pediatrics Flashcards
1
Q
most common paediatric cancer
A
ALL (acute lymphocytic leukaemia)
2
Q
lymphoma is the ___most common paediatric cancer
A
3rd
3
Q
age for lymphomas in children
A
common in 4-14 y.o.
4
Q
what causes HD in kids
A
HD in kids is common in EBV in developing countries
5
Q
what HD is common in what age group
A
nodular sclerosing is most common in adults and adolescents less common in children
mixed cellularity is more common in children
6
Q
most children with lymphoma present with what kind of LN
A
cervical lymphadenopathy
7
Q
is mediastinal involvement more common in children or adolescents with lymphoma
A
mediastinal involvement affects 75% of adolescents and is less common 33% in children 1-10y,o,
8
Q
prognostic factors for lymphoma in chilren
A
stage is most important to determine treatment
b symptoms have a higher risk of relapse
T4 has worse prognosis
WBC count above 11500 hemoglobin less than 11 g/dl
lymphocyte depleted has a worse prognosis than other subtypes
children <10 have better prognosis
9
Q
howccommon are the presentation of b symptoms in paediatric lymphoma pts
A
1/3 of patients present with b symptoms
fever >38c
night sweats
weight loss >10% of its body weight in 6 months
10
Q
primary treatment for paediatric lymphoma patients
A
chemo
11
Q
most common paediatric brain tumours
A
low grade gliomas medulloblastomas gliomas ependymomas craniopharyngiomas pineal region tumurs embryonal rumours choroid plexus tumours
12
Q
treatment for low grade gliomas for patients <10 and >10
A
<10 SX+CX then XRT RESERVED FOR PROGRESSION
>10 XRT up front
13
Q
planning for paediatric low grade gliomas CTV
A
CTV expansion of 5 mm for localized tumours and dose of 54/30
14
Q
high grade glioma treatment
A
start with surgery adjuvant XRT5940/33
15
Q
2nd most common types of paediatric tumours
A
brain tumours
16
Q
XRT alone in lymphoma of paeds
A
Radiation alone is almost never done
Possible exception is the fully grown child with localized nodular lymphocyte predominant HD
17
Q
dose for XRT for paeds
A
low dose 15-25.5Gy
18
Q
is hodgkins or non hodgkins more common in paeds
A
Non hodgkins is more common
19
Q
most common nodal areas involved in NHD
A
Abdomen is most common followed by mediastinum
20
Q
use of XRT in paediatric brain tumours
A
reserved for unresectable or recurrent lesions 50-54Gy
21
Q
CTV,PTV for low grade astrocytomas pediatric
A
CTV-1.5cm around GTV
PTV-.5cm around CTV
22
Q
Treatment optic glioma
A
chemo for younger patients, 50-54Gy with IMRT, proton or 3DCRT
23
Q
What can cause optic glioma in children
A
neurofibromatosis
24
Q
where does ependymoma arise from
A
from the ependyma cells that line the ventricles
25
presenting symptoms of ependymoma paper like what other CNS tumour?
medulloblastoma
26
treatment of ependymoma in children
treated by a gross total resection XRT after surgery is generally given if CSF seeding is noted then CSI XRT is given 1cm CTV is treated to 5400-5940 cGy chemo is given neo or adjuvantly if the tumour is unresectable
27
medullablastoma is a _____fossa malignancy
posterior
28
medulloblastoma constitutes ___% of all childhood brain tumours
20
29
medulloblastoma arises from what type of cells
cerebellar stem cells
30
histologic appearance of medulloblastoma
small round blue cells
31
invasion of medulloblastoma
arises midline of cerebellum, invades 4th ventricles and brainstem and high propensity to invade CSF
32
Distant mets in medulloblastoma
can occur most likely to the bone
33
treatment for medulloblastoma
posterior occipital craniotomy is used followed by adjuvant XRT CSI 2340-5400 followed by chemotherapy
34
where do germ cell tumours arise from
develop from embryologic nests of tissue in midline brain usually in the suprasellar or pineal region
35
germinoma is radio --------.
sensitive
36
what is the most common type of paediatric germ cell tumours
germinoma
37
treatment germ cell tumour
low dose csi followed by tumour boost to 50 Gyor cisplatinum based chemo followed by XRT boost
non germinomas are treated with all 3: cispltinum chemo, art and XRT boost
38
presentation of brainstem glioma
present with cranial nerve deficits that develop over weeks to months that affect vision, facial function and swallowing
39
surgery for brainstem glioma
usually inresectable as brainstem gliomas usually involve the pons
40
treatment of brainstem glioma
XRT is mainstay of treatment as brainstem gliomas are often unresectable there should be a 1-2cm margin dose is 54Gy
41
where is the retinoblastoma gene located in children?
chromosome 13 a tumour supressor gene
42
how is Rb diagnosed
discovered as a result of an abnormal light reflex, reflects white rather than red) can be discovered during a dr examination or from the flash of a camera
43
retinoblastoma staging
```
st dudes hospital staging
intraocular disease
1a. retinal tumour single or multiple
1b.extension to lamina criborsa
Optical disease
2a.optical tumour
2b.optic nerve invasion
intracranial mets
3a.+CSF fluid
3b.CNS mass lesion
```
44
treatment of retinoblastoma
small focal tumours of the optic disc and the macula is treated with cryosurgery, photocoagulation, laser hyperthermia, local treatments are more commonly done with CX,
EBRT is used for inoperable unilateral disease or bilateral disease dose is 40-50Gy
45
side effects of XRT in Rb
altered growth of the bony orbit, - vision, cataract formation, dry eye, and high risk of secondary cancer
46
neuroblastoma is derived from what cells
cells of the neural crest
47
what is different about neuroblastoma
it can spontaneously regress
48
what oncogene is associated with neuroblastoma
n-myc
49
where does neuroblastoma commonly occur in children
in the adrenal glands
50
S&S neuroblastoma
include flushing and diahrrea abode mass, bowel disturbance,
51
how common is mets in neuroblastoma in children
60% at presentation
52
s&s of metastatic neuroblastoma cancer
fatigue, anemia, bleeding from bone marrow invasion, fever weight loss, bony mets, blue skin lesions, abode distension from liver involvement
53
most important prognostic indicator in neuroblastoma
tumour stage is most important
54
4s neuroblastoma category
occurs in infants less than 1 year many of these patients spontaneously regress
55
staging system for neuroblastoma
stage1- confined to area of origin LN -, complete excision with or without microscopic disease
stage2a- unilateral tumour without complete excision ln-
Stage2b- unilateral tumour with or without completed excision,with +ipsilateral LN -contralateral LN
stahe3-tumour across midline with or without LN involvement , unilateral tumour with contralateral LN involvement or midline tumour with bilateral regional lN involvement
stage4-dissemination of tumour to distant LN, bone, bone marrow, liver or other organs
stage 4s- localized primary tumour as defined stage1,2 with dissemination to liver or skin
56
what age group does neuroblastoma spontaneously regress in
in new borns
57
treatment of neuroblastoma
localized disease without LN are cured by SX alone, for incomplete resections adjuvant XRT 500cGY (infants) 20Gy for children is added + multi agent cx (platinum, etopisode, cyclophosphamide, vincristine, doxorubicin
58
side effects of art IN NEUROBLASTOMA
more common in children getting >20Gy than infants getting 500cGy, these side effects include: decreased or asymmetric growth of bone and soft tissues, lung fibrosis can occur if the lungs are in field, kidney and liver function can be impaired, `
59
what type of cells does films tumour arise from
tumour of the kidney that arises from embryonal cells
60
age for films tumours
median age is 3-4 y.o.
61
what syndromes can cause films tumour
occurs more frequently in patients with WAGR syndrome (Wilms aniridia genitourinary malformations and mental retardation_) also common in patients with beck with Wireman syndrome and in patients with hemihypertrophy (one side of the body is larger than the other) and aniridia (absence o of the iris)
62
Wilms tumours with unfavourable prognosis nd why?
anapaestic wilms tumours. clear cell sarcoma of the kidney Associated with bone mets, rhaboid tumour of the kidney --associated with spread to the CNS
63
how do films tumours present
painless abdominal mass the enlarging mass can sometimes cause organs to rupture or bleed
64
diagnostic methods for wilms tumour
CT scan of the chest and abdomen, the contralateral kidney should be examined as the contralateral kidney is involved in 4-8% of cases bone scant and brain cans can be done as rhaboid and clear ell tumours have a high tendency for mets
65
Wilms staging system
national Wilms staging system
1. limited to the kidney but completely excised
2.beyond kidney but completely excised.
local tumour spillage or vessel invasion permitted but completely excised
3.residual nonhematogenous tumour confined to abdo
involved Ln, diffuse tumour spillage or grossly unresected tumour
IV. hematogenous mets usually liver blood bone or brain
V.bilateral kidney involvement at time of diagnosis
66
treatment for Wilms
in europe neoadjuvant chemo or rads is given as there is a high propensity for wilms to have tumour spillage during surgery, surgery is performed ( nephrectomy)and then adjuvant XRT is given to the tumour bed, if there is tumour spillage then the whole abode is treated with XRT pdoses are 1080-2000cgy. XRT is not required for stage1,2 that are completely excised and received chemo 1080 is used for microscopic disease, LN mets, bilateral tumours, pulmonary mets( only get 1500) , gross residual disease is boosted to 20Gy
67
XRT field size for wilms
for unilateral disease: width of 1 vertebral body +1cm contra laterally the growth plates of the long bones are shielded
sup- and inf = 1cm from the preoperative renal tumour
68
chemo agents used for wilms
acitomycin-d + vincristine +doxorubicin for advanced stages
69
side effects of treatment for wilms
for thoracic treatment of a prepubertal female breast development may be impaired, high doses of acitomycin-d can cause liver damage, because the child only has 1 kidney it is important that we are cautious if they get UTI which can lead to stones, infections or diseases from damaging the remaining kindye
70
most common STS in kids
rhabdomyosarcoma
71
what tis the tissue of origin in RMS
striated muscle
72
age for STSD
MOST common in kids <10
73
RMS is associated with translocations on what chromosome
13
74
where is RMS most common
h&n 34%
abdo 25%
GU 23%
Extremities and trunk 17%
75
where is embryonal subtype most commonly occur
orbit and GU
76
STAGING OF rms
```
intergroup RMS staging system
tumour
T1: confined to origin site
T2:extension to surrounding tissues
a.<5cm
b.>5cm
histology
g1: favorable: embryonal, undifferentiated, mixed
G2:unfavourable alveoler
Nodes: N0: not clinically involved
N1: clinically involved by tumour
Mets
M0: no distant mets
M1:mets at time of diagnosis
clinical system:
1:localized completely resected
2:grossly resected with microscopic residue or involved Ln
3:gross residual tumour
4:distant mets
```
77
diagnosis of RMS
First an incisional biopsy is done to confirm histology ( alveolar, mixed, undifferentiated, embryonal )
physical exam, CT , US, MRI o BM biopsy and chest CT can determine mets
78
what area of the body is mire prone and less prone to METS with RMS
not common with tumours of the orbit
| more common with tumours of the extremities and trunk
79
treatment of RMS
must determine f the patient is eligible for surgery, if so: removal of mass with margins is the first treatment, for tumours of the GU we want to preserve organ function therefore it is advisable to give cx +xrt rather than surgery for tumours that are unresectable or have +margins after sx cx+xrt is used with a 1cm margin around GTV
80
dose RMS
36Gy in 180cGy fx are used for microscopic disease, 41.4gy for ln + disease and 50.4Gy for gross disease
81
CHEMO AGENTS RMS
used adjuvantly after sx and is treated with vincristine, actinomycin D and cytoxan
82
late effects RMS treatment
bone and soft tissue growth is affected in the XRT of the extremities, in the H&N dryness of the eyed or retinal damage can occur , fertility is affected after chemo for the pelvic region, secondary tumours can also occur most commonly (HD,ALL, STS)
83
WHEN IS XRT used in ALL
When there is CNS or testicular involvement where there is less chemo penetration TBI is also used to prepare patient for BM transplant
84
dose for petiatric all
1200-1800cGy
85
typical treatment for ALL
Chemotherapy for 2 years is typical treatment
86
treatment for langerhans cell histocytosis
abnormal proliferation of immune systems langerhans cells
focal bony lesions can be treated with surgical cutter age if fracture is a concern or if tumour is unresectable low dose XRT is given 400cgy-1200cGy steroids, vinblastine and cyclophosphamide can also be used
87
HD treatment
chemo is initial treatment 15-25Gy is given following surgery
88
XRT field for unilateral neck HD
Unilateral neck + ipsilateral supraclavicular; the SUP border should extend form the midpoint of the chin through the mid-tragus; this should provide a minimum of 2 cm margin at the tip of the mastoid; the INF border must be 1.5 cm below the clavicle; the lateral field border should be determined by the most lateral extent of disease on the CT study of the neck
89
XRT field of the suoraclav and axilla for HD
Supraclavicular + mid/low neck + infraclavicular
| Axilla +/- infraclavicular.supraclavicular
90
XRT field the mediastinum / hill HD
Mediastinum + hila+ infraclavicular/supraclavicular; the SUP border should encompass the initial sup extent of disease + 2 cm; INF border should be placed 2 cm or at least one vert body below the lowest initial extent of disease; LAT borders will be treated based on the width of the disease and the mediastinum after chemo not the width of the original mass; 1.5 cm margin should be given on any residual mass and the normal mediastinal contour; lateral margins should cover the bilateral hila
91
XRT FIELD of the paraAORTICS IF in HD
Paraaortics +/- spleen; SUP border placed at insertion of the diaphragm; INF border at the level of the aortic bifurcation; LAT border should cover the initial extent of disease with 1.5 cm margin
92
XRT FIELDof the iliac HD
Iliacs + inguinal/femoral; if adequate superficial and deep coverage of these nodes can be obtained using only an anterior field with photons or an electron beam of appropriate, this is encouraged; SUP border 2-3 cm above and parallel to the inguinal fold; INF border should be parallel to the upper border; MED border should be the medial border of the obturator canal; LAT should be the lat border of the acetabuluM
93
PROGNOSTIC INDICATORS OF NHD
Stage, serum lactate dehydrogenase and soluble interleukin-2-receptor levels
94
role of XRT in NHD
Emergency treatment of mediastinal disease or spinal cord compression; treatment for pts who fail to obtain a complete remission after induction chemo; palliation of pain or mass effect; consolidation before bone marrow transplantation in patients with recurrent disease; over CNS lymphoma at diagnosis or relapse; leukemic transformation at diagnosis
30 Gy for small cell lymphocyte/blast to 45 Gy for large cell histiocytic
Palliation, total doses as low as 10 Gy
