Oncology Flashcards
Leading cause of death in 1-19 YO
pediatric cancer
Warning signs for childhood cancer
Continued, unexplained weightloss
Headaches - oftening w/ vomiting- early night/ early morning
Increased swelling or pain in bones, back, legs,
Lumps/mass
Development of excessive bruising, bleeding rash
Constant infections A whitish colour behind the pupil Nausea which persists of vomiting w/o nausea Constant tiredness or paleness Eye or vision changes Recurrent FUO
Most common malignancy in children
Acute lymphoblastic leukemia (ALL)
What causes ALL
uncontrolled proliferation of immature lymphocytes
B-precursor lineage (85%)
Peak incidence: 2-5 YO
Cause unknown; genetic factors (down syndrome); viral associations (viral infections, exposure to radiation)
Presentation of ALL
intermittent fever, fatigue, pallor bleeding (50%) - petechiae, purpura BONE PAIN! (pelvis, vertebral bodies, legs) hepatosplenomegaly LAD
Lab finding
anemia/thrombocytopenia w/ normal or depressed WBC
Neutropenia in differential (ANC <1000)
Peripheral smear: lymphoblasts
Dx of ALL
bone marrow bx (leukemic blasts replacing normal marrow)
Tx for ALL
may take 2-3 years to complete
Chemo (1st line)
Hematopoietic stem cell transplant (HSCT) - best form matched sibling
Prognosis for ALL
> 85% survival rate
tumor lysis syndrome
oncologic emergency!
Hyperkalemia, hyperuricemia, hyperphosphatemia
acute renal failure
Acute myeloid leukemia cause
myeloblasts cant differentiate into mature cells
Predisposing factors for AML
no risk factors often
congenital: down syndrome, NF1
Environmental: radiation, benzene (smoking) and previous chemo
Clinical findings for AML
fatigue, pallor, bleeding or infection (fever) CNS INVOLVEMENT (5-15%) - h/a, lethargy, mental status change, cranial nerve palsies
Lab findings for AML
anemia, thrombocytopenia, neutropenia (ANC <1000)
WBC >100,000! (2%)
Hyperleukocytosis (>75,000) may be associated w/ life-threatening complications- Medical emergency
Hyperleukocytosis
AML
Dx of AML
smear: myeloblasts >20% Auer rods (pathognomoic)
Dx requires both:
- bone marrow bx showing >20 blasts
- leukemic cells must be of myeloid origin
Tx for AML
chemo
HSCT
Prognosis
65-75% survival
CML
myeloproliferative disorder - uncontrolled proliferation of mature and maturing granulocytes
rare (5% of child canger)
Philadelphia chromosome
CML; translocation between chromosome 9 and 22
Presentation of CML
chronic phase –> accelerated phase –> blast phase
most asymptomatic bone pain fever, NIGHT SWEATS, fatigue (B sx) pallor, ecchymosis MASSIVE SPLENOMEGALY, variable hepatomegaly
Lab findings for CML
anema, thrombocytosis, and marked leukocytosis
smear: myeloid cells in all stages of maturation, increased basophils and blasts
blast cells >20% = blast crisis
Dx for CML
smear w/ myeloid cells in all stages
confirmation: philadelphia chromosome
Tx for CML
TKI*
HSCT
TKI
TK allows for dysregulation cellular proliferation
50% of childhood lymphomas
Hodgkin Lymphoma
Etiology of hodgkins
4:1 male predominance
familial, EBC association
arises in lymph nodes and spreads to contiguous nodal groups
Diagnostic for hdogkins
Reed-sternberg cells (germinal center B cells that have undergone malignant transformation)
Response to to hodgkinds
children have better response that adults
Presentation of hodgkin lymphoma
painless cervical or supraclavicular adenopathy
Mediastinal mass (75%)
B symptoms
complication w/ hodgkin lymphoma
SVC syndrome- dyspnea, cough, orthopnea, facial/upper extremity edema
Dx of hodgkin lymphoma
tissue bx w/ reed-sternberg cells
Auer rods
AML
Staging of hodgkin lymphoma
CXR
CT scan (chest, abdomen, and pelvis)
bone marrow bx
Tx for hodgkins
chemo
radiation
HSCT
Prognosis for hodgkins
90% 5-10 survival rate
NHL epidemiology
5th most common pediatric cancer
3:1 male predominance
arises in lymphoid tissue and spreads to distant nodes
NHL is associated w/
congenital and acquired immunodeficiency syndromes
EBV
NHL children
worse than in adults; rapidly proliferating, high-grade, diffuse malignancies
Clinical findings for NHL
fast, 1-3 weeks
enlarging, non-tender LAD
ab pain, b symtoms
hepatomegaly/splenomegaly in advanced stage
Dx of NHL
tissue bx
Tx for NHL
chemo
HSCT for those that relapse
watch for tumor lysis
Evaluation for brain tumor
measure head circumference
observe gait
Most common solid tumor of childhood
brain tumors
Presentation of brain tumor
AM h/a, vomiting, papilledema (optic nerve swelling)
younger children: vomiting, unsteadiness, lethary, irritability, macrocephaly, FTT, delayed development
Older: h/a, visual sx, seizure, focal neuro deficits, school failure and personality changes
Imaging for brain tumor
MRI (preferred) CT scan (less time)
Dx of brain tumor
tissue bx
Types of brain tumors
- Glial - astrocytomas, ependymomas
2. Nonglial: medulloblastoma
Tx for brain tumor
- surgical removal
2. radiation/chemo
Prognosis for brain tumor
60-90% 5-10 year survival w/ low grade astrocytoma
Most common abdominal tumor
neuroblastoma
Most common solid neoplasm outside of the CNS
neuroblastoma
Most common site for neuroblastoma
adrenal gland
Clinical manifestation of neuroblastoma
abdominal mass (firm, fixed and irregular shape; EXTENDS BEYOND MIDLINE) bone pain from metastatic disease fever, weight loss, irritability, ab pain, anorexia
Lab findings for neuroblastoma
anemia URINARY CATECHOLAMINES (dopamine, Epi, NE)
Tx for neuroblastoma
surgical resection coupled w/ chemo
surgery alone if low grade
radiation sometimes necessary
Prognosis of neuroblastoma
<40% survival in chidlren w/ high-risk disease
Wilms Tumor aka
Nephroblastoma
Second most common abdominal tumor in children
Wilms tumor (nephroblastoma)
Age nephroblastoma is common
2-5 yo
Clinical findings for wilms tumor
asymtomatic ab mass/swelling
RARELY CROSSES MIDLINE
smooth, firm, well demarcated
can extend inferiorly into pelvis
fever, hematuria, HTN
Dx for wilms
U/S or CT of abdomen, CT of chest (can spread to lungs)
bx or surgical excision is diagnostic
Tx for wilm’s tumor
surgical exploration
chemo
radiation
Prognosis for wilm’s
90% overall 5 year survival
Most common primary bone malignancy in peds
osteosarcoma
Epidemiology of osteosarcoma
male predominance
13-16 yo
occurs in long bones (metaphysis) - distal femor (40%)
Signs of bone tumor
bone pain at the site
mass formation
fracture through the area of cortical destruction
antalgic gait (due to pain)
Dx of osteosarcoma
X-ray- destruction of normal trabecular pattern and irregular margins
MRI- eval of soft tissue involvement
Bonescan and CT of the chest (metastasis)
Imaging and bx
Treatment of osteosarcoma
surgery
chemo
Prognosis of osteosarcoma
70-75% long term survival w/ localized tumor
Second most common primary bone tumor in peds
ewing sarcoma
Epidemiology of ewing sarcoma
M>F in 2nd decade of life
long bones (diaphysis)
extremities/pelvis
rarely in soft tissue
Clinical findings for ewing sarcoma
worsening localized pain +/- swelling
bone pain WORSE @ NIGHT
fatigue, fever, +/- weight loss
Dx for ewing sarcoma
x-ray
CT/MRI of primary lesion
Staging for ewing sarcoma
CT scan of chest
bone scan
bone marrow aspirates
biopsy
Dx of ewing
biopsy
Tx for ewing
chemo
surgery
radiation
combination
Prognosis of ewing sarcoma
70-75% long term survival if small localized tumor
90% of tumors diagnosed before age 5 are
retinoblastoma
Etiology of retinoblastoma
can be inherited (parent may be silent carrier)
usually unilateral
Prognosis of retinoblastoma
metastasis rare
death w/i a year is typical
Presentaiton of retinoblastoma
leukocoria (white pupillary reflex) <2 yo
strabisus, nystagmus and red inflamed eye possible
Dx of retinoblastoma
opthalmolgic exam under anesthesia - chalky, off white retinal mass w/ soft, fraible consistency
ocular U/S
MRI of brain and orbits
Tx of retinoblastoma
external beam irradiation
chemo if confined to globe
Most common soft tissue sarcoma in childhood
rhabdomyosarcoma
Epidemiology of rhabdomyosarcoma
most diagnosed by age 10
slight male predominance
can resemble fat, fibrous tissue and muscle
can effect any body part
Most common sites for rhabdomyosarcoma
head and neck
presentation of rhabdomyosarcoma
painless, progressively enlarging mass
Orbital rhabdo
proptosis/exopthalmos
Bladder rhabdo
hematuria
urinary obstruction
pelvic mass
Dx for rhabdomyosarcoma
imaging: xray, CT, MRI
Chest CT and skeletal survey to r/o metastasis
Tx for rhabdomyosarcoma
(any combo)
surgery
chemo
radiation
Prognosis of rhabdomyosarcoma
70-75% 3 year survival
Hepatic tumors stats
2/3 of liver masses are malignant
90% are either hepatoblastoma or hepatocellular carcinoma
Lab for hepatic tumors
alpha-fetoprotein (AFP) elevated
***AFP is a good marker for response to tx
Presentation of hepatic tumors
enlarging abdomen
Imaging of hepatic tumors
abdominal U/S, CT or MRI
Tx for hepatic tumors
surgery and chemo
complete resection essential for survival
liver transplantation when tumors are unresectable
Prognosis of hepatic tumors
1/3 w/ complete resection have long term survival