Caner in Children FRCR CO2A Flashcards
(104 cards)
1
Q
Hardest part of consultation in Childhood malignancies
A
Late toxicity of treatment
2
Q
How should information about treatment should be passed
A
verbal information with written information
3
Q
which part of day is good for RT in children
A
morning, fasting is required for anesthesia
4
Q
staffs for RT involved for children
A
minimum number and same staffs, if machine breaks down, same staff should take pt to other machine
5
Q
Consent for Treatment in Children should include :
A
acute and later effects of treatment
6
Q
Late effects :
A
- sexual and reproductive health
- Neurocognitive deficits
- Hormonal deficiencies n need for HRT
- Effects on bone and soft tissue growth
- vision and hearing defects
- Second malignancies
7
Q
legal age of capacity in england, wales, ireland and scotland
A
18 in all except 16 in scotland
8
Q
who should give consent for children < 16 yrs of age and not yet competent?
A
any person or body with parental responsibility, even if the child is refusing treatment
9
Q
is a competent child legally entitled to withhold consent to treatment?
A
Yes
10
Q
How should RT room be designed for children?
A
Child friendly with distraction devices, music and an intercom
11
Q
Advantage and disadvantages of IMRT in children
A
Advantage: increased conformality
Disadvantage: low dose bath and high integral dose
12
Q
Difference between proton and X rays in terms of RBE and Dosimetry
A
equal RBE but due to bragg peak of proton, there is near zero dose beyond PTV, so it has dosimetric advantage
13
Q
Inclusion criteria for proton therapy in UK
A
- Curative Intent
- absence of metastases
- PS 0, 1
14
Q
Tumors for Proton therapy
A
- Pelvic sarcoma
- spinal and paraspinal adult type sarcoma
- rhabdomyosarcoma
- para meningeal and orbital locations
- Ewing sarcoma
- Ependymoma
- Optic pathway and other low grade gliomas
- craniopharyngiomas,
- Pineal parenchymal tumors
- Retinoblastoma
- Base of skull chordoma
- Chondrosarcoma
- Medulloblastoma
15
Q
Indications of brachytherapy in Children
A
- soft tissue sarcoma of pelvis and Head and Neck
- local recurrence in a previously treated radiation volume
16
Q
Commonest CAncers in Child
A
- wilms tumor
- Neuroblastoma
- Retinoblastoma
- Hepatoblastoma
- Infant Germ Cell Tumors (GCTs)
- ALL
- AML and other leukemia
- Hodgkin Lymphoma
9.. Non Hodgkin Lymphoma - Bone Sarcoma
- Adolescent Germ Cell Tumors
- Adult type sarcoma
- Carcinoma and Melanoma
- Rhabdomyosarcoma
- CNS malignances: Medulloblastoma and other PNETS, Ependymoma, Diffuse intrinsic pontine glioma, gliomas (low grade and high grade), Craniopharyngioma, Intracranial Germ Cell Tumors, Meningioma
17
Q
RT dose to cause drop in spermatogenesis
A
0.5 Gy
18
Q
Azoospermia RT Dose
A
2 to 3 Gy
19
Q
at what RT dose permanent sterility
A
Beyond 6 Gy
between 4 to 6 Gy, recovery may take 5 years or may never recover
20
Q
Testosterone = 0, at what RT dose
A
> 24 Gy
declines at 15 Gy
21
Q
ovarian failure at age 20
A
with dose of 16.5 Gy
22
Q
Risk of 2nd malignancy at 30 yrs
A
20.5 %
x 6 than the general population
23
Q
who are at increased risk of 2nd malignancy
A
extensive chemo, especially with alkylating agent and genetic predisposition to cancer
24
Q
Hypothalamic and pituitary failure at what RT dose
A
24 to 35 Gy
25
NICE recommendation for annual mammogram screening
women received > 30 Gy of supradiaphragmatic irradiation as a child
26
who are at highest risk of cognitive function deterioration with RT
children < 3 yrs
27
RT dose for SNHL
> 30 to 40 Gy
28
what other factors increases risk of SNHL with RT
high dose platinum compounds , aminoglycosides
29
chronic sinusitis at what RT Dose
> 30 Gy
30
RT dose for defect in teeth
20 Gy
31
Neuroblastoma, origin
from anywhere in the sympathetic nervous system
32
Neuroblastoma, sites % wise
Adrenal 40%
Abdominal 25%
Thoracic 15%
Cervical 5%
Pelvic symphathetic ganglia 5%
33
where does neuroblastoma metastasize to
LNs
Bone marrow
Bone
dura
orbits
liver and skin
less commonly to the lungs and CNS
34
investigations for neuroblastoma
Cross sectional imaging
bone scan
bone marrow
an iodine 123 MIBG scan
24 hour urinary catecholamines and
Tumor Biopsy
35
risk stratification of neuroblastoma
as per INPC (international neuroblastoma pathology classification)
36
Intermediate and high risk INPC patients treatment
Local RT is given to the primary site
37
CTV in neuroblastoma
prechemotherapy tumor volume and residual enlarged areas with 1 to 2 cm margin
38
RT dose for neuroblastoma
21 Gy/14 #
39
Wilm's tumor
what's difference in practise between america and Europe
America: COG : immediate nephrectomy with histology guided stratification of subsequent chemo
EUROPEAN SIOP Approach:
Biopsy, 4 wks of preop vincristine and dactinomycin for localized tumors and additional 2 weeks of chemo including doxorubicin for metastatic tumor
40
Stages of wilms tumor (EUROPE)
stage I: localized to kidney
Stage II: extend beyond the kidney but are completely removed
Stage III: incomplete removal, where nodal or peritoneal spread has occured or where tumor has ruptured
Stage IV: hematogenous spread
41
histological factors for risk factors
higher grade elements like blastemal elements and degree of. necrosis in nephrectomy specimen
42
low grade Stage I tumor, adjuvant Rx
observation
43
44
45
46
Stage II and III low risk
st I, II and III intermediate risk
Vincristine and Dactinomycin
46
RT dose for wilms tumor
14.4 Gy in 8 fractions with a boost of 10.8 Gy in 6 fractions to macroscopic residual disease
46
when is flank RT given
intermediate risk stage III tumors
47
High risk st II and III wilms tumor Rx
Chemo with vincristine, dactinomycin and doxorubicin, flank RT to dose of 25.2 Gy in 14 fractions,
sites of metastatic disease are also treated
48
Retinoblastoma association with which mutation
germline loss of one of RB1 gene allele
49
presentation of retinoblastoma
leukocoria (a white pupil rather than red eye on flash photography)
squint
nystagmus and
an inflamed eye
50
Retinoblastoma survivors are at increased risk of which cancer
sarcoma, about 51 % incidence
51
Rx options in Retinoblastoma
Chemotherapy into vitreous or opthalmic artery
Surgery: enucleation, laser photocoagulation, cryotherapy, iodine 125 or ruthenium 106 plaque
52
5 yr survival rate of retinoblastoma
95%
53
which is commonest liver tumor in children < 3 yrs old
Hepatoblastoma
54
Rx of hepatoblastoma
initial biopsy
preop chemo
complete surgical excision (may involve hepatectomy and liver transplant)
55
chemo for hepatoblastoma
Standard risk: cisplatin monotherapy
high risk: cisplatin and doxorubicin
Very high risk: dose dense weekly cisplatin and doxorubicin
56
Infant GErm CEll Tumors origin
mostly in midline usually sacrococcygeal, mediastinal or retroperitoneal
57
Infant Germ Cell Tumor Nature
most benign, elevated AFP, contain yolk sac elements
58
Rx for infant GCT
surgery and observation and with CArboplatin, etoposide and bleomycin at relapse
59
which is the common leukemia in children
ALL
60
cure rate in low risk and high risk ALL
98 % and 86%
61
who are low risk ALL pts
age 1 to 10 yrs at diagnosis and WBC max 50000
62
who are high risk ALL pts
all other than low risks
63
current Chemo regimen for ALL
Early Induction phase with dexamethasone
Consolidating phase with dexamethasone, vincristine, mercaptopurine, Systemic and intrathecal methotrexate and at times, asparginase
maintenance phase of vincristine, dexamethasone, mercaptopurine and methotrexate
64
Sanctuary sites for lymphoblasts
CNS and testes
65
when is RT used in ALL and dose?
at relapse, 24 Gy/ 12 #
66
Rx for relapsed pts responding poorly to chemotherapy
TBI f/b Allogenic Stem Cell Transplant
67
Dose for TBI
12 Gy in 6 #, 3 days
68
histological feature of rhabdomyosarcoma
presence of rhabdomyoblasts which are malignant cells containing striated muscle
69
Syndromes a/w Rhabdomyosarcoma
1. Li-Fraumeni Syndrome
2. Pleuropulmonary blastoma
3. NF type I
4. Beckwith wiedermann
5. Costello and Noonan Syndrome
70
spread of Rhabdomyosarcoma
common to LNs
Blood: Lung, liver and bone
71
Rx of Rhabdomyosarcoma
Complete excision
but if too mutilating, 2nd look surgery after chemotherapy for incomplete excision
72
Risk Stratification for Rhabdomyosarcoma
by the extent of initial surgery into Intergroup Rhabdomyosarcoma Studies Stages (IRS) 1, 2 or 3
73
what are IRS 1, 2, 3 and 4
1: complete microscopic excision R0
2. excision with microscopic residual
3. macroscopically incomplete excision
4. metastatic disease
74
poor prognostic factors in Rhabdomyosarcoma
1. alveolar morphology
2. age 10 or more
3. tumor size > 5 cm
75
favorable sites Rhabdomyosarcoma
1. orbit
2. Genitourinary
3. Non Bladder/prostate and
4. non parameningeal head and neck
all others: unfavorable
76
European Pediatric soft tissue study group 2005 Treatment
Low risk pts
only vincristine and dactinomycin and surgery if R0
77
European Pediatric soft tissue study group 2005 Treatment
Standard risk
Ifosfamide, Vincristine and Dactinomycin (IVA)
R0: observation
R1: RT with dose of 36 to 50.4 Gy in 1.8 Gy/#
78
European Pediatric soft tissue study group 2005 Treatment
high and very high risk pts
IVADo and RT (41.4 Gy to 50.4 Gy)
79
RT CTV for rhabdomyosarcoma
initial tumor extent and clinically involved nodes plus 1 to 2 cm margin
metastatic sites to be irradiated
80
which Chemo drug should be avoided during RT to trunk, abdomen or HnN region for rhabdomyosarocma
Dactinomycin
81
Medulloblastoma origin and common site
primitive neuroectodermal tumor (PNET), arise in posterior fossa
around the 4th ventricle
82
dd of medulloblastoma
ependymoma
83
Rx of medulloblastoma
Surgery f/b adjuvant treatment
84
investigations post surgery for medulloblastoma
MRI craniospinal axis and CSF cytology atleast 14 days post surgery
85
what if CSF is examined earlier after surgery?
False Positive
85
Adverse RFs for medulloblastoma
1. residual tumor > 1.5 cm2
2. n myc gene amplification
3. large cell anaplastic morphology
4. delay of RT for > 6 weeks post Sx
86
standard risk for recurrence medulloblastoma
RT dose
CSI 23.4 Gy with boost to 54 Gy with adjuvant chemotherapy
unable to receive adjuvant chemotherapy, typically doses of 30–36 Gy CSI, with involved field boost to the primary brain site to 54–55.8 Gy are used.
87
* High Risk for Recurrence Medulloblastoma RT
36 Gy CSI boost to primary brain site to 54–55.8 Gy with adjuvant systemic therapy
88
CSI fields
cranial: two half beam blocked lateral fields
spinal : two posterior fields
89
how is posterior divergence of spinal field matched with cranial fields
by giving collimator rotation
90
how is inferior divergence of cranial fields avoided for CSI
by giving couch rotation
91
Rx of ependymoma
Surgery fb post op RT
92
RT dose in ependymoma
54 Gy, better prognosis seen with 59.4 Gy
93
CTV in ependymoma
post op tumor bed with 1 cm margin
94
Diffuse intrinsic pontine glioma, Diagnosis, Treatment, Survival
MRI, biopsy not required
RT is the only Rx
median survival 10 months only and 2 yr survival rate is less than 10 %
95
Low Grade Glioma Rx in Children
Surgery only
96
when is RT indicated in low grade glioma in children
recurrent inoperable tumor
97
HGG RT in children
Dose 54 Gy in those over age 3 yrs,
CTV encompassing tumor bed with 25 mm margin
98
did higher dose (72 Gy) improve survival in High Grade Glioma
N0, fulton et al 1992
99
Role of TMZ in HGG in children
standard (stupp et al)
100
common sites for intracranial germ cell tumors:
1. Pineal Gland
2. suprasellar region
3. basal ganglia
4. hypothalamus
101
Diagnosis of intracranial germ cell tumors:`
imaging and biopsy
but elevated blood or CSF AFP or Beta HCG is diagnostic and may prevent need for biopsy
