Oncology Flashcards
what are the two classes of acute leukemia
ALL: 80%
AML: 15%
what are the two types of chronic leukemia
CML
JMML
what percentage of patients with leukemia will not have blasts on peripheral smear
20%
what are some symptoms of leukemia
lack of normal bone marrow output
anemia- pallor, fatigue
thrombocytopenia- bruising, bleeding
infection
infiltration by blasts HSM lymphadenopathy bone pain limp fever
Sanctuary sites
CNS symptoms
testicular
list 5 childhood conditions that predispose them to the development of leukemia
down syndrome NF1 Fanconi anemia Li- Fraumeni syndrome Noonan Bloom Ataxia telangiectasia WAS SCID DBA SCN
what type of leukemia can be seen in babies with trisomy 21? Do they need treatment?
transient myeloproliferative disorder
present with increased WBC, peripheral blasts, pancytopenia, hepatosplenomegaly
typically do not need any treatment
what is the most common leukemia is children with down syndrome
ALL
what investigations are done to help diagnose leukemia
CBC+ smear
bone marrow aspirate and biopsy
LP
Testicular exam
what percentage of blasts in the BM is required for the diagnosis of leukemia?
> 25% blasts in the BM
what is the most common childhood cancer?
ALL
what is the peak age for ALL
2-5 years
is ALL usually B cell or T cell?
80% B cell
15% T cell
how long is treatment for ALL? what’s the prognosis?
2.5-3.5 years
prognosis is good, 90% cure
what are the prognostic factors for ALL
age <1 WBC >50 testicular or CNS disease cytogenetics( t9:22 is very poor) MRD
what is MRD
minimal residual disease
disease reassessment after 1 month of therapy
one of the most important prognostic indicators
how long is the therapy for AML? what’s the cure rate?
6 months of intensive chemotherapy
cure rate is 60%
what is hyperleukocytosis
WBC >100
how would you manage a patient with hyperleukocytosis
hydration- 1.5x maintenance allopurinol rasburicase platelets antibiotics call ICU
what are the common chemotherapy drugs for ALL
Vincristine
steroid
asparaginase
daunorubicin
a patient presents with a supraclavicular lymph node, what is the next step?
CXR
have to rule out a mediastinal mass
safety first!
what is a common cancer seen in age 15-19
Hodgkins lymphoma
how many blasts are seen on bone marrow aspiration for lymphoblastic lymphoma?
<25%
what investigations would you do in an 8 year old male with an abdominal mass
CBC + smear LFTs electrolytes renal function LDH Urate
what is the most common cause of lymphadenopathy
infection
what size lymph node is normal in cervical area? pelvic?
1cm in cervical area
1.5cm in pelvic area
what does a reed Sternberg cell mean?
Hodgkin’s lymphoma
what are the 3 classes of non-hodgkins lymphoma?
Mature B cell: Burkitt’s
Mature T cell: Anaplastic
Precursor T or B: Lymphoblastic lymphoma
what investigations should be done prior to chemotherapy for Hodgkins lymphphoma
echo
PFT
what is the expected outcome for Hodgkins lymphoma?
90% cure
but huge consequence of cure
what are some of the late toxicities associated with Hodgkin’s lymphoma
infertility pulmonary fibrosis high risk of secondary malignancy- breast ca, thyroid ca psychosocial cardiovascular disease, heart failure
at what age do we typically see non-hodgkins lymphoma
grade 4 or 5 (8 to 10 years old)
what group of conditions is non-hodgkins lymphoma associated with?
up to 10% of children with congenital or acquired immunodeficiency develop NHL
what are some conditions associated with non-hodgkins lymphoma?
ataxia telangiectasia wiskott aldrich syndrome congenital hypogammaglobulinemia Post solid organ transplant HIV
how do patients with lymphoblastic lymphoma present?
bone marrow aspirate with <25% blasts
large mediastinal mass
elevated white count
how does Burkitts lymphoma present?
abdominal mass 80%
waldeyer’s ring 20%
what is waldeyer’s ring?
The palatine tonsils, nasopharyngeal tonsil (adenoid) and lingual tonsil constitute the major part of Waldeyer’s ring
what is the most important thing to worry about for burkitts lymphoma
Tumor lysis syndrome
fastest proliferating malignancy that exists
what are the concerns with a mediastinal mass?
airway compromise- CXR, CT, PFTs
SVC syndrome- echo
RVOT obstruction- echo
cardiac tamponade- pulses paradoxus, ekg, echo
what is the ddx for anterior mediastinal mass?
thyroid
terrible lymphoma
teratoma
thymus
how does superior vena cava syndrome present?
dilated neck veins
facial swelling
altered mental status
what are some management options for a patient with a mediastinal mass
DO NOT LAY FLAT biopsy under local CT neck/chest/abdo/pelvis PET scan bilateral bone marrow aspirate/biopsy Echo consult anesthesia, ICU, Oncology
for what tumors do we worry about tumor lysis syndrome
Burkitts, other NHL
lymphoma
leukemia
has been reported in solid tumors
what are the laboratory features associated with tumor lysis syndrome
elevated k
elevated phosphate
elevated urate
low calcium
can have a metabolic acidosis and therefore low bicarb
what are 2 treatment options for high urate?
rasburicase
allopurinol
what are the most common solid tumors in children?
brain tumors
what is the most common type of glioma
astrocytoma
sometimes these terms are used interchangeably
what are common low grade astrocytomas in supratentorial area? infratentorial area?
supratentorial- optic pathway glioma
infratentorial- pilocytic astrocytoma
what is the most common malignant brain tumor
medulloblastoma
what is the most common type of brain tumor
glial tumors
where are most brain tumors found in children?
infratentorial
what are the common infratentorial brain tumors in children
1) astrocytomas
- cerebellar
- brainstem (diffuse intrinsic pontine glioma)
2) medulloblastoma
3) ependymoma
what are signs of supratentorial brain tumor
seizures** visual changes hemiparesisis hemisensory loss hyperreflexia
DI can indicate what type of brain tumor?
germinoma
what are signs of infratentorial brain tumor
raised ICP- h/a, N/V, 6th nerve palsy, papilledema, lethargy, cushings triad
ataxia
what syndromes are associated with gliomas/astrocytomas
NF1
TS
what syndromes are associated with medulloblastoma
FAP Gorlin Rubinstein- Taybi Li fraumeni Fanconi anemia
gorlin syndrome is associated with which cancers?
PTCH mutation SHH pathway mutation *GROUP
Gorlin syndrome- associated with basal cell carcinoma primarily
but increased risk of medulloblastoma
also associated with Odontogenic cysts, skeletal and cutaneous abnormalities
Li- Fraumeni syndrome
mutation in tumor suppressor gene, p53. breast cancer osteosarcoma sarcomas brain tumors leukemias
what could a 6th nerve palsy indicate?
brain tumor!!
what is considered the gold standard treatment for pediatric oncology patients
participation in clinical trials
which tumor is associated with claw sign on CT
wilm’s tumor
Which tumour diagnosis would be supported by a raised AFP level? a) Hepatoblastoma b) Wilms tumour c) Neuroblastoma d) Burkitt’s lymphoma
hepatoblastoma
What is the commonest site for metastases in Osteosarcoma? a) Lymph nodes b) Adjacent bone (“skip lesions”) c) Brain d) Lung
lung
Which of these IS a long long-term side effect of Cisplatin? a) Hepatic fibrosis b) Hearing loss c) Infertility d) Cardiomyopathy
hearing loss
Which of these is NOT a short short-term side effect of Doxorubicin a) Myelosuppression b) Hair loss c) Nausea/vomiting d) Haemorrhagic cystitis
hemorrhagic cystitis
what type of cell do these suggest:
CD19
CD3
MPO
CD19- B cell
CD3- T cell
MPO- myeloid marker
Acute Lymphoblastic Leukaemia comprises what proportion of childhood cancer cases? a) 10% b) 15% c) 25% d) 40%
25%
Which of these drugs MUST NOT be given intrathecally?
a) Hydrocortisone
b) Vincristine
c) Methtrexate
d) Cytarabine
Vincristine- huge neurotoxin
Intrathecal administration:
- Severe demyelination
- Severe encephalopathy
- Severe pain
- Death, usually
Which of these is NOT a B symptom?
a) Drenching night sweats (within the last month)
b) Persistent itch
c) Weight loss >10% (over six months)
d) Fevers >38 o C
persistent itch
what are the 2 treatment options for SVC syndrome
- steroids
2. radiotherapy
staging for lymphoma (stage 1, 2, 3, 4)
Stage 1- Single nodal group
Stage 2- 2 or more nodal groups on SAME side of diaphragm
Stage 3- 2 or more nodal groups on BOTH sides of diaphragm
Stage 4- Involvement of extra-lymphoid tissues
what is the definition of febrile neutropenia
Fever
> 38.3 oC x 1, or > 38oC over at least 1 hour
Absolute neutrophil count
< 0.5 x 10^9/L
+/ -evidence of sepsis = hypotension needing fluid resuscitation
what are some long term side effects from radiation therapy
Fibrosis/scarring Growth impairment (children) Endocrinopathies Second malignancies Xerophthalmia Xerostomia Cataract Intellectual impairment
what are some short term side effects from radiation therapy
Nausea/vomiting
Fatigue
Radiation “burns”
what is an emergent treatment for hydrocephalus secondary to brain tumor
dexamethasone
A 6 yo girl presents with 2 month hx of head tilt,
diplopia, papilledema and ataxia
Where is the lesion?
posterior fossa
what signs would suggest brainstem involvement
head tilt
cranial nerve palsies
A 3 year old girl presents with abdominal
distention and pallor. A mass is palpable on exam.
Hgb 80, normal WBC.
What is your ddx?
List 3 investigations that may help you
narrow your ddx pre biopsy
- hepatoblastoma
- neuroblastoma
- wilm’s tumor
- lymphoma
- AFP
- urine VNA, HVA
- ultrasound
what are some physical exam
features that may be associated with neuroblastoma?
hypertension abdominal mass bruising below eyes bone pain nystagmus
Opsoclonus-myoclonus-ataxia (OMA) is seen in which of the following malignancies? a) Leukemia b) Rhabdomyosarcoma c) Neuroblastoma d) Medulloblastoma
neuroblastoma
what are two unique paraneoplastic syndromes associated with neuroblastoma?
• Opsoclonus-Myoclonus-Ataxia
Rapid eye movements, ataxia, myoclonus
• VIP-induced diarrhea
get hypokalemia and dehydration
Better prognosis with neuroblastoma is associated with? a) Female b) High excretion of VMA/HVA c) Age < 12-18 months d) Normal blood pressure e) Unilateral disease
age <12- 18 months
what is the most common renal tumor in children
wilm’s
what are 3 predisposing syndromes for wilm’s tumor
Beckwith-Wiedemann
Hemi-hyperplasia
WAGR
what are 3 predisposing conditions for hepatoblastoma
BWS
Hemihyperplasia
FAP – family history of polyps
Prematurity/LBW
90% have ↑↑↑ alphafetoprotein=marker of embryonic liver tissue
A child presents with a distended abdomen. An US
shows an adrenal mass and hepatomegaly. Which
of the following tests will make the diagnosis?
a) MIBG
b) Serum AFP
c) Urine VMA/HVA
d) CT chest/abdo
Urine VMA/ HVA
what is the most common intraabdominal tumor in children
Neuroblastoma is the most common intraabdominal
tumor in children
wilm’s= second most common
14 year old female presents with pain above right
knee, worse at night and with activity. On exam there
is no fever, but there is tenderness to palpation above
the knee.
X-ray shows periosteal elevation and mottling of the
distal femur. The most likely diagnosis is:
a) Osteomyelitis
b) Osteosarcoma
c) Osteoid osteoma
d) Ewing’s sarcoma
e) Sub-periosteal hematoma
osteosarcoma
how does an osteoid osteoma present
dull pain at night
improves with NSAIDS
on x ray: well defined lucency surrounded by sclerotic bone
where does osteosarcoma occur
metaphysis of long bones
what is the xray appearance for osteosarcoma
sunburst appearance
what is the xray appearance for Ewings sarcoma
onion skinning
what are two predisposing conditions for osteosarcoma
LFS, RB
where does Ewings sarcoma occur?
diaphysis of long bones
axial skeleton
what are the treatment options for osteosarcoma
chemotherapy and surgery
what are the treatment options for Ewings sarcoma
chemo, surgery and radiation
A 12 year old boy presents with an X-ray (described, no picture given) of mottled bone on right rib with new bone forming on top. What is the most likely diagnosis? a) Osteosarcoma b) Ewing sarcoma c) Osteoid osteoma d) Enchondroma
Ewing sarcoma
List 3 side effects of each of the following drugs: • Vincristine • Asparaginase • Prednisone
Vincristine- SIADH, peripheral neuropathy, constipation
Asparaginase- Allergic reaction, hepatopathy, coagulopathy/thrombosis, pancreatitis
prednisone- Hypertension, hyperglycemia, AVN, cataracts
The most common organism isolated in episodes of febrile neutropenia is? a) PCP b) E.Coli c) CONS d) Pseudomonas e) Candida
CONS
gram positives> gram negatives
Wilms tumor is associated with all of the following except? a) 11p deletion b) BWS c) Wiskott Aldrich syndrome d) NF-1 e) Anirida
WAS
Which condition is most associated with childhood leukemia? a) Electromagnetic field exposure during pregnancy b) NF-1 c) Family history of adult leukemia
NF1
Child with hemi-hypertrophy. What
condition would you suspect that would
require serial follow-up?
Beckwith Wiedemann syndrome
Hepatoblastoma: AFP q 3 months until age 4
Wilms’ Tumor: Abdo U/S q 3 months until age 8
An 11 mo. old presents with generalized scaly rash
worse in diaper area. He also has exopthalmos
and HSM. Xrays show bony lucencies of the skull.
What is the likely dx?
a) Langerhans cell histiocytosis
b) Neuroblastoma
c) ALL
d) Multifocal osteomyelitis
Langerhands cell histocytosis
- can have CNS involvement and present with DI
This baby has had a rash for 4 months, refractory
to barrier creams and clotrimazole. She soaks
through 12 diapers a day
What is your diagnosis?
What test would you do to
confirm your diagnosis?
LCH
skin biopsy
Poor prognosis (mortality risk) in LCH is associated with a) Mastoiditis b) Pancytopenia c) Lymphadenopathy d) Chronic lung disease e) Vertebral body involvement
pancytopenia
A newborn presents with an abdominal mass,
thrombocytopenia, systolic heart murmur, and a
murmur over the liver. An ultrasound shows a
3cm mass in the liver.
What would be your next investigation?
a) DIC work up
b) Bone marrow evaluation
c) Echocardiogram
d) Liver function tests
DIC workup
Kasabach-Merritt
• Disseminated intravascular coagulation with capillary hemangiomas
• Associated with kaposiform hemangioendotheliomas or tufted angioma
• Thrombocytopenia from sequestration/ destruction of platelets in vascular malformation
• Treatment can include steroids and chemotherapy
what are the common side effects for: bleomycin cisplatin/carboplatin MTX Cyclophosphamide Vincristine
bleomycin- pulmonary fibrosis
cisplatin/carboplatin- ototoxicity, renal toxicity
MTX- myelosuppression
Cyclophosphamide- hemorrhagic cystitis
vincristine- peripheral neuropathy, SIADH, constipation
6 month old baby with pallor, hepatosplenomegaly, irritable. Xray shown with very white bones. Diagnosis:
osteoporosis
Complication of doxorubicin?
- Restrictive cardiomyopathy
- Dilated cardiomyopathy
- Hypertrophyic cardiomyopathy
- Pericarditis
dilated cardiomyopathy
Newborn has a flank mass. What is the most likely cause
a. Wilms tumour
b. Hydronephrosis
c. Polycystic kidney
hydronephrosis
You are a rural doctor seeing a child who had just completed chemotherapy. What side effects of anthracyclines do you need to monitor for? Hearing loss Cardiac dysfunction Thyroid dysfunction Renal dysfunction
main adverse effect is cardiotoxicity
Which is the most permanent toxicity of vincristine?
Peripheral neuropathy
Seizures
Constipation
peripheral neuropathy
Child with pancytopenia, short stature, abnormal thumbs. What is the diagnosis?
Fanconi anemia
Clinical manifestations:
Hyperpigmentation of the trunk, neck, intertriginous areas, as well as cafe-au-lait spots and vitilgo
Short stature (50%)
Absence of radii and thumbs that are hypoplastic, supernumerary, bifid, or absent
The “r” radial pulse may be weak or absent
Anomalies of the feet, congenital hip dislocation, and leg abnormalities are seen
In males: underdeveloped penis, undescended, atrophic or absent testes, hypospadias or phimosis
Females: malformations of the vagina, uterus and ovaries
FA “facies”: microcephaly, small eyes, epicanthal folds, and abnormal shape, size or positioning of the ears
