ONCOLOGY Flashcards
Commonest pediatric malignancy
Leukemia
2nd most common & 3rd most common malignancy.
CNS tumor
Embryonal and sarcomas
What are the 2 peaks of childhood malignancies
Early childhood
Adolescence
There’s an increased risk of cancers with?
Neurofibromatosis type 1 & 2
Down syndrome
Beckwith - wiedemann syndrome
Tuberous sclerosis
Ataxia telangiectasia
Klinefelter’s syndrome
The types of cancers associated with
1. Down syndrome
2. Beckwith-wiedemann syndrome
3. Tuberous sclerosis
4. Ataxia telangiectasia
5. Klinefelter’S syndrome.
- Leukemia
- Kidney, liver cancers
- Kidney cancer
- Skin cancers
- Testicular cancers
Infections and their associated pediatric malignancies.
Hepatitis B&c- HCC
HPV - cervical cancer
HIV - kaposi sarcoma
Peak age of onset of acute lymphoblastic leukemia
Between 2-6 years
Most pediatric malignancies are common among males or females?
Males.
Possible etiological factors for acute lymphoblastic leukemia
Down syndrome
Ionizing radiation
advanced maternal age. (>35 years)
Predominant cell type in acute lymphoblastic leukemia
Pre b-cells
Bad prognostic factors of acute lymphoblastic leukemia
Male
<1 year and >10 years
T cells
Mediastinal mass
WBC> 25x109/L
CNS involvement
t(9:22)
t(4:11)
Hypodiploidy
Longer time to respond to treatment
Two chromosomal dislocations that are bad prognostic features of ALL
t(9:22)
t(4:11)
Ix of ALL
Blood picture
Bone marrow aspiration
CBC
Blood picture findings of ALL
Thrombocytopenia
blast cells might not be seen
CBC findings of ALL
WBC high
Platelet, Hb reduced
Rx of ALL
Chemotherapy (vincristine)
Steroid (Dexa, prednisone)
CNS therapy of ALL route
Intrathecal - directly to spine
CNS therapy of ALL given on
At the start of treatment
once more during induction
Maintenance phase of ALL lasts
2-3 years
Survival of ALL
> 90%AT five years
Second most common malignancy in paeds
Brain tumors
T/F
1.Brain tumors are mostly primary.
2.Brain tumors are mostly malignant
1.T
2.T
Commonly seen age group for brain tumors in children
<= 7 years
Infratentorial and midline brain tumors clinical features
Features of increased ICP
Disorders of the equilibrium, gait, coordination
Blurry vision
Diplopia
Nystagmus
Supratentorial brain tumors features
Changes in personality
mentation
motor weakness
sensory changes
speech disorders
seizures
reflex abnormalities
hand preference
Imaging standard of brain tumors
MRI
Medulloblastoma age of presentation
5-7 years
Bad prognostic factors of medulloblastoma
Young age
Clinical features of medulloblastoma
Increased ICP
cerebellar signs
Mx of medulloblastoma
Surgery
Chemo
Radiotherapy
location of craniopharyngioma
within the suprasellar region
MRI shows…. in craniopharyngioma
Calcification
Clinical features of craniopharyngioma
panhypopituitarism
growth failure
visual loss- bi-temporal hemianopia
Mx of craniopharyngioma
Surgery only.
Signs of tumors of the brainstem
Motor weakness
cranial nerve defects
cerebellar defects
increased ICP
Torticollis
Why is there a poor prognosis on tumors of the brain
Because it is difficult to operate
Mother says while giving a bath she noticed a lump on the babies abdomen. the child is always irritable and sweating. Once taken to the GP clinic it was found out the child has high BP. Dx?
neuroblastoma
Tumor/ lump crossing the midline+ calcifications
Neuroblastoma
Tumor/ lump not crossing the midline and no calcifications
Renal tumors
Neuroblastoma cell type
Sympathetic ganglia.
Most commonly diagnosed neoplasm in infants
Neuroblastoma
Neuroblastoma age of presentation
<2 years
poor prognostic factors of Neuroblastoma
advanced stage
advanced age
characteristics of a Neuroblastoma
firm nodular mass
cross the midline
calcifications on the X- Ray
Signs of metastasis in Neuroblastoma
fever
irritability
failure to thrive
bone pain
bluish subcutaneous nodules
orbital proptosis
periorbital ecchymosis
MC site of metastasis in Neuroblastoma
long bones bone marrow
skull bone marrow
liver
lymph nodes
Skin
CNS signs of Neuroblastoma
Horner’s syndrome
Spinal cord compression
Opsomyoclonus (Rapid eye movements, jerky movements of arms and legs)
Some Neuroblastoma tumors secrete…..
Catecholamines
Clinical features of Neuroblastoma that secrete catecholamines
Increased sweating
Hypertension
secretory diarrhea
4S stage of neuroblastoma
subcutaneous tumor nodules
Massive liver involvement
Subcutaneous tumor nodules of Neuroblastoma appearance is also known as
Blueberry muffin appearance
Ix of Neuroblastoma
breakdown products of adrenaline on urine - HVA, VMA
CT
Bone marrow
USS Abd
Mx of Neuroblastoma
surgical resection +/- chemo +/- radiotherapy
Nephroblastoma is also known as
Wilm’s tumor
Age of onset of Nephroblastoma
2-5 years
Wilm’s tumor is associated with
Anirida
WAGR syndrome
Beckwith Wiedemann syndrome
Neurofibromatosis 1
Wilm’s tumor clinical features
abdominal mass often detected by doctors.
no calcification
Rarely crosses the midline
HTN
hematuria
Anaemia
Fever
Is hematuria common in Wilm’s tumor
no
Is bilateral involvement of kidneys common in Wilm’s tumor
Rare
Mx of wilm’s
surgery and chemo
Possible
X ray findings of wilm’s
Chest and abdomen mets in the lungs
Are all cases of retinoblastoma bilateral
no. some are unilateral
Can retinoblastoma be misdiagnosed?
Yes. U/L can be misdiagnosed
Gene involved in Retinoblastoma
RB1 inactivated
Sx of retinoblastoma
Leukoria
Leukoria
White pupillary reflex (usually red when shining a light)
DDs of leukoria
Cataract
Visceral larva migrans
chorodial coloboma
ROP(Retinopathy of prematurity)
Retinoblastoma
Dx test contraindicated in retinoblastoma
Pupillary biopsy
Ix of retinoblastoma
Orbital USS
CT
MRI
Rx of retinoblastoma ( Primary and secondary goals)
Primary - cure
Secondary- preserving vision
Mx modality for B/L involvement of retinoblastoma
Chemo - laser photocoagulation or cryotherapy
Situations where enucleation is performed in retinoblastoma
No potential for useful vision
optic nerve involvement
Enculeation?
Removing the eye
Retinoblastoma has a good prognosis if it’s associated with
Osteosarcoma
Hepatoblastoma age of presentation
<3 years
Hepatoblastoma is associated with
FAP
Beckwith- wiedemann syndrome
Hemihypertrophy
Sx of Hepatoblastoma
Large asymptomatic abdominal mass on the right side (R>L)
Ix of Hepatoblastoma
alpha feto protein levels high
Bilirubin and liver enzyme levels in Hepatoblastoma
Usually normal
Mx of Hepatoblastoma
Surgery/ Chemo
HCC is seen ….. (age group)
Adolescents
HCC is associated with
Hep B,C
Galactosemia
Glycogen storage disease
alpha- 1 anti trypsin deficiency
biliary cirrhosis
chronic hereditary tyrosinemia
HCC presents as
Hepatic mass
Abdominal distension
Anorexia
Weight loss
Abdominal pain
AFP ( Alpha feto protein levels ) in HCC vs and Hepatoblastoma
Levels Hepatoblastoma> HCC
HCC prognosis is usually good or bad
Bad
Characteristic age of malignant tumors of bone
2nd decade
Two examples of malignant tumors of the bone
Osteosarcoma
Ewing’s tumor
T/F
Malignant tumors of the bone is common in males than females
True
Etiology of osteosarcoma
Retinoblastoma
Paget disease
Radiotherapy
Li- Fraumeni syndrome
Affected part of bones in osteosarcoma
Metaphysis of long bones
Affected part of bones in Ewing sarcoma
Diaphysis of long bones
Flat bones
Sx of osteosarcoma
Local pain and swelling
Osteosarcoma has a Hx of
Injury
Sx of Ewing sarcoma
Local pain and swelling
Fever
Radiographic findings of Osteosarcoma
Sclerotic destruction
Sunburst appearance
Radiographic findings of Ewing sarcoma
Primarily lytic
Onion skinning appearance
Onion skinning appearance in Ewing sarcoma is due to
Multilaminar periosteal reaction
Mets of both Osteosarcoma and Ewing sarcoma- sites
Lung
Bones
Mx of osteosarcoma
Chemo > surgery
Mx of Ewing’s sarcoma
Chemo
Radiotherapy
Outcome of bone tumors with mets at presentation
<= 20% survival