Neuro-oncology Flashcards
Most common primary brain tumor
Astrocytoma, of which grade IV (GBM) is the most malignant type
Location of ependymomas
Occur anywhere throughout the ventricular system or spinal canal, but is particularly common in the 4th ventricle and cauda equina
Uncommon cause of hydrocephalus due to excessive CSF production
Choroid plexus papilloma
Common location of PNETs
Commonly infrantentorially in children, where they are called medulloblastomas and found in the cerebellar vermis
Two common histopathological characteristics of meningiomas
Whorls of meningothelial and spindle cells, calcified psammoma bodies
Common locations of hemangioblastomas
Cerebellar parenchyma or spinal cord
Von Hippel-Lindau syndrome
Cerebellar or spinal cord hemangioblastomas with similar tumors in the retina and cystic lesions in the pancreas and kidney
Common location of primary CNS lymphoma
Forms around periventricular parenchymal blood vessels
Tumor marker for metastatic carcinoma
Cytokeratin
Tumor marker for neuronal tumors
Synaptophysin
Extrinsic tumors found in the posterior fossa
Schwannoma (VIII, V), meningioma, epidermoid/dermoid cyst, arachnoid cyst, mets
Intrinsic tumors found in the posterior fossa
Mets, hemangioblastoma, medulloblastoma, astrocytoma
Most common primary cerebellar tumor in adults
Hemangioblastoma
Imaging characteristics of hemangioblastomas
Strongly enhancing solid tumor in the cerebellum or a tumor nodule lying in the wall of a well defined cystic lesion
Most common location of medulloblastoma
Arise in the cerebellar vermis and usually extend into the 4th ventricle
Imaging characteristics of medulloblastoma
Isodense midline lesion in the cerebellar vermis, compressing and displacing the 4th ventricle and enhancing strongly with contrast
Most common location of cerebellar astrocytoma
Usually lie in the cerebellar hemisphere or vermis but occasionally extend through a peduncle into the brain stem
Many have cystic components
Imaging characteristics of cerebellar astrocytoma
Often a low density cystic area abuts or encircles the tumor mass
Most common location of epidermoid/dermoid cysts
Commonly found in the posterior fossa
Imaging characteristics of meningiomas
On T1 MRI - isointense with brain and diffusely enhance
Most common primary tumors with hemorrhage
GMB, oligodendroglioma, pituitary adenoma
Most common metastatic tumors with hemorrhage
Lung, melanoma, thyroid, renal, choriocarcinoma
Pneumonic for hemorrhagic tumors
CT MRB
Choriocarcinoma, thyroid, melanoma, renal, breast/bronchogenic
Pneumonic for ring-enhancing lesions
Magic Dr. L
Mets, abscess, GBM, infarct, cyst, demyelinating, radiation, lymphoma
Most common malignant brain tumor in children
Medulloblastoma
Medulloblastoma characteristics on CT scan
Hyperdense
Syndromes associated with medulloblastoma
Gorlin syndrome, Turcot syndrome, ataxia telangiectasia, and Li-Fraumeni syndrome
Gorlin syndrome
Autosomal dominant condition characterized by basal cell carcinoma, skeletal abnormalities, and microcephaly
Turcot syndrome
Increased risk of colorectal cancers and medulloblastoma
Ataxia telangiectasia
Autosomal recessive disorder
Patients are at a higher risk for hematologic and breast cancer in addition to medulloblastoma
Li-Fraumeni syndrome
Autosomal dominant disorder
Patients are at a higher risk for cancers of the breast, bone, soft tissue, brain, blood and adrenal cortex
Common imaging characteristics of juvenile pilocytic astrocytoma (JPA)
Discrete cystic lesions with an enhancing mural nodule that tend to occur in the cerebellar hemisphere, optic nerve, hypothalamus, and brainstem
Associations of optic nerve JPAs and well-differentiated astrocytomas
NF type II
Characteristics of pleomorphic xanthoastrocytomas
Astrocytic neoplasms found in young adults with a long history of seizures
Usually superficial in the cerebral cortex and may consist of a mural nodule associated with a cyst
Characteristics of oligodendroglial tumors
Most present in young adult with onset of seizures
Calcifications are typical
Common location and presentation of ependymal tumors
Floor of the 4th ventricle and presents with increased ICP (nausea, vomiting, headache, gait ataxia, diplopia, vertigo)
Important workup for ependymymas
Neuro-axis imaging for drop mets
Treatment of intramedullary spinal cord ependymomas
Surgery alone
Common location for myxopapillary ependymomas
Conus or filum terminale of the spinal cord
Common locations for choroid plexus tumors
In children less than 2, they are usually located in the lateral ventricles
In adults, the usually occur in the 4th ventricle, foramen of Luschka or CPA
Characteristics of gangliocytoma
Primarily supratentorial with the most common location being the temporal lobe
Majority present in the first 2 decades of life
Symptoms of Parinaud’s syndrome
Pupillary mydriasis, paralysis of upgaze, and convergence retractorius
Characteristics of neuroblastoma
Arises in deep cerebral hemispheres of young children (
Imaging characteristics of spinal neurofibromas
Dumbbell appearance when they extend across the neuroforamena
Most common intradural, extramedullary tumors
Meningiomas, nerve sheath tumors, and filum terminale ependymomas
Dumbbell appearance of intradural, extramedullary tumors
Schwannomas (69%) and neurofibromas (12%)
DDx for benign, extradural tumors
Hemangioma, enchondroma/chondroma, osteochondroma, osteoma/osteoblastoma
DDx for malignant, extradural tumors
Osteosarcoma, chondrosarcoma, chordoma, Ewing sarcoma, Giant cell, plasmacytoma, multiple myeloma
DDx for intradural, extramedullary tumors
Meningioma, Schwannoma, neurofibroma, filum terminale ependymoma
DDx for intradural, intramedullary tumors
Astrocytoma, ependymoma, hemangioblastoma
Most common spinal cord tumor in the pediatric population
Astrocytomas
Intramedullary tumors that may have an associated syringomyelia
Ependymomas, followed by hemangioblastomas and cavernomas
Gene locus and gene affected in NF1.
17q11
NF1 gene
Gene locus and gene affected in NF2.
22q12
NF2 gene
Gene locus and gene affected in tuberous sclerosis.
9q34/16p13
TSC1 and TSC2 genes
Gene locus and gene affected in von Hippel-Lindau.
3p25
VHL gene
Gene locus and gene affected in Li-Fraumeni syndrome
17p13
TP53 gene
Gene locus and gene affected in Gorlin syndrome
9q22
PTCH gene
Gene locus and gene affected in Turcot syndrome
5q21
APC gene
Gene locus and gene affected in Cowden disease.
10q23
PTEN gene
Gene locus and gene affected in MEN1.
11q13
MEN1 gene
Gene locus and gene affected in rhabdoid tumor predisposition syndrome.
22q11
SMARCB1 gene.
CNS neoplasms seen in NF1.
Neurofibroma, malignant peripheral nerve sheath tumor, optic nerve glioma, meningioma.
CNS neoplasms seen in NF2.
Scwhannoma, meningioma, ependymoma.
CNS neoplasms seen in tuberous sclerosis.
Subependymal giant cell astrocytoma.
CNS neoplasms seen in von Hippel-Lindau.
Hemangioblastoma.
CNS neoplasms seen in Li-Fraumeni syndrome.
Glioma.
CNS neoplasms seen in Gorlin syndrome.
Medulloblastoma.
CNS neoplasms seen in Turcot syndrome.
Astrocytoma, glioblastoma, medulloblastoma.
CNS neoplasms seen in Cowden disease.
Dysplastic gangliocytoma of cerebellum.
CNS neoplasms seen in MEN1.
Pituitary adenoma.