Questions volume 2 (oncology, few questions in previous volume) Flashcards
1
Q
Describe which way each of the following labs goes in tumour lysis syndrome (up/down) uric acid potassium phosphate calcium
A
uric acid increases ->hyperuricemia
potassium increases ->hyperkalemia
phosphate increases ->hyperphosphatemia
secondary hypocalcemia
2
Q
What is the mechanism of renal failure in tumour lysis syndrome
A
secondary to uric acid and calcium phosphate crystals in the microvasculature of the kidneys
this can worsen hyperkalemia, lead to fatal arrythmias
3
Q
All but which of the following is a method to prevent tumour lysis syndrome
a) allopurinol
b) aggressive hydration (2x maintenance)
c) alkalization f the urine for pH>7.5
d) monitoring of electrolytes
A
c) false - do want to do alkalination to increase the excretion of uric acid, want to monitor the urine pH with target of 7.0-7.5
if 7.5 can lead to calcium phosphate crystal formation
treatment: when abnormalities of metabolics occurs, treat ASAP, if renal failure occurs, need to do dialysis
4
Q
Which patients are most at risk of complications from leukocytosis?
a) Burkitt’s lymphoma
b) ALL
c) AML
A
c) patients most at risk of complications from hyperleuocytosis are those with AML
those with ALL and other hematologic malignancies typically do not experience complications unless WBC is greater than 200000
5
Q
Please list the complications of hyperleukocytosis
A
Definition: WBC >100000
3 organ systems primarily affected:
1. pulmonary: pulmonary leukostasis and secondary pulmonary hemorrhage
2. CNS: leukostasis in cerebral vasculature, hemorrhagic stroke (more common) ischemic stroke (rare)
3. renal: leukostasis in microvasculature ->renal failure
4. other: leukostasis in small vessels->can lead to priapism, dactylics, GI bleed, pericardial tamponade
4. increased risk of TLS
6
Q
Which type of stroke is more common secondary to hyperleukocytosis?
a) hemorrhagic
b) ischemic stroke
A
a) hemorrhagic stroke is more common, ischemic stroke (rare) –>seems counterintuitive
from leukostasis in cerebral vasculature
7
Q
A patient with AML arrives and has a WBC count of 125. What are your management strategies
A
Prevent TLS: aggressive hydration, alkalinzation, allopurinol
Prevent hemorrhage ->keep platelets >20
Avoid RBC transfusions ->they can increase viscosity, if needed, consider exchange
If symptoms of leukostasis are present -consider leukapheresis to quickly decrease WBC (temporizing measures)->definitive treatment ->start chemo
8
Q
True or false - a patient with cancer is complaining of back pain. The neurological exam is normal. Can you rule out cord compression clinically?
A
No->absence of neurologic symptoms does not rule out cord compression, MRI is the radiographic study of choice
spine X rays are abnormal in <50% of patients with spinal cord compression, but can be a good first step while organizing MRIw (should give dex first if cord syfunction says Oski, if cord compression suspected but no neuro symptoms, lower dex dose and MRI in 24 hours)
progressive neuro symptoms will happen with undiagnosed cord compression
9
Q
A patient is found to have a mediastinal mass. Please name 7 common causes
A
- Hodgkin disease
- NOn-Hodgkin lymphoma
- Germ cell tumour
- Neuroblastoma
- Teratoma
- Acute lymphoblastic leukaemia (most commonly T cell, this is why we should always do a CXR in patients with leukaemia)
- Sarcoma
10
Q
What are two interventions that are contraindicated in medastinal mass patients?
A
- sedation and anxiolytics
2. place the patient upright
11
Q
Name two results of anterior mediastinal masses
A
- compression of the trachea
- compression/obstruction of the SVC ->results in SVC syndrome (characterized by
**Oski says steroids or chemo may be necessary to prevent mortablity/morbidity despite lessening the chance of tissue diagnosis
12
Q
name 3 risk factors for ALL
A
- Down syndrome
- Ataxia telangiectasia
- prior exposure to radiation
however in most cases, no predisposing condition exists
13
Q
Which is the most common childhood cancer
A
ALL - 1/3 of all childhood cancer
approximately 80-85% of all childhood acute leukaemia is ALL
5000 cases in US each year
arisses in lymphoid cell lineage - can be either B cell or T cell derived
14
Q
When is the peak age of diagnosis with ALL
A
age 2-3 (baby nelson says 2-5 year old)
15
Q
How can you distinguish between infectious mono and ALL?
A
both can present with HSM, lymphadenopathy, and mild cytopenias
on peripheral smear ->atypical lymphocytes (in mono) vs. leukemic blasts (ALL)** although can be hard to distinguish ; and sometimes in automated readings will say atypical on the initial reading
in general-do P/E, blood smear and bone marrow to diagnose
16
Q
What is the difference between aplastic anemia and cancer?
A
aplastic anemia should not have a increased blast count
other malignancies can metastasize to the BM
17
Q
What are some other childhood illnesses than can be on the differential for ALL?
A
- JIA - can present with fever, pallor, joint pain, HSM->do BM before steroids
- ITP common in childhood ->ONLY platelets should be affected
- mono (se above)
- aplastic anemia
- other malignancies with mets to the BM - Neuroblastoma, sarcomas, retinoblastoma
18
Q
All but which of the following is a sign/symptom associated with ALL?
a) testicular atrophy
b) gingival hypertrophy
c) leukemia cutis
d) renal insufficiency
A
a) in fact testicular enlargement is associated
gingival hypertrophy and leukoemia cutis - most common in infants
other manifestations: BM cytopenias, pain secondary to marrow crowding, HSM, Anterior mediastinal mass, increased LN
19
Q
Where do chloromas most commonly occur?
A
in the orbits -results in proptosis, these are tumour of leukaemia cells
20
Q
Why are boys treated 1 year longer than girls for ALL?
A
to minimize risk of testicular relapse
21
Q
Which patients with ALL get intrathecal chemo?
A
all of them, to treat or prevent CNS involvement
22
Q
What is the event free survival in ALL
A
85-90%
23
Q
Which patients are deemed high risk ALL?
A
Baby Nelson:
for ALL - 4 prognostic groups based on age, initial WBC count, genetic characteristics and response o induction therapy
Low Risk Patients:
- Age 1-9 year old
- Initial WBC 10 year old
- initial WBC >50 at diagnosis
- CNS or testicular disease at diagnosis
- unfavorable cytogenetics - ie T4;11
Very High Risk Patients:
- hypodiploid DNA index
- t 9;22 translocation
- fail remission after 4 weeks of therapy
Everybody else is standard risk!!
Other factors which can influence: immunophenotype, minimal residual disease, and early response to therapy
24
Q
Which percentage of childhood leukaemia is AML?
A
15-20% , equal in male and female
AML is high in neonatal period then drops and stabilizes into teens, then stable into adulthood
25
True or false - most people with AML have a predisposing condition
false - most don't, however ionizing radiation, benzenes and prior chemo increase the risk of AML
26
Please name 5 risk factors for AML
1. Down syndrome
2. Li-Fraumeni sydnrome
3. Neurofibromatosis type I
4. Klinefelter syndrome
5. BM failure syndromes (Fanconi, Shwachman-Diamond, ataxia telangiectasia)
characterized based on morphology according to the FAB classification
presentation of AML ->essentially same as ALL
subtypes are described in chart
27
What is the long term survival of children with AML
40-60% with exception of M3 AML and patients with Down syndrome, who do a lot better
28
Which genetic translocation is associated with chronic myeloid leukaemia?
associated with philadelphia chromosome -resuls in a fusion protein (bcr/abl)
chronic meloid leukemia - <5% of all childhood leukaemia
hyperpropliferation of mostly mature BM cells
presentation: fever, night sweats, fatigue, HSM and splenomegaly
can become myeloproliferative disease or blast phase
treatment - historically chemo and stem cell, new med - Imatinib
29
What are the lab findings in CML
1. leukocytosis with left shirt
2. increased eosinophils and basophils
3. anemia
4. thrombocytosis
30
What is the first line therapy for CML ?
a) chemo
b) Gleevac (imatinib)
c) stem cell transplant
b) Gleevac - small molecule inhibitor of bcr/able
31
True or false - Hodkin's lymphoma presents with painful lymphadenopathy
false - presents with painless lymphadenopathy , can also present with mediastinal mass
Differential includes other causes of lymphadenopathy:
mono, atypical mycobacterium, toxo, non Hodkin lymphoma, metastatic adenopathy
32
What diagnosis is suggested by the presence of Reed-Sternberg cells
Hodgkin lymphoma - classic
| the other type of Hodgkin lymphoma is nodular lymphocyte predominant
33
What is the treatment of Hodgkin lymphoma
surgery, chemo and radiation
prognosis -
locatlized - EFS 90%
high stage - 60-80%
34
All but which of the following suggests a worse prognosis for Hodgkin lymphoma?
a) high WBC count
b) bulky mediastinal disease
c) low hemoglobin
d) female gender
d) in fact male gender is a risk factor for worse outcome
the others also suggest a worse outcome (this is from once)
```
baby nelson pg 546 - staging of Hodkin lymphoma:
low
intermiate
high risk
based on stage and nodal bulk
```
35
Which type of lymphoma is more common in children younger than 10 years old
a) Non-Hodgkin lymphoma
b) Hodgkin lymphoma
Non-Hodkin more common in <10 year old
| Hodgkin more common in 15-19 year old
36
What are the three types of non-Hodgkin lymphoma?
1. Burkitt's/B large cell (B lymphocyte)
2. lymphoblastic (T lymphocyte)
3. Large cell (B lymphocyte/anaplastic)
37
Which non-Hodkin lymphoma most likely to present with intussusseption
Burkitt's/B-large cell->can present with rapidly grown abdo mass, massive TLS,
endemia Burkitt's occurs in regions in Afriac, characterized by involvement of the maxilla, orbit and other facial bones
38
Which non-Hodkin Lymphoma does not commonly have CNS involvement?
large cell
(Burkitt's and lymphoblastic do have CNS invovlement)
prognosis - low stage 90% EFS, high stage 60-70% EFS
39
Which non-Hodkin lymphoma commonly presents with effusions?
lymphoglastic (see the chart)
40
What is the genetic aberration most common in non Hodgkin lymphoma
t(8,14) ->fusion of cMyc with gene for immunoglobulin chain, occurs in most Burkitt's lymphoma
41
A child from Africa arrives with significant lymphadenopathy as well as lymphoma in maxilla, orbit and other facial bones - what virus is involved with the disease
this is endemic Burkitt's lymphoma
caused by EBV, perhaps in the setting of chronic malaria
the other type of Burkitt's is non-endemic, with primarily abdominal involvement
42
Who are two groups that are at increased risk of non Hodkin lymphoma from EBV?
1. underlying congenital immune deficiency
| 2. post-solid organ transplant
43
Which malignancy is most likely for patient's with HIV?
non Hodgkin lymphoma, primarily Burkitt's
44
Which is the most common solid tumour in children?
brain tumours
they are the second most common cancer in childhood after acute leukaemia
account for the most paediatric cancer deaths
the #2 solid tumor is neuroblastoma (after CNS tumours)
45
Where are most paediatric brain tumours located?
infratentorial - 70% (vs adult are mainly supratentorial)
46
A patient has paralysis of the upward gaze without paralysis of convergence. What is their diagnosis
Parinaud syndrome
| associated with midbrain tumours
47
What is the survival of medulloblastoma
medulloblastoma - most common paediatric CNS malignancy - survival of approximately 80% when localized
meanwhile- brain stem gliomas - dismal prognosis, 10% at 2 years
treatment: medulloblastoma and germ cell tumours are sensitive to chemo, gliomas are less so
48
What are two effects of cranial radiotherapy:
1. endocrine dysfunction with radiation to hypothalamus - i.e. growth failure, thyroid deficiency, Gn deficiency
2. neurocognitive deficits - especially if the patient was <8 years old at time of treatment
* *this is why we try very hard to avoid high dose CNS radiation in very young kids
49
What disease has Homer-Wright peudorosettes
neuroblastoma, classic histological finding (but acutely infrequent), small round blue cell tumour
NB arise from neural crest cells that were destined to differentiate into sympathetic neurone
50
What is the most common and prognosticaly significant genetic abnormality in neuroblastoma
amplification of the N-myc gene on chromosome 2p24
51
What is the median age of diagnosis of neuroblastoma
median age at diagnosis is 22 months, 90% female
52
What are the paraneoplastic syndromes associated with neuroblastoma
1. opsoclonus-myoclonus
2. secretory diarrhea
overall NB presentationd epends on the site of tumour
some to remember - abdo can include lower extremity/scrotal edema, resp sx with liver infiltrate, hypertension rom renal infiltration
thoracic/cervical - horner
orbital bone - ptosis and periorbital ecchymosis
blueberry -muffin nodules of tumour cells
symptoms from increased catecholamines (regardless of the site of the tumour)**
metastasis for neuroblastoma: to liver, bone and bone marrow and lymph nodes are common
53
What is the most common site of neuroblastoma at diagnosis?
a) paraspinal
b) adrenal medulla
c) thoracic
d) cervical
b) in the abdomen - adrenal medulla is the most common primary site (45% of abdominal), 25% are retroperitoneal sympathetic ganglia
2nd is paraspinal regions (30%) - can invade and cause spinal cord compression
3rd are thoracic primary tumours (20%)
rarely - pelvic or cervical area
**neck or apical masses can cause a Horner syndrome
54
What percentage of patients with neuroblastoma have opsoclonus myoclonus ?
2-3% of patients with neuroblastoma
paraneoplastic syndrome - symptoms include myoclonic jerks of limbs and trunk, random eye movements, ataxia
outcome is better in terms of NB, but high risk of long term cognitive, motor, language deficits and behavioural problems
Treamtnet: steroids, IVIG, gabapentin with variable success
55
Why is MRI better than CT for brain tumour diagnosis?
initially do CT
MRI better because - visualization of posterior fossa is better
will get a better look enough that you don't need to do a biopsy
should also do MRI of spine and CSF studies (since risk of drop metastasis)
56
What tests should be done for evaluation of neuroblastoma
Imaging:
CT/MRI of the primary tumour
MIBG scintiscan
-analogue of catecholamineprecursors and concentrates in NB cells, localizes to tumour cells in 90-95% of patients
- can damage the thyroid - therefore give iodine prior to MIBG
Biopsy or resection of the primary tumour - will do light microscopy, electron microscopy and immunohistology
bone marrow, biopsies from at least two sites
Labs: CBC, LDH, ferritin,
urine HVA and VMA (will be positive in 90% with neuroblastoma)
57
What is the treatment for low stage disease neuroblastoma ?
low stage - surgery alone - (as long as biology is favourable)
many young patients with localized disease have spontaneously resolution - however if it is identified, need to resect it
for higher risk - surgery, chemo, radiation, stem cell transplant, resection AFTER multiple rounds of chemo, can do radiation, high dose chemo with stem cell rescue has improved the outcome for patients with high-risk neuroblastoma
retinoid acid - has also improved survival
chemo drugs used: vincristine, cyclophosphamide, doxorubicin, cisplatin, etoposide
Side effects: ototoxicity nephrotoxicity, growth problems and second malignancies
58
All but which of the following is a characteristic of stage IV-S disease in neuroblastoma?
a) localized tumour
b) mets limited to liver, skin or bone marrow
c) no N-Myc amplification or unfavourable histology
d) age >1 year old
d) in fact age t be stage IVS
3 groups:
low risk:
average risk:
high risk:
oler patients with stage 4 more commonly have unfavourable biology, more than 50% will relapse due to drug-resistant residual disease
59
What is the prognosis of neuroblastoma with N-Myc amplification?
Not good- for neuroblastoma with N-Myc and/or unfavourable histology, Event free survival - 30-50%
whereas overal EFS of >95% even if ipsilateral LN
stage IV-S even though metastatic, has very good prognosis (EFS >95%)
unfavorable biologic factors: lack of cell differentiation, MYC-N amplification, lack of hyperdiploidy, mutations of chromosome 1p, 11q or 6p22
Favorable biology- usually the patients are younger, often have localized disease
60
Which race is most likely to get Wilms' Tmour
a) black
b) white
c) asian
a) blacks>white>asian
61
At what age does unilateral Wilms' Tumor present?
a) 3.5 year old
b) 2.5 year old
c) 1.5 year old
d) none of the above
a) unilateral tumour: mean age at presentation is 3.5 years for boys, 4 years for girls (remember it is OLDER than for neuroblastoma)
no clear gender predilection
bilateral presents wearier - 2.5 years for both sexes
presentation - parent notices abdominal mass/fullness while changing/bathing the child
abdo pain
gross hematuria
25% will have hypertension at time of diagnosis
62
Please name the congenital syndromes associated with Wilms' tumour
1. WAGR : Wilms, Aniridia, Genitourinary malformations, Retardation of mentation and growth
2. Denys-Drash syndrome: pseudohermaphroditism, degenerative renal disease (nephrotic syndrome or Wilms tumour)
3. Beckwith Wiedemann : overgrown syndrome, visceromegaly, macroglossia, hyperinsulinemic hypoglycemia, predisposition to embryonal tumours
**need to do periodic screening with periodic renal imaging
4. Sporadic hemihyperplasia: in 25% of patients with WT
Other:
- cryotorchidism
- hypospadias
63
In males with Wilms' tumour, which is more common, cryptorchidism or hypospadias?
cryptorchidism in 46.6% of males with WT
| vs hypospadias in 20%
64
Why should coags be done with bloodworm upon diagnosis of Wilms' tumour?
to look for acquired von Willebrand disease, occurs in 8% patients at diagnosis
other labs to do: renal function, liver function, calcium, CBC, urinalysis, urine catecholamines ( ddx from NB)
Imaging: US, CT, CXR ; need to make sure about IVC since timor can extend there from the kidney
do imaging of chest abdo, pelvis
histology for diagnosis
Treamtnet: surgery (for all), if can't resect, do a biopsy
prognosis not bad - low -stage, good histology EFS 90%, mets and unfavourable historloy - 66%
65
which bone cancer is more likely to occur in the diaphyseal region?
Ewing sarcoma - in diaphyseal region of long bones (also common in pelvis, chest wall)
vs. osteosarcoma - metaphyseal region
66
What is the most common site of mets in Ewing sarcoma?
lungs -
1/3 of patients will present with metastasis
median age of presentation 15
most commonly present with pain (look for red flags) >1 month, mass, no trauma, night pain
overall prognosis is poor-
non metastatisc- 2/3 cure
mets - 1/3 survive
67
Which is the most common malignant bone neoplasm in kids?
osteosarcoma
| Ewing is second
68
Which part of the long bones bone is involved in Ewing sarcoma?
diaphyses (shaft): most common sites are femur and pelvis as per Baby nelson, will present with local pain and swelling, may also have systemic symptoms
osteosarcoma is metaphysis or epiphysis (at anatomical sites associated with maximum growth velocity, i.e. distal femur, proximal tibia, proximal humerus) but any bone can be involved
69
Which bone cancer has a typical "sunburst"appearance?
osteosarcoma
when presents, often thought to be related to trauma
70
Where does osteosarcoma commonly metastasis?
lungs most commonly, rarely to other bones
in teens - when there is the maximum growth velocity
hx of bilateral Rb (hereditary neuroblastoma) increases the risk of developing osteosarcoma
previous radiation or chemo falso increases the risk of osteosarcoma as a second malignancy
presentation is pain and may have a palpable mass, Xray will show a lytic lesion
most patients might have micrometastatic disease
71
What is the genetic change found in 85% of Ewing sarcoma?
translocation of chromosome 11 and 22 (seen in 95% of tumours)
MRI of the primary lesion should be performed to delineate the extent of the lesion and any associated soft tissue mass
evaluate for mets: bone scan, chest CT, bone marrow
72
Which of the following benign bone tumours is found incidentally (can pick multiple)
a) osteroid osteoma
b) fibroma
c) unicameral bone cyst
d) aneurysmal bone cyst
incidental are unicameral bone cyst and fibroma (when done for other regions)
osteoid osteoma - presents with bone pain, worse at night, better with NSAIDS
Aneurysmal bone cyst - pain and swelling
73
Groups with increased risk of acute leukaemia
1. Down syndrome
2. Fanconi anemia
3. Bloom syndrome
4. Ataxi Telangiectasia
5. Wiskott-Aldrich syndrome
6. Neurofibromatosis I
7. sibling of kids with leukaemia - 2-4x increased risk
increases for twins (up to 25% in monozygotic twins)
8. environmental factors: ionizing radian, exposure to certain chemotherapy agents
74
most common cancers in kids
leukemia/lymphoma/CNS tumors
75
Signs and symptoms of acute leukemias
1. infiltration of leukaemia cells into normal tissues, results in:
a) bone marrow failure - anemia, neutropenia, thrombocytopenia
b) specific tissue infiltration (lymph nodes, liver, spleen, brain, bone, skin, gingiva, testes)
common presentations: fever, pallor, petechiae or ecchymoses, lethargy, malaise anorexia and bone/joint pain
P/E: increased LN and HSM
testes and CNS are common extra medullary sites for ALL
76
what is the WBC count in acute leukaemia
can be low, normal or high
77
which chromosomal abnormality in ALL most common
t12:21 most common - good prognosis
78
ALL chromosomal abnormality with poor prognosis
79:22, t4:11
79
what is transient myeloproliferative disorder
newborns with T21 - elevated WBC counts with peripheral blasts, anemia and thrombocytopenia
usually resolves with supportive care, can have significant increased risk of developing true leukaemia within the next few months/years of life
80
Phases of treatment for chemo for ALL
1. induction - 3 or 4 agent, based on initial risk
2. consolidation and CNS directed therapy
3. continuation therapy (intermittent vincristine, steroids, 6MP, methotrexate)
81
Phases of treatment for chemo of AML
very different, non myelosuppressive drugs not effective, need lots of cycles of very intense myelosuppressive chemotherapy
if there is an HLA matched sibling donor, usually do stem cell transplant in the first remission (except if T21 or factorable cytogenetics)
82
Complications of chemotherapy
Shot term complications:
- bone marrow suppression - bleeding, neutropenia increase infections Iincluding PCP, therefore septra prophylaxis), consider fungal prophylaxis
Long term seqeualae:
neurocognitive impairment, shot stature, obesity, cardiac dysfunction, infertility (from alkylating agents, i.e. cyclophosphamide) , second malignant neoplasm, psychosoail)
83
What is the current cure rate for childhood ALL
ALL is 80%
84
What is the current cure rate for childhood AML
AML is 50%
| better prognosis if get matched sibling stem cell transplant rather than just chemo
85
Where does ALL most commonly relapse
bone marrow
brain
testes
worst if relapse during treatment
if have relapse should have a stem cell transplant from matched sibling donor
86
Late adverse effects of treatment for lymphoma
```
secondary malignancies - including AML or myelodysplasia, thyroid malignancies, breast cancer
hypothyroidism
impaired soft tissue and bone growth
cardiac dysfunction
pulmonary fibrosis
```
87
What are some syndromes which increase the risk of CNS timor
**most CNS tumours happen in kids with no known underlying disorder or risk factor
the following syndromes can increase the risk of CNS timor:
1. neurofibromatosis type 1 and 2
2. Li-Fraumeni
3. Tuberous Sclerosis
4. Turcot syndrome
5. von Hippel-Lindau
88
Cranial nerve deficits in CNS tumors
sixth nerve involvement - common sign of increased ICP
| other CN involvement suggests brainstem involvement
89
How commonly might you see seizures in supratentorial tumours
20-50% (baby nelson)
90
CNS timor with increased ICP, one immediate treatment
dexamethasone - will help reduce the immediate tumor-associated edema
91
What is cerebellar mutism syndrome
occurs in 25% of kids after resection of a posterior ofssa timor
1. acute decrease in speech (mutism)
2. behaviural changes - irritability, apathy or both
3. diffuse cerebellar dysfunction
4. other neurologic abnormalities
can start within hours to days of surgery
usually resolves within weeks to months
can get cerebellar ataxia and dysmetria 9which can persist)
92
What is post CNS tumor somnolence syndrome
excessive fatigue and sleepiness
may occur in the months after competion of radiation therapy
self limited
93
What is posterio fossa syndrome
headache and aseptic meningitis days to weeks after surgery in posterior fossa
94
5 year survival of all CNS tumours
50-60%
very good for astrocytoma cerebellar and medulloblastoma , very bad for glioma and gliomblastoma multiform have very bad prognosis
95
neuroblastoma, usually inherited or not?
not usually inherited
not usually inherited (only rarely 1-2% as per baby nelson) but in these cases there can be a family history of neuroblastoma
the mutations in ALK and PHOX2B associated with most familial cases
96
What is the treatment of Wilms Tumor
in North america - immediate nephrectomy with adjuvant chemo
in Europe - chemo first to shrink the tumour then surgery
chemo
radiation for minority of patient
97
long term effects of treatment for wilma timor
from drugs: cardiomyopathy, scoliosis, hypertension, pre-hypertension, renal/bladder insufficiency, pulmonary dysfunction, hepatic dysfunction, infertility, second malignancies
bilateral - might end up with renal insufficiency o failure
98
Prognostic factors for Wilms tumor
stage
histologic features - anapaestic have worse prognosis
mutations at 1p or 16p worse prognosis
survival for localized disease - 85% with pulmonary mets 70-80%
99
What is the most common soft tissue sarcoma in children
rhabdomyosarcoma - derived from mesencymal cells committed to skeletal muscle lineage
less common soft tissue sarcomas: fibrosarcoma, synovial sarcoma, extra osseous Ewing sarcoma
there are two classes of sarcomas - soft tissue and bone cancer
100
What are two conditions that increase the chance of soft tissue sarcomas?
Li-Frameni syndrome
| Neurofibromatosis
101
you do light microscopy, and you see small round, blue cell timor, what type of timor is it
Ewing sarcoma or rhabdomyosarcoma will look like this
| two major histology variants for rhabdomyosarcoma - embryonal and alveolar
102
What is the presentation of rhabdomyosarcoma
peaks in kids 2-6 year old: GU region, head and neck
teenagers : extremities, trunk, male genitourinary tract
boyx 1.5 x more than girls
clinical presentation: depends on the site and mass effects
can get mets to bone or bone marrow
103
evaluation for osteosarcoma
should do imaging of chest, abdo pelvis, bone scan, and bone marrow and biopsy (to check f infiltration)
104
Treatment for rhabdomyosarcoma
based on staging
chemo
radiation
initial surgery
105
treatment of osteosarcoma
```
chemo first (to shrink the tump) follow by surgery and then chemo
radiation is NOT great for osteosarcoma
```
106
treatment of Ewing Sarcoma
pre op chemo, surgery, then more chemo
radiation is also good for Ewing
**remember that radiation can have significant effect on growth and development particularly in young kids
107
Prognosis for sarcoma
most important factor is the presence of metastatic disease at diagnosis (lung mets lower the cure rate significantly, as does mets to other bones)
in osteosarcoma and Ewing - the degree of timor necrosis after prep chemo is also important (i.e. how much you can get rid of it)
