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
