Central Nervous System Tumors in Children Flashcards
The etiology of pediatric CNS tumors remains largely unknown. Only 2% to
5% can be ascribed to a known genetic predisposition.
Enumerate the conditions with genetic predisposition to develop CNS tumors in children.
NF1
NF2
nevoid basal cell (Gorlin’s) syndrome
familial adenomatous polyposis
Li Fraumeni syndrome.
In pediatric CSI, what should be the lower limit of the spine CTV?
The lower limit of the CTV for the spine field should be determined
using MRI.
The lower border of the spinal CTV should include the lower border
of the thecal sac, which can be as high as L5 or as low as S3
Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:
Field matching over cervical spine/risk of over- or underdosage
Angle brain fields
use half-beam block for brain fields
use couch rotation or match line wedge
Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:
Inhomogeneity along spinal axis
Use compensator, MLC
Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:
Irradiation of normal tissues - mandible teeth
Neck extension
Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:
Irradiation of normal tissues - thyroid
Care with level of junction
Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:
Irradiation of normal tissues - heart
Use lower junction
Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:
Irradiation of normal tissues - GI tract
Care with width of spine field
Use protons, IMRT, electrons
Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:
Irradiation of normal tissues - gonads
Care with lower limit and width of spine field
Use protons, IMRT, electrons
Central Nervous System Tumors in Children
LGA’s account to a third to half of all pediatric CNS tumors.
TRUE or FALSE?
True
Central Nervous System Tumors in Children
Most pediatric CNS tumors are supratentorial.
TRUE or FALSE?
False
Infratentorial
Central Nervous System Tumors in Children
What is the most common type of astrocytoma in children?
Pilocytic astroctyoma
Central Nervous System Tumors in Children
Describe the histology/pathologic picture of pilocytic astrocytoma.
well circumscribed with an associated cystic component.
biphasic pattern with a varying proportion of compacted bipolar cells with Rosenthal fibers and loose-textured multipolar cells with microcysts and granular bodies.
Rare mitoses, occasional hyperchromatic nuclei, microvascular proliferation, and even infiltration of meninges.
KIAA1549-BRAF fusions have been identified in 75% of patients
with PA using an RT-PCR assay from FFPE tissue.
Central Nervous System Tumors in Children
What variant of pilocytic astrocytoma, first described in infants and children with
chiasmatic/hypothalamic tumors, appears to be associated with more aggressive
behavior that may include leptomeningeal seeding?
pilomyxoid astrocytoma
Central Nervous System Tumors in Children
Where are the two most common locations of pilocytic astrocytoma, wherein they account for almost all LGAs in those areas?
cerebellum
anterior optic pathway
Central Nervous System Tumors in Children
Where are the two most common locations of diffuse astrocytoma?
cerebral hemispheres
pons
Central Nervous System Tumors in Children
Describe the histology/pathologic picture of diffuse astrocytoma.
well-differentiated fibrillary or gemistocytic neoplastic astrocytes on a background of loosely structured, often microcystic, tumor matrix.
moderately increased cellularity.
nuclear atypia is a diagnostic criterion
(mitotic activity, necrosis, and proliferation are absent)
low Ki-67 and MIB-1 labeling indices.
Central Nervous System Tumors in Children
What is the syndrome commonly manifested by young children with chiasmatic/hypothalamic tumors that present with the ff:
emaciation with loss of subcutaneous fat despite normal or increased appetite, alert appearance, increased vigor and euphoria, pallor without anemia, and nystagmoid movements of the eyes
diencephalic syndrome
Central Nervous System Tumors in Children
Neuroimaging findings in pilocytic astrocytoma
well circumscribed with an associated cystic component.
There is usually little edema or mass effect.
The solid component enhances brightly and uniformly with contrast material.
Central Nervous System Tumors in Children
Diffuse or fibrillary astrocytomas are usually not well seen on
nonenhanced CT or MRI and usually show little enhancement with contrast
material.
What sequences usually best demonstrate the extent of disease?
T2-weighted
or
FLAIR
Central Nervous System Tumors in Children
For pilocytic astrocytomas and diffuse or fibrillary LGAs, postoperative adjuvant therapy is not indicated after complete resection.
TRUE or FALSE?
TRUE
Central Nervous System Tumors in Children
For pilocytic astrocytomas and diffuse or fibrillary LGAs, postoperative adjuvant therapy observation can be an option.
How frequent is surveillance MRI performed?
every 6 months for 3 years.
Central Nervous System Tumors in Children
For pilocytic astrocytomas and diffuse or fibrillary LGAs, postoperative adjuvant therapy observation can be an option.
What are the options at the time of progression?
Second surgical resection.
if not resectable RT (or chemotherapy: for children ≤10 and children of all ages with NF1)
Central Nervous System Tumors in Children
What are the indications for RT in LGA?
following incomplete resection in situations when tumor progression would compromise neurologic function (e.g., “threat to vision”)
patients with progressive and/or symptomatic disease that is unresectable.