Peds

Question 1

S&S of ALL

Answer 1

Anemia, infx, hemorrhage, Fever, Malaise

Question 2

S&S of Ewings Sarcoma

Answer 2

Pain, swelling, mass

Question 3

MC site Ewings Sarcoma

Answer 3

Femur

Question 4

S&S of Retinoblastoma

Answer 4

Leukochoria “cat eye”

Question 5

S&S of Neuroblastoma

Answer 5

Pain! lethargic, ill appearing child <2yrs w/ abdo mass
blue skin lesions
70% present with metastatic disease–>orbital, bone, liver

Question 6

MC site neuroblastoma

Answer 6

Adrenal Medulla

Question 7

S&S Wilms Tumour

Answer 7

Painless abdo swelling–>smooth, firm, one sided

Question 8

S&S Rhabdomyosarcoma

Answer 8

Asymptomatic mass
Favourable (Embryonal)–>Orbit
Unfavourable (Alveolar)–>Bladder

Question 9

S&S Glioma Astrocytoma

Answer 9

Headaches & decrease in coordination

Question 10

S&S Medullablastoma

Answer 10

Morning headaches & vomiting
45% spread beyond posterior fossa
invasion of 4th ventricle stopping flow of CSF

Question 11

Epi/Eti ALL

Answer 11

MC childhood cancer

2-10 yrs boys

Question 12

Epi/Eti Ewings sarcoma

Answer 12

MC bone tumour in children

10-20yrs, males

Question 13

Epi/Eti Retinoblastoma

Answer 13

MC intraocular tumour in children
6mo-4yrs
inheritable, sporadic

Question 14

Epi/Eti Neuroblastoma

Answer 14

2nd most common solid tumour

<2yrs, males

Question 15

Epi/Eti Wilm’s

Answer 15

3-4yrs

10-15% are associated with WAGR

Question 16

Epi/Eti Rhabdo

Answer 16

Before 10yrs
Males
Genetically inherited disorders

Question 17

Epi/Eti Glioblastomas

Answer 17

MC solid tumours in children

<3yrs

Question 18

Epi/Eti Medullabastoma

Answer 18

2-12yrs; males

INFRATENTORIAL

Question 19

Prognostic Indicators for ALL, Ewings, Retino, Neuroblastoma, Wilm’s,
Rhabdo, Glioma, Medulla

Answer 19

ALL & Rhabdo= <1yr or >10yr
Ewings, wilms & Medulla=extent of disease
Neuro & Glioma= Age & stage

Question 20

Routes of spread ALL

Answer 20

immature lymphoblast replace bone marrow

sanctuary sites: CNS, testis, optic/retina nerve

Question 21

Routes of spread Ewings Sarcoma

Answer 21

LN involvement=RARE

Lungs

Question 22

Routes of spread Retinoblastoma

Answer 22

Brain through the optic nerve invasion

Question 23

Routes of spread Neuroblastoma

Answer 23

LN involvement is common.

70% of patients have metastatic disease

Question 24

Routes of spread Wilm’s Tumour

Answer 24

Lungs and liver

Question 25

Routes of spread Rhabdo

Answer 25

Lung, bone marrow, bone

Question 26

Routes of spread Glioma Astrocytoma

Answer 26

No lymphatic spread

Question 27

Routes of spread Medullablastoma

Answer 27

Extends locally; grow and fill 4th ventricle or involves brain stem. can met to bone high risk of CSF seeding

Question 28

Pathology ALL (2 ways to classify)

Answer 28

1. morphologic 2. Immunologic-->main system a) pre-b cells 80-85% b) T Cell 12-15%

Question 29

Pathology Ewings

Answer 29

Small blue round cells; diffuse pattern if spindle cells are present it becomes osteosarcoma

Question 30

Pathology Retinoblastoma

Answer 30

small blue cell tumour originating from neuroectoderm tumours usually grow out of their blood supple and cause necrosis and hemorrhage

Question 31

Pathology Neuroblastoma (3 types of tumours & some staging)

Answer 31

1) Glaglioneuroma (benign) 2) Ganglioneuroblastoma 3) Neuroblastoma (small blue cell-undifferentiated) Stage IVS- children <1yr; stage I or II; often disappears and becomes ganglioneuroma

Question 32

Pathology Rhabdo

Answer 32

Arises from mesenchymal cells--> immature cells that develop to form muscle embryonal (60%)-->good prognosis Botryoid (6%)--> good prognosis Alveolar (21%)--> poor prognosis Pleomorphic (1%) Rare

Question 33

Pathology Glioma Astrocytoma

Answer 33

MC-Gliomas main types: 1) astrocytoma-->GBM 2) Ependymoma 3) Oligodendrocytes 4) Mixed

Question 34

Pathology Wilm's

Answer 34

Contains renal tubular | Favorable and unfavorable

Question 35

Pathology Medullablastoma

Answer 35

Arises from neuroectodermal cells and arises in posterior fossa. small blue round cells

Question 36

Disease Management ALL

Answer 36

Chemo-main tx Induction-->given immediately Consolidation-->After remission Maintenance-->Daily chemo orally Length: 3yrs for boys (disease harbored in testis) 2yrs for girls and adults

Question 37

Disease Management Retinoblastoma

Answer 37

Surgery--> enucleation | Chemo

Question 38

Disease Management Ewings

Answer 38

Small tumours in bones such as clavicle or fibula may be cured with RT & Sx lg lesions will get sx if not responding to chemo limb sparing-->bone graft or amputation

Question 39

Disease Management Neuroblastoma

Answer 39

Low risk-->resectable; good prognosis; post-op use of adj chemo or RT intermediate risk--> intensive tx High risk--> BMT -->combine induction chemo, sx, autologous (self) stem cell & RT to primary

Question 40

Disease Management Wilms

Answer 40

Sx then chemo RT post-op to involved flank, used for: - All stage I unfavourable - Stage II unfavourable - Stage III fav and unfav RT NOT used for stage I&II favorable Whole abdo is used when: - peritoneal seeding - rupture

Question 41

Disease Management Rhabdo

Answer 41

sx primary | chemo post-op (VAC)

Question 42

Disease Management Glioastrocytoma

Answer 42

Low & high grade-->sx Chemo low-->can delay or avoid RT Chemo high-->Dex, chemoRT post-op= standard

Question 43

Disease Management medullablastoma

Answer 43

Sx: - initially for diagnosis & debulking - posterior occipital craniotomy - sx not alone due to seeding - placement of shunt Chemo: -if <3yrs chemo used to delay RT to let brain mature RT: - used post-op - CSI typically done in young children who fail to respond to chemo or RT to post fossa only - Posterior fossa boost--HIGH risk of recurrence

Question 44

Dose ALL

Answer 44

TBI- 1200-200 BID 3days CSI-helmet: 2400/150 Spine: 1500/150 Cranial/helmet: 1200/180-200

Question 45

Dose Ewings

Answer 45

Typically 45Gy | Total dose 60Gy

Question 46

Dose Glioma Astrocytoma

Answer 46

Low grade: 50-54Gy | High grade: 60Gy

Question 47

Dose Medullablastoma

Answer 47

CSI: 3600/150-180 followed by a boost to posterior fossa 54-55

Question 48

1. TD 5/5 of bone marrow 2. Tolerance of cornea, lens, and optic nerve 3. OARs for wilms tumour

Answer 48

1. 250 2. 50; 500; 60 3. remaining kidney, liver, growth plates, bowel

Question 49

ALL tx borders

Answer 49

Cranial field: inf: include cribriform plate and temporal fossa (level C2). include retina and optic nerve ``` spine field Prone: chin tuck Sup: junction inf border of cranial field Inf: below S2 lat: cover vertebrae bodies ```

Question 50

ALL treatment technique

Answer 50

CNS prophylaxis -high risk; T cell diagnosis TBI: prep for BMT prescribed at umbilics TLDs--> head, neck, shoulder, Axilla (no build up), umbilics, head of femur, b/w knees (no build up) Tx of CNS -CSI Testicular irradiation

Question 51

Ewings sarcoma tx technique

Answer 51

spare uninvolved epiphysis's if is a growth plate shaping fields to maintain LN drainage (1cm strip) to avoid lymphedema Tx to femur -2/3 pt develop shortening; 1/3 fractures

Question 52

Retinoblastoma tx technique

Answer 52

EBRT used for bilateral or inoperable unilateral treat entire retina to avoid recurrence in ant eye brachy-iodine 125

Question 53

Wilms tumour prescribed dose borders and tx technique

Answer 53

Flank Most FH tumours respond to RT Stage I+II--> no RT Stage III-->1080 UFH Stage I&II-->1080 Stage III-->1980 Whole abdo and lung - 1050 <12mo - 2100/4 stage III Borders: Sup; dome of diaphragm med: cross midline PA nodes 4-6MV APPA

Question 54

Medullablastoma Tx borders

Answer 54

CSI-cranial Inf: include cribriform plate (high risk of recurrence) and temp fossa C2 (POST ORBIT NOT INCLUDED LIKE ALL) Sup: Flash to cover cranium CSI-spine Sup: jxn of inf border of cranium inf; below S2 (Thecal sac) lat: entire vertebrae w/ 1cm margin Posterior fossa-->boost 54-55Gy *again not necessary to include retina and optic nerve as done in ALL pts