BRS- Onc Flashcards
Leading cause of death from disease in childhood?
Most common childhood cancer?
Most common solid childhood cancer?
Cancer= leading cause of death from disease
Childhood cancer = ALL
Solid childhood cancer= brain tumors
Two immunodeficiencies that predispose kiddos to cancer
- Wiskott Aldrich: lymphomas & leukemias
- X linked lymphoproliferative disease: EBV –> lymphoma
EBV assc cancer?
HIV assc cancer?
EBV –> Burkitts
HIV –> Kaposci’s
Downs syndrome assc cancers
ALL, AML
Syndrome assc with GI cancers
Blooms syndrome
NF II assc cancer
acoustic neuroma
Two abdominal mass types
Wilms Tumor
Neuroblastoma (crosses midline)
Soft mass on extremities
rhabdomyosarcoma
Early morning H/A + vomiting cause
brain mass
Tumor causing leukocoria
Retinoblastoma
Tumors causing HTN
Neuroblastoma
Wilms tumor
Pheochromocytoma
Peak incidence/ prototype patient with ALL
-white male age 2-6 years
Two classification types for ALL
- cell morphology
- immunophenotype (+/- CALA)
Describe cell morphology types + which is most common in ALL?
L1: small with little cytoplasm
L2: between
L3: large lymphoblasts
*L1 more common
Describe immunophenotypes in ALL + which is most common?
T/B/PreB cell +/- CALLA
Most common Pre B cell + CALLA
What does CALA stand for?
common acute lymphocytic leukemia antigen
Most common ALL symptoms?
fever and bone pain
CBC Findings in ALL
low platelets anemia variable WBC count **CBC does not rule out leukemia **Must confirm with marrow evaluation
Staging for ALL
no staging system
Favorable age, sex, race for ALL prognosis
- 2-9
- female
- Caucasian
Favorable immunophenotype/genetic makeup for ALL:
+CALLA
-t(9,22)
Three stages of ALL management
- induction
- consolidation
- maintenance
What drug is given to all ALL patients in the induction stage of treatment?
intrathecal MTX
What is the consolidation phase of ALL treatment and what is its purpose?
How long is maintenance phase of ALL tx?
intrathecal MTX
cranial irradiation, high risk 5+
prevents CNS involvement
-maintenance up to 3 years
Lab findings assc with tumor lysis syndrome
-^^uric acid, ^^potassium, ^^phospohate (=low Ca)
Three sequelae of tumor lysis syndrome
- tetany
- renal disease
- cardiac disease
Overall ALL survival rate
85%
Four syndromes assc with AML
- Fanconi
- Downs
- Kostmann
- Neurofibromatosis
What are the 7 types of AML
M1: acute myeloblastic, immature M2: acute myeloblastic, mature M3: acute promyelocytic M4: acute myelomonocytic M5: acute monocytic M6: erythroleukemia M7: acute megakaryotic
What are the 4 more common types of AML
M1,2,4,5
Which type of AML is assc with Auer Rods?
assc with Down Syndrome?
Acute promyelocytic (M3)= auer rods Acute megakaryocytic (M7) = Downs
Organ systemic more commonly involved in AML than ALL? Common oral finding in AML?
CNS involvement + gingival hyperplasia common in AML
Which type of AML is most successfully cured?
AML assc with Downs= most responsive
50% cured with chemo; 70% cured with marrow transplant (in general cases)
Least common type of leukemia in kiddos is _____.
Two types are ____ & ______.
CML
Adult type
Juvenile CML
Chromosomal abnormality assc with adult type and juvenile type CML?
adult type: 9,22 BCR ABL
juvenile type: chromosome 7,8 involvement
Age groups assc with adult type and juvenile type CML?
Which is more fatal
adult: older kiddos/teens
juvenile: younger than two, worst prognosis (often fatal, half relapse)
WBC count and sign suggestive of adult CML? Juvenile?
Adult: massive splenomegaly; extremely high WBC
Juvenile: facial rash, moderate leukocytosis
Hodgkins vs Non-Hodgkins lymphoma:
- which is more common?
- which grows more rapidly/is more aggressive?
- which is commonly assc with systemic symptoms?
- common: non
- rapid: non
- systemic: hodgkins
Emergent sequelae assc with nonhodgkins lymphoma:
- SVC syndrome
- airway compression
Describe the staging assc with Hogdkins lymphoma
Ann Arbor System: I: single node II: two+ nodes, same side of diaphragm III: nodes on both sides of diaphragm IV: diffuse
A: lack of symptoms
B: symptoms present
What predisposes kiddos to Hodgkins Lymphoma?
EBV mononucleosis
Cell assc with Hodgkins Lymphoma?
Binucleated reed Sternberg cell
Complications of Hodgkins Lymphoma therapy:
1) hypothyroidism
2) secondary malignancies
3) male sterility
4) growth retardation
Non-Hodgkins Lymphoma:
- age group/sex
- predisposing factor
-5+
-male
immunodeficiency predisposes
Three major categories of Non-Hodgkins lymphoms
1) lymphoblastic lymphoma (T cell)
2) small non-cleaved (B cell)
3) large cell (B cell)
Of the three non-hodgkins lymphomas, what type includes burkitts?
small non cleaved B cell
Most common presenting feature of non-hodgkins lymphoma
painless lymphadenopathy
Common presentation lymphoblastic lymphoma
anterior mediastinal mass w/ airway or SVC obstruction
Endemic Burkitts:
- location in the world
- location in the body
- Africa
- Jaw
Intussusception in a kiddo older than 3 requires workup for?
lymphoma (especially small cell)
Common location of large
- tonsils
- adenoids
- peyers patch
Three diseases assc with brain tumors
- VHL
- TS
- NF
Most and second most common peds brain tumors
-glial #1
-PNETs #2
ependymomas & craniopharygiomas also common
High grade brain tumor common location?
Low grade?
High= supratentorial Low= infratentorial
Most common PNET tumor?
-medulloblastoma in the cerebellum
MC infratentorial tumor? supra?
supra= astrocytoma infra= medulloblastoma
Ataxia is assc with what brain tumors?
Seizures?
ataxia= infratentorial (cerebellum) seizures= supratentorial
Principal treatment of brain tumors?
-resection or debulking
Peds brain tumor with worst prognosis
brainstem glioma
Neuroblastoma:
- most common location
- most common age
- diagnosis
- 75% in abdomen or pelvis, can be anywhere on the sympathetic ganglia chain
- first 5 years of life
- marrow biopsy + urine HVA/VMA
Neuro complication assc with 2% of neuroblastomas
-acute cerebellar atrophy
“dancing feet and eyes”
=ataxia, opsoclonus, myoclonus
Staging of neuroblastoma:
- I: localized
- II: spread but not crossing midline
- III: crosses midline
- IV: crosses midline + mets
- IVS: stage I-II + mets
What type of neuroblastoma may spontaneously regress?
-Stage IVs in a baby
What marker is significant for poor prognosis in neuroblastoma?
n-myc
Most common childhood renal tumor
Wilms, usually kiddos under 5
Describe Beckwith Wiedemann syndrome
- hemihypertrophy
- macroglossia
- visceromegaly
Describe WAGR syndrome + assc chromosome
chromosome 11
- wilms tumor
- aniridia
- genitourinary abnormalities
- retardation
Two symptoms assc with Wilms tumor
Hematuria
HTN (pressure on renal artery)
Staging Wilms tumor
- I: limited to kidney
- II: local spread, completely excised
- III: residual tumor after resection
- IV: distant mets
- V: bilateral renal involvement
Prognosis Wilms Timor:
90% survival
Condition predisposing to Rhabdo
neurofibromatosis
Most common sites for rhabdo
head and neck including the orbit
Most common and second most common bone tumors in kiddos? Prognosis of each?
- osteosarcoma (#1), 60% survive
- ewings sarcoma, 80% survive
Osteosarcoma:
- peak incidence
- common location
- radiographic findings
- presentation
- adolescent males during growth spurt
- metaphysis of long bones, esp common at knee
- sunburst pattern
- poor systemic involvement
Ewings Sarcoma:
- most common location
- symptoms
- radiographic findings
- diaphysis of long bones
- fever, weight loss, leukocytosis, ^ESR
- onion skin pattern
Ewings Sarcoma chromosomal abnormalities
t(11,21)
Most common type of liver tumor?
Most common age group?
Assc syndrome?
hepatoblastoma
under 3
Beckwith Wiedemann syndrome
Hepatocellular carcinoma:
most common assc disease
chronic hepatitis B
Prognosis for hepatoblastoma/ hepatocellular carcinoma
-both= poor, hepatocellular carcinoma worst
Six germ cell tumors seen in kiddos
- teratoma**
- seminoma (Male)
- dysgerminoma (Female)
- Yolk Sac
- Embryonal
- Choriocarcinoma
Most common germ cell tumor seen in kiddos
sacrococcygeal teratoma, 95% benign
Elevation of what protein is assc with yolk sac tumors?
AFP
How commonly are ovarian tumors malignant?
1/3rd younger the child= more malignant
Most common site for Langerhans Cell Histiocytosis
skull
painful bony lesions +/- pathologic fractures
Skin finding in LCH
mimics cradle cap
