BRS Oncology Flashcards
1
Q
B and T cell dysfunction, atopic dermatitis, thrombocytopenia, assoc with leukemia and lymphoma
A
wiskott-aldrich syndrome
2
Q
X-linked lymphoproliferative disease is associated with ______ infection and may result in ______
A
EBV
lymphoma
3
Q
Burkitt’s lymphoma associated with _____ infection
Kaposi’s sarcoma associated with _____ infection
Hodgkin’s lymphoma
A
EBV
HIV
EBV
4
Q
_____ is the most common childhood cancer
A
ALL
5
Q
peak incidence and most likely demographic of ALL
A
2-6 years, male caucasian
6
Q
classification of ALL cell morphology
A
L1, L2, L3 with L1 being the most common in childhood (small with little cytoplasm)
7
Q
immunophenotypes of ALL
A
pre-B-cell: 70% –> 70% CALLA +, 30% CALLA -
T cell: 25%
B cell: < 5%
8
Q
clinical features of ALL
A
fever, bone/joint pain
pallor, bruising, HSM, LAD
9
Q
what does CBC show in ALL
A
anemia
thrombocytopenia
WBC is variable- leukemic blasts are often seen
10
Q
how to confirm dx of ALL
A
bone marrow biopsy demonstrates marrow replacement by lymphoblasts
-then do cytogenetics
11
Q
good prognostic factors in ALL
A
1-9 years of age
female
white
<50,000 WBCs
hyperploidy (more than 53 chromosomes in leukemic cells)
CALLA +
no organ involvement or chromosomal translocation
12
Q
3 stages of treatment for ALL
A
- induction with steroids, vincristine, L-asparaginase and also intrathecal methotrexate**
- consolidation with intrathecal methotrexate +/- cranial irradiation
- daily and periodic chemo for up to 3 years if no sxs
13
Q
when to do BMT for ALL
A
very high risk children
those who have relapsed
14
Q
some complications of ALL and the tx for ALL
A
- infection 2/2 neutropenia: staph, e coli, pseudomonas
- opportunistic infections: fungi, PCP
- tumor lysis syndrome: hyperuricemia (renal probs), hyperkalemia (arrhythmias), hyperphosphatemia (hypocalcemia and tetany)
15
Q
prognosis for ALL
A
generally good
85% long term survival
16
Q
AML is often associated with ______
A
Down syndrome
17
Q
classification of AML
A
M1-M7
M1, 2, 4, and 5 are most common
M3 (acute promyelocytic leukemia)- Auer rods
M7 (acute megakaryocytic leukemia)- associated with Down syndrome
18
Q
which is CNS involvement more common?
AML vs. ALL
A
AML
19
Q
clinical signs and sxs of AML
A
fever, HSM, bruising and bleeding, gingival hypertrophy, bone pain
LAD and testicular involvement are uncommon
20
Q
lab findings of AML
A
pancytopenia or leukocytosis and DIC
21
Q
how to dx AML
A
- blood smear shows leukemic myeloblasts esp with *Auer rods
- bone marrow biopsy for confirmation
22
Q
how to tx AML
A
AML (unlike ALL) requires intensive myeloablative therapy to induce remission –> then BMT is highly recommended
23
Q
prognosis of AML
chemo, BMT, assoc with Down syndrome
A
chemotherapy- 50% respond
BMT- 70% respond
if assoc with Down syndrome, very responsive to tx
24
Q
2 types of CML (least common leukemia in kids)
A
adult-type: older children, teens, Philadelphia chromosome, t(9,22) BCR/ABL1 juvenile CML (JMML): infants and kids < 2 years, no Philadelphia chromosome
25
presentation of adult-type CML
nonspecific sxs:fatigue, weight loss, pain
massive splenomegaly
extremely high WBCs > 100,000
26
presentation of JMML (juvenile CML)
```
fever
chronic eczema-like facial rash
suppurative LAD
petechiae and purpura
moderate luekocytosis WBC <100,000; anemia, thrombocytopenia
```
27
how to tx CML
BMT and gleevec (imatinib)
28
prognosis of CML
adult type- biphasic course
| JMML- often fatal; relapse in at least 50%
29
cancer of the antigen-processing cells found in the lymph nodes or spleen
Hodgkins lymphoma
30
Hodgkin's lymphoma most common presentation
slow and indolent
painless LAD in the supraclavicular/cervical regions
relatively common to see systemic symptoms
31
reed stern berg cell is seen in _____
Hodgkin's lymphoma
32
how to dx Hodgkin's lymphoma
lymph node bx where you see reed stern berg cell
33
staging of Hodgkin's lymphoma
stage I: single lymph node
stage II: on same side of diaphragm
stage III: both sides of diaphragm
stage IV: disseminated to one or more extra-lymphatic organs
34
how to tx hodgkin's lymphoma
| what are some complications of tx
chemo and XRT
- growth retardation
- secondary malignancies
- hypothyroidism
- male sterility (very common)
35
prognosis of Hodgkin's lymphoma
stage I and II: >80% long term survival
| 60-70% for more advanced disease
36
non-hodgkin's lymphoma is ______ (indolent/aggressive)
more male or female?
associated with _______ states
aggressive
male
immunodeficiency states (HIV, wiskott-aldrich, ataxia telengiectasia, prior EBV)
37
3 categories of non-Hodgkin's lymphoma
1. lymphoblastic lymphoma- T cell, histologically similar to ALL, anterior mediastinal mass, SVC syndrome or airway obstruction
2. small non cleaved cell lymphoma such as burkitt's- B cell, intussusception, jaw mass in burkitt's
3. large cell lymphoma- B cell, enlargement of lymphoid tissue in tonsils, adenoids, peyer's patches
38
most common lymphoma in childhood
burkitt's
39
most common presenting feature in non-Hodgkin's lymphoma
painless LAD
40
suspect _______ in any child > 3 years presenting with intussusception
lymphoma- esp small non cleaved cell lymphoma
41
how to dx non-Hodgkin's lymphoma
LN biopsy and imaging of various sorts for staging
42
how to tx non-Hodgkin's lymphoma
treatment must be rapid
- surgery
- chemo
- ppx for CNS disease
- tx tumor lysis syndrome
43
after leukemia, _____ are the second most common childhood cancer
they are also the most common solid tumors in kids
brain tumors
44
4 most common brain cancers by histology
1. glial cell tumors: astrocytomas
2. primitive neuroectodermal tumors (PNET): medulloblastomas in the cerebellum
3. ependymomas
4. craniopharyngiomas
45
in kids, _______ (infratentorial/supratentorial) tumors are more common
infratentorial
46
MC infratentorial tumor
| MC supratentorial tumor
medulloblastoma
| astrocytoma
47
brain tumor: diminished vision, visual field deficits, strabismus
optic glioma
48
brain tumor: growth retardation, delayed puberty, visual changes, DI, other hormonal problems
craniopharyngioma
49
how to dx brain tumors
MRI
| CSF analysis at the time of surgery
50
how to tx brain tumors
- surgery
- chemo
- try not to do XRT if under age 5 years
51
poorest prognosis with brain cancer
brainstem gliomas
not resectable
chemo doesn't work
52
malignant tumor of neural crest cells arising anywhere along the sympathetic ganglia chain and within the adrenal medulla
neuroblastoma
53
peak incidence of neuroblastoma
age 2 years
54
most common presentation of neuroblastoma
firm abdominal mass that crosses the midline
55
how to dx neuroblastoma
-definitive dx by bone marrow bx + elevated urine catecholamines (VMA, HVA)
OR
-tissue biopsy
also CT or MRI for assessing tumor spread and then bone scan/skeletal survey to assess for mets to the bone
56
staging of neuroblastoma
I: tumor confined to structure of origin
II: extends beyond structure of origin but doesn't cross midline
III: tumor goes past midline
IV: distant mets
IVS: localized tumor at stage I or II but distant mets to any organ but bone
57
how to tx neuroblastoma
surgery alone for stage I and II
chemo for metastatic disease and locally advanced dz
XRT for advanced disease
58
prognosis of neuroblastoma
```
good prognosis
-children < 1 year
-stage I and II disease
-spontaneous regression can happen in young infants with stage IVS disease
bad prognosis
-stage III and IV disease
-amplification of oncogene N-myc
-high levels of ferritin, LDH, and neuron-speicfic enolase
```
59
______ is a childhood tumor of the kidneys
Wilm's tumor (nephroblastoma)
60
peak incidence of wilm's tumor
age 3 ears
61
hemihypertrophy, macroglossia, visceromegaly
beckwith-wiedemann syndrome
62
wilms tumor, aniridia, GU abnormalities, MR
WAGR syndrome
63
most common presentation of wilm's tumor
abdominal mass (smooth, firm, rarely crosses midline)
abdominal pain
hematuria
HTN
64
how to dx wilm's tumor
CT or MRI
| bx and histology
65
how many stages of wilm's tumor
I-V
| V is bilateral renal involvement
66
how to tx wilm's tumor
prompt surgery
chemo
XRT for advanced dz
67
prognosis of wilm's tumor
excellent in most cases
68
most common soft tissue sarcoma in childhood
rhabdomyosarcoma- malignant tumor of the same embryonic mesenchyme that gives rise to skeletal muscle
69
common presentation rhabdomyosarcoma
painless soft tissue mass
70
most common areas involved in rhabdomyosarcoma
1. head and neck
- orbital tumors- proptosis, chemosis, eyelid swelling, CN palsies
- nasopharyngeal tumors- epistaxis, airway obstruction, chronic sinusitis
- laryngeal tumors- hoarseness
2. GU tract- hematuria, urinary tract obstruction, abd mass
3. extremities- painless growing mass
71
how to dx rhabdomyosarcoma
CT or MRI
| bx and histology
72
how to tx rhabdomyosarcoma
surgical resection
| +/- chemo and XRT
73
prognosis of rhabdomyosarcoma
| which sites have the best prognosis
head and neck and GU tumors have the best prognosis
74
most common malignant bone tumor
| peak incidence and gender
osteogenic sarcoma
| male teens
75
most likely location for osteogenic sarcoma
near the knee
| *metaphyses of tubular long bones
76
systemic sxs are _______ common/uncommon in osteogenic sarcoma
how about ewing's sarcoma
uncommon with osteogenic sarcoma
| common in ewing's sarcoma (fever, malaise, weight loss)
77
X-ray finding in osteogenic sarcoma and ewing's sarcoma
osteogenic- sunburst
| ewing's- onion skin
78
most common locations for ewing's sarcoma
flat bones and diaphysis of tubular bones; most commonly in axial skeleton (esp pelvis)
79
how to dx primary bone cancer (osteogenic and ewing's sarcoma)
MRI --> tissue biopsy --> bone scan, chest CT and other stuff to look for mets
80
how to tx osteogenic sarcoma
surgery for primary lesion and pulmonary mets
| chemo
81
prognosis of osteogenic sarcoma
survival > 60%
82
bone cancer with small, ground, blue cell tumor
ewing's sarcoma
83
second most common malignant bone tumor occurring in male adolescents
ewing's sarcoma
84
chromosomal translocation in ewing's sarcoma
t (11,21)
85
how to tx ewing's sarcoma
| prognosis
multiagent chemo followed by surgical excision
XRT if complete excision is not possible
-prognosis good for local dz, poor if mets are present
86
most common liver tumor in childhood
what age group
what is it assoc with
hepatoblastoma
< 3 years
beckwith-wiedemann syndrome
87
hepatocellular carcinoma in childhood is most likely 2/2 _____
chronic active hep B infection
| also biliary atresia, glucogen storage disease type I, alpha-1-antitrypsin deficiency, hereditary tyrosinemia
88
presentation of liver tumors in childhood
RUQ mass, loss of appetite and weight
| jaundice is usually absent
89
how to dx liver tumors in childhood
CT or MRI
| look for elevated alpha-fetoprotein in the serum
90
how to tx liver tumors in childhood
| what's the prognosis
chemo and surgical resection
| overall, prognosis is quite poor and often end up with mets
91
```
______ is the most common teratoma during first year of life
female vs. male
what's the presentation
benign or malignant
how to tx
```
```
sacrococcygeal
female predominant
soft tissue mass growing from coccyx
benign
tx with surgical excision
```
92
anterior mediastinal teratomas are generally _______ (benign/malignant) and can present with ______
benign
| airway obstruction
93
ovarian teratomas are the most common ovarian tumor and are generally _____ (benign/malignant)
you may see ______ on abdominal xray
benign
| calcium in the tumor
94
most common testicular tumors
yolk sac tumor > teratoma
95
peak ages of testicualr tumor
< 5 years and during teen years
96
in kids, chances of testicular tumor being benign
| what about adults
in kids, 1/3 are benign
| in adults, all are malignant
97
elevated alpha fetoprotein is seen in which germ cell tumor
yolk sac tumor
98
for ovarian tumors, 1/3 are malignant
| the younger the child is, the more likely that the tumor is ____ benign/malignant
malignant
99
eosinophilic granuloma
Hand-Schuller-Christian disease
Letterer-Siwe disease
all are part of Langerhans Cell Histiocytosis (LCH)
| -severe immune dysregulation disease that may be cancer-like
100
common clinical involvement with langerhans cell histiocytosis (LCH)
- skeletal involvement- skull, bony lesions, chronic draining ears indicates mastoid LCH
- skin involvement- seborrheic dermatitis of the diaper area and scalp
- pituitary or hypothalamic involvement
101
how to dx LCH
histologic features on skin or bone bx
102
how to tx LCH
| prognosis
if single lesion or organ --> local curettage, low dose XRT, steroids, or single agent chemo
if multiple lesions --> multi agent chemo
prognosis is variable based on extent of disease
103
Down syndrome puts you at risk for these cancers
ALL and AML
104
beckwith-wiedemann syndrome predisposes to these tumors
wilms, rhabdomyosarcoma, hepatoblastoma
105
neurofibromastosis type I predisposes to these tumors
brain tumors and lymphoma
