CNS neoplasms Flashcards
Features of grade I CNS neoplasms
Low proliferative potential and cure with surgical resection
Features of grade II CNS neoplasms
Diffuse infiltrative
Low mitotic activity
Probability of recurrence
Some tend to progress to high grade
Features of grade III CNS neoplasms
Anaplastic tumor
Malignant histologic features
High recurrence rate
Need chemotherapy and radiotherapy
Features of grade IV CNS neoplasms
Obviously malignant
Necrosis-prone and fast capability of recurrence
Diffuse spreading in CNS
Typical age range associated with glioblastoma, astrocytoma, and oligodendroglioma
Adults
Typical age range associated with pilocytic astrocytoma, medulloblastoma, and pineoloma
Children
Types of CNS cancers that are found in both children and adults
Craniopharyngioma
Ependymoma
Overexpression of these protooncogenes is associated with CNS tumor
EGFR
PDFR
Loss of these tumor suppressor genes is associated with CNS malignancy
P53
CDKN2A/B
PTEN
Typical mutation in lower-grade astrocytomas
IDH mutation with intact 1p/19q
Typical mutation oligodendrogliomas
IDH mutation and codeletion of 1p/19q
IDH type associated with a better prognosis
IDH mutant type –> IDH positive
IDH type associated with a poor prognosis
IDH wild type –> IDH negative
WHO grade and type of glioblastoma multiforme
Grade IV astrocytoma
Most common malignant primary brain tumor
Glioblastoma multiforme
Typical IDH type in glioblastoma multiforme
Negative
Histology shows atypia, mitosis, and microvascular proliferation with areas of necrosis and hemorrhage surrounded by pseudopalisade arrangement of tumor cells that are EGFR and GFAP positive
Glioblastoma multiforme
WHO grade of pilocytic astrocytoma
Grade I –> relatively benign
Most common benign tumor of childhood
Pilocytic astrocytoma
T1 MRI shows well demarcated cystic lesion with an enhancing mural nodule in the cerebellum, or perhaps the brainstem
Pilocytic astrocytoma
Histology shows bipolar cells with hairlike processes, called Rosenthal fibers
Pilocytic astrocytoma
Common location of oligodendroglioma
Cerebral hemisphere, usually involving the frontal lobe
Grossly a well-circumscribed gray mass with calcification diffusely infiltrating the cortex and white matter
Oligodendroglioma
Histology shows sheet of cells with round regular nuclei and perinuclear halo, dense network of branching capillaries, and perineuronal satellites
Oligodendroglioma
Tumor that is derived from ependymal cells lining the ventricular surface, frequently arising from wall of fourth ventricle, within the posterior fossa
Ependymoma
Histology shows perivascular pseudorosettes and ependymal rosettes, usually GFAP positive
Ependymoma
Electron microscopy findings in ependymoma
Cilia and microvilli
Junctional complexes
Most common malignant brain tumor of childhood
Medulloblastoma
Poorly differentiated tumor of the cerebellum, common in childhood
Medulloblastoma
MRI shows densely enhancing mass in the posterior fossa, within the cerebellum
Medulloblastoma
Drop metastases
Dissemination through CSF to spinal cord
Histology shows small blue cells, Homer-Wright/neuroblastic rosettes with central lumen of neutrophils, sheets of primitive cells and high mitotic figures. Maybe synaptophysin positive.
Medulloblastoma
Characteristics of small blue cells on histology
Densely packed cells with hyperchromatic nuclei and scan cytoplasm
WHO grade of meningioma
Grade I, slow-growing and benign
CNS tumor associated with ionizing radiation and NF2, with a female predominance
Meningioma
Grossly a well-demarcated, firm, rubbery dural-based tumor
Meningioma
Histology shows whorls of arachnoid cap cells, laminated calcified psammoma bodies. It is EMA, PR, and somatostain positive. It is GFAP and synaptophysin negative.
Meningioma
Two types of craniopharyngioma and there associated age
Adamantinomatous –> children
Papillary –> adults
Most common non-neuroepithelial intracranial tumor in children
Adamantinomatous craniopharyngioma
Mutation associated with adamantinomatous craniopharyngioma
Beta-catenin gene
Mutation associated with papillary craniopharyngioma
BRAF/V600E mutation
CNS tumor derived from remnant of Rathke’s pouch
Craniopharyngioma
Tumor signaling pathway found in adamantinomatous craniopharyngioma
WNT pathway
Tumor signaling pathway found in papillary craniopharyngioma
MAPK pathway
Imaging shows displaced midbrain with hydrocephalus and suprasellar cyst mass
Cranipharyngioma
Grossly a suprasellar, partially cystic, focally calcified mass filled with brownish-yellow viscous fluid rich in protein and cholesterol crystals
Craniopharyngioma
Histology shows epithelial islands containing amorphous pink areas of keratinization, surrounded by a layer of palisading cells
Craniopharyngioma
Imaging shows one or more discrete lesions that are usually ring-enhancing. Grossly a well-circumscribed mass(es) along the gray-white junction.
Brain metastasis
Brain metastasis type that is common in malignancies that spread to bone
Epidural metastasis
Brain metastasis type that is common in hematologic malignancies and solid tumors
Leptomeningeal metastasis
Tumors associated with neurofibromatosis 1
Neurofibromas
Optic gliomas
Mutation in neurofibromatosis 1
NF1 on chromosome 17
Tumors associated with neurofibromatosis 2
Bilateral schwannomas
Meningioma
Mutation in neurofibromatosis 2
NF2 on chromosome 22 encoding merlin protein
Tumors associated with tuberous sclerosis
Cortical tubers
Subependymal nodules
Mutations associated with tuberous sclerosis
TCS1 on chromosome 9 –> hamartin
TCS2 on chromosome 16 –> tuberin
Mutation associated with Von Hippel-Lindau syndrome
VHL on chromosome 3
Tumors associated with Von Hippel-Lindau syndrome
Hemangioblastomas - cerebellar and retinal
Pheochromocytomas
Multiple bilateral kidney cysts and clear cell renal carcinoma
WHO grade of schwannoma
Grade 1 –> benign
Benign nerve sheath tumor arising from Schwann cells
Schwannoma
Merlin protein
Cytoskeletal protein that mediates contact inhibition
Common location of vestibular schwannomas
Internal auditory meatus at the cerebellopontine angle
Well-circumscribed, variegated massed with a tan appearance and macro-cysts. Appears as densely enhancing lesion on MRI.
Schwannoma
Hypercellular and hypocellular regions with Verocay bodies, characterized by distinct palisades with a fibrillary core. With well-formed capsules and hyalinized vessels. S100 strong and diffusely positive, and highly positive for pericellular collagen IV.
Schwannoma
Hamartomatous neurocutaneous disease
Tuberous sclerosis
Skin findings in tuberous sclerosis
Adenoma sebaceum
Hypomelanotic macule
Shagreen patches
Cortical hamartomas, subependymal nodules, and subependymal giant cell astrocytomas
Tuberous sclerosis
Non-CNS tumors associated with tuberous sclerosis
Angiomyolipomas in kidneys
Rhabdomyomas in heart
Tumor syndrome associated with impaired down-regulation of mTOR signaling pathways
Tuberous sclerosis
Tumor syndrome associated with increased HIF-1
Von Hippel-Lindau disease
Mutation in Sturge-Weber syndrome
GNAQ gene somatic mosaic mutation
Syndrome associated with ecephalotrigeminal angiomatosis
Sturge-Weber syndrome
Conditions associated with Sturge-Weber syndrome
Mental retardation
Epileptic spasm as infancy
Early-onset glaucoma
CT shows tram-track sign of cortical and subcortical calcification
Sturge-Weber syndrome
T1 MRI, post-gadolinium, shows prominent leptomeningeal enhancement of the affected area (pial angiomatosis)
Sturge-Weber syndrome
Inheritance of tuberous sclerosis
Autosomal dominant
