Oncology Flashcards
Who develops transient myeloproliferative disorder?
10% of DS children develop transient abnormal myelopoeisis in the first three months of life
What is transient myeloproliferative disorder?
accumulation of immature megakaryoblasts in blood and liver (lesser degree in bone marrow)
What genetic mutation is transient myeloproliferative disorder associated with?
GATA 1
How does rasburicase work?
Urate oxidase –> promotes degradation of uric acid.
Langerhans cell histiocytosis - what is this?
Rare histiocytic disorder characterized by osteolytic bone lesions with infiltration by histiocytes. Can infiltrate nearly every organ, but most commonly bone and skin.
What are the most common adolescent breast lesions?
Juvenile fibroadenoma - Account for 30-50% of breast masses in adolescents.
What are most common x-ray findings in Ewings versus Osteosarcoma?
Ewing - most common X-ray finding is onion skinning or periosteal reaction (bone building on top of bone)
Osteosarcoma – most common X-ray finding is lytic lesions (sun burst pattern, building new born perpendicular to old bone)
5 childhood conditions that predispose to developing leukemia
Neurofibromatosis Type 1 Trisomy 21 Ataxia Telangiectasia Fanconi anemia Li-Fraumeni Syndrome
What type of leukemias present in childhood?
ALL (80%)
AML (15%)
CML
JMML
How many patients with leukemia will have circulating blasts at diagnosis?
80%
x-ray findings in bony leukemia involvement
- transverse radiolucent metaphyseal growth arrest lines
- periosteal elevation with reactive subperiosteal cortical thickening
- osteolytic lesions
- diffuse osteopenia/ osteoporosis
Other risk factors for leukemia (non-genetic conditions)
previous chemotherapy, radiation (including in utero), benzene
Prognosis of all
> 90%
prognosis of AML
60%
ALL - poor prognostic factors
- AGE: < 1 and > 10 yrs*
- WHITE CELL COUNT: > 50 *
- CNS and/or TESTICULAR DISEASE
- CYTOGENETICS (Good – t (12;21) = TEL-AML; hyperdiploidy / Poor – MLL gene rearrangement (11q23) / V. poor – t (9;22) = Ph chr and hypodiploidy)
-DISEASE RESPONSE
(MRD at the end of induction chemotherapy)
What brain tumour do patients with tuberous sclerosis develop?
15% develop subependymal giant astrocytomas (SEGA)
What brain tumour do patients with tuberous sclerosis develop?
15% develop subependymal giant astrocytomas (SEGA)
What brain tumour can patients with NF1 develop?
optic nerve glioma
Risk for pediatric renal medullary carcinoma?
Sickle cell trait!
How many children with leukemia will not have circulating blasts?
20%
ALL vs JIA - 3 factors that predict ALL
Low WBC < 4
Low-normal Plts 150-250
night time pain (hx)
(sensitivity 100% specificity 85%)
Poor prognosis in ALL is suggested by
Age < 1 yr, > 10yr
7 childhood conditions predisposing to leukemia
T21 NF1 Ataxia-telangiectasia Fanconi Bloom Noonan Li-Fraumeni
Most common type of childhood leukemia
ALL (80%)
Treatment of CML
tyrosine kinase inhibitor (imatinib)
X-ray findings in leukemia
transverse metaphyseal growth arrest lines
osteolytic lesions
diffuse osteopenia
fractures
Extramedullary disease in leukemia
chloroma leukemia cutis gingival hyperplasia HSM LNs Lymphomatous masses CNS disease* Testicular disease*
Good cytogenetics
tel-aml
trisomy 4, 10
poor cytogenetics
Ph chromosome
hypodiploidy
MLL
Generalized lymphadenopathy - definition
> 2 contiguous groups
Chronic lymphadenopathy
> 4 weeks
Leading cause of death among survivors of hodgkin’s lymphoma > 15 yrs post treatment
second malignancy
then cardiovascular disease
Most common presentation of Burkitt’s lymphoma
abdominal mass
w/ pathologic lead point
Doubling time of Burkitt’s lymphoma and subsequent risk?
doubling time 24 hrs
risk of TLS
DDx anterior mediastinal mass
lymphoma (hodgkins, NHL) thymus thymoma teratoma thyroid
Presentation of SVC syndrome
plethora of the face/upper body
Change in LOC
Coma/stroke
Causes of SVC syndrome
Mass (extra luminal)
Thrombus (intra luminal)
Two most common posterior fossa tumours
Cerebellar astrocytoma
Medulloblastoma
Symptoms of posterior fossa tumour
diplopia
headache
ataxia
Most common malignancy childhood brain tumour
medulloblastoma
What is posterior fossa?
cerebellum
brainstem
Signs of supratentorial mass
behavioural problems
neuroendocrine deficits
seizures
visual changes
Brainstem triad
Ataxia
Long tract signs (hyperreflexia)
Cranial nerve involvement
Dull bone pain worse at night, better with NSAIDs
X-ray: well-defined lucency surrounded by sclerotic bone
Osteoid Osteoma
Non-painful, bony mass
Often in metaphysis of long bones
May have stalk-like base
Osteochondroma
Expansile lesion within bone filled with blood/solid components
often in metaphysis
aneurysmal bone cyst
Most common bone tumour in adolescents
osteosarcoma
Osteosarcoma - sites of metastases
lung, bone
Ewing’s sarcoma involves which bones?
Lots!
flat bones of axial skeleton, long bones (diaphysis)
Features of Ewing’s on x-ray
moth-eating appearance w/ “onion skin” periosteal reaction
associated soft tissue mass
x-ray appearance Osteosarcoma
sunburst reaction
“bone outside of bone”
Paraneoplastic syndromes in neuroblastoma
Opsoclonus-myoclonus-ataxia
Vaso-active intestinal peptide (VIP) secretion - leading to diarrhea and hypokalemia
Prognosis of a child with neuroblastoma < 12-18 months?
Favourable
Staging of neuroblastoma
CT/MRI MIBG Bone scan Bone marrows Urine HVA/VMA Biopsy (for biology, n-myc)
Infant with stage 4S neuroblastoma
Skin involvement (blue spots)
Hepatomegaly
Bone marrow < 10%
NO mets to bone
May not require treatment!
Four risk factors for hepatoblastoma
BWS
hemihypertrophy
family hx of FAP
prematurity
Tumour lysis
hyperkalemia
hypocalcemia
hyperphosphatemia
hyperuricemia
Definition of hyperleukocytosis, organs that it can affect
WBC > 100
Brain (intracranial hemorrhage)
Lungs
Kidney
Management of hyperleukocytosis
Hydration!
Rasburicase
Platelets - at risk of bleeding (plts and FFP do not increase viscosity)
Patient with hyperleukocytosis. When would you transfuse pRBCs?
Only if symptomatic from anemia (heart failure) - give small amount of blood (2-5 cc/kg)
Oncologic emergencies
TLS Fever + Neutropenia Cord compression Mediastinal mass APML
Most common bug in febrile neutropenia
gram positive organisms
How do you manage an immunocompromised child exposed to varicella? (with no symptoms)
VZIG
3 side effects of vincristine
siADH
constipation
jaw pain
3 side effects of peg-asparaginase
pancreatitis
anaphylaxis
thrombosis
3 side effects of bleomycin
pulmonary fibrosis
hypersensitivity, rash
side effects of cyclophosphamide
hemorrhagic cystitis
infertility
side effects of etoposide
allergic reaction secondary malignancies (leukemia)
Side effects of cisplatin
N/V
hearing loss
nephrotoxicity
methotrexate side effects
renal failure
mucositis
leukoencephalopathy
late effects after neck radiation
hypothyroidism
poor prognosis in LCH is associated with…
bone marrow involvement (pancytopenia)
hemorrhagic skin lesions
hepatomegaly
young age at diagnosis (< 18 mos)
diagnostic test for LCH?
biopsy of involved bone/skin
Autologous transplant - what is the principle
Give them back their own bone marrow as a “rescue”
Allogeneic transplant side effect
Graft versus host disease
Acute GVHD commonly affects what organs
Liver
Gut
Skin
RFs for bad GVHD
mismatched/unrelated donor
bone marrow transplant
older age
when does chronic GVHD occur?
> 100 days
can affect any organ!
What is osteopetrosis?
inherited bone disease involving dense, sclerotic bones prone to fracture
Cure: BMT
What is Kasabach-Merritt
DIC associated with capillary hemangiomas
What investigation does a child with WAGR need regularly?
Abdominal ultrasound for Wilm’s tumour
Wilm’s tumour is associated with
NF 11p Denys-Drash Aniridia, WAGR B-W Hemihypertrophy
What predisposes to leukemia:
- exposure to rads
- mom or dad with leukemia
- NF1
NF1
Two tumours that children with Beckwith-Wiedemann are at increased risk of?
hepatoblastoma
Wilms tumour
Retinoblastoma - increased risk of what other cancers?
Osteosarcoma
Melanoma
MEN2 - what types of cancers are they at risk of?
medullary thyroid cancer
pheochromocytoma
familial adenomatous polyposis - at risk of what type of cancers?
colorectal ca
hepatoblastoma
thyroid ca
