Neoplastic Disease Flashcards
Where is the pterion?
located at the junction of the frontal, parietal, temporal, and greater wing of sphenoid bones It is located two fingerbreadths above the zygomatic arch and a thumb’s breadth behind the frontal process of the zygomatic bone
Where is the asterion? What does it signify?
located at the junction of the lambdoid, occipitomastoid, and parietomastoid sutures lies on top of the lower half of the transverse/ sigmoid sinus junction
What is the bregma?
located at the junction of the coronal and sagittal sutures
What genetic mutations are characteristic for IDH mutant diffuse astrocytomas?
ATRX loss P53 mutation
Pilocytic astrocytomas on MRI imaging generally are ________ but when in the optic nerve, are generally _____.
cystic with mural nodule solid and enhancing
What disease is associated with pilocytic astrocytoma?
NF-1
Describe the epidemiology and symptomology of pilocytic astrocytomas based on location.
What is the 5 and 10 yr survival for PXAs?
80 and 70%
What is the most common location for oligodendrogliomas?
frontal lobe (50%)
Most GBMs that develop from other astrocytomas are _____.
IDH mutants
What is the Stupp protocol?
maximal safe resection followed by adjuvant chemotherapy/radiation
fractionated radiation of 2 Gy 5 days/week for six weeks total 60 Gy, plus daily temozolomide at 75 mg/m^2, 7 days a week for 60 days followed by six cycles of adjuvant temozolomide 150-200 mg/m^2 for 5 of 28 days
In the fourth ventricle, ependymomas usually arise from the _____.
floor
Spinal ependymomas are associated with a syrinx in what percent of cases?
90%
“Tiger stripes” in unilaterally in the cerebellum are pathognomonic for _____.
Lhermitte Duclos disease
What is the treatment for Lhermitte Duclos disease?
surgical resection is curative
What is the chance for seizure freedom after DNET resection?
80% at 5 years, 60% at 10 years
What’s the difference between a gangliocytoma and ganglioglioma?
Gangloicytoma: WHO grade I growth of mature neurons
Ganglioglioma: WHO grade I and II mixed population of ganglion and glial cells
What is the semiology of hypothalamic hamartomas? What other symptoms can they classically present with?
gelastic seizures
precocious puberty
What are the different types of paraganglioms? Provide name and location.
What is the overall 5 year survival rate for paragangliomas?
90%
What percent of pineoblastomas have CSF seeding?
50%
What is the treatment for pineoblastoma?
surgical resection + cranial/spinal radiation and chemotherapy
What is the survival rate in pineoblastoma?
Fifteen percent 10-year survival in case of residual tumor versus 100% if no residual left
What chromosome abnormality can be seen with medulloblastoma?
loss of 17p in 50% of patients
What is the 5 year survival for medulloblastoma in standrard risk patients (no mets, older than 3 years, Gross total resection)?
100% if ERBB-2 tumor protein negative, 54% if positive
There are many types of meningiomas. What are they and what WHO grade system do they fall under?
WHO Grade I: meningothelial, fibrous, transitional, psammomatous, angiomatous, microcystic, secretory, metaplastic, lymphoplasmacyte-rich.
WHO Grade II: atypical, clear cell (predilection for spinal cord and posterior fossa), chordoid (“chordoma-like”), brain invasive.
WHO Grade III: anaplastic, rhabdoid, papillary.
Describe the Simpson Grading system.
What is the most common primary CNS sarcome (malignant mesenchymal tumor)?
fibrosarcoma
Soitary fibrous tumors are distinguished form hemangiopericytomas and meningiomas by _____.
CD 34 +
Leiomyosarcomas are associated with what virus?
EBV
What is the median survival in primary CNS lymphoma?
Median survival 1–4 months without treatment, 1–4 years when treated (2–6 months in AIDS)
What is the treatment for germinomas?
biopsy followed by chemo/radiation
Klinefelter’s syndrome is associated with what CNS tumor?
embryonal carcnomas
What percent of pituitary adenomas are micor vs macor?
50-50 split
“Stalk effect” elevated prolactin levels fall into what range?
25 - 150 ng/mL
For adrenocorticotrophin releasing pituitary adenomas, what medication can be used as a second line treatment?
ketoconazole
Summarize the the most common mets, percent with cerebral mets, and general survival with treatment.
What were the results for the Radiation therapy Oncology Group brain metastases trial?
Class 1: KPS > or = 70, < 65 years old, controlled primary with no extra cranial metastases— median survival 7.1 months.
Class 3: KPS < 70—median survival 2.3 months.
Class 2: all others—medial survival 4.2 months
What is the difference in possible meningitis complications in dermoids vs epidermoids?
Epidermoid: aseptic Mollaret recurrent meningitis
Dermoid: septic meningitis
What is the most common location for chrodomas?
Sacrum (50%) and clival (35%)
What type of radiation is most effective for chordoma?
proton beam
