost resembling nonneoplastic choroid plexus but with mild cellular atypia and without significant mitotic activity.
Choroid plexus carcinoma
mostly children
lat ventricles
40% @ LiFraumeni sy –> TP53 mutation
