Oncology Flashcards
1
Q
incidence of cancer in children aged 0-14?
A
- 13,500 new cases/year in US: 1/7000 kids dx annually peak incidence: 1st year of life - 2500 cancer related deaths/year - #1 cause of disease related mortality (behind trauma, homicide, and suicide)
2
Q
Most common cancers 0-14 yos?
A
- leukemia: 32%
- CNS 20%
- lymphoma: 11%
- neuorblastoma 8%
- soft tissue sarcomas: 7%
- kidney (wilms): 6%
3
Q
Common cancers in 15-19 yos?
A
- lymphoma: 25%
- germ cell: 14%
- leukemia: 12%
- CNS: 10%
- soft tissue sarcoma: 8%
4
Q
Down syndrome is assoc with higher risk of getting what cancers?
A
- ALL (12x risk)
- AML (46x risk)
- AMKL (466x risk)
5
Q
Beckwith-Wiedeman assoc with higher risk of getting what cancers?
A
- Wilm’s (4-20%)
- hepatoblastoma
- rhabdomyosarcoma
- adrenocortical carcinoma
- neuorblastoma reported
6
Q
Li Fraumeni syndrome is assoc with higher risks of what cancers?
A
- bone tumor
- soft tissue sarcomaas
- breast cancer
- brain tumors
- adrenocortical carcinoma
7
Q
Brain tumors are assoc with what genetic syndromes?
A
- neuorfibromatosis
- tuberous sclerosis
- turcot syndrome
- von hipple lindau
- gorlin
8
Q
Bone tumors are assoc with what genetic syndromes?
A
- hereditary retinoblastoma
- paget’s disease
- Rothmund-Thompson
- Li-fraumeni syndrome
- Poikiloderma, small stature, skeletal dysplasia
9
Q
Kidney tumors are assoc with what genetic syndromes?
A
- congenital aniridia
- hemihypertrophy
- GU malformations
- WAGR
- Denys-Drash
- Frasier
- Beckwith-Wiedemann
10
Q
Liver tumors are assoc with what genetic syndromes?
A
- polyposis
- gardner
- beckwith-wiederman
- hemi-hypertrophy
- tyrosinemia
- glucogen storage dx
- alpha 1 anti-trypsin
- hemochromotosis
11
Q
Known environmental causes of cancer in kids?
A
- ionizing radiation: leukemia, brain tumors
- radiation therapy: bone tumors
- radium (high dose): bone tumors
- prior chemo: bone tumors, leukemia
- immunosuppression: lymphoma
- ## EBV infection: lymphoma (burkitts)
12
Q
If a pt comes in complaining about abdominal pain what should you be thinking about?
A
- constipation, cyst, bladder etiologies
(more common) - cancer
13
Q
Common causes of isolated cytopenia?
A
- infection, ITP, toxin
14
Q
Posisble cause of recurrent fever and bone pain?
A
- leukemia
15
Q
Possible cause of morning HA with vomiting?
A
- CNS tumor
16
Q
possible cause of neck mass not responding to abx?
A
- lymphoma
17
Q
Possible cause of chronic bone pain, swelling and limp?
A
- bone tumor
18
Q
Possible cause of progressive abdominal distension?
A
- abdominal tumor
19
Q
Possible cause of bleeding, bruising, pallor and fatigue?
A
- leukemia
20
Q
Possible cause of combined cytopenia?
A
- leukemia, marrow failure
21
Q
What are the presenting sxs of leukemia?
A
- sxs due to bone marrow replacement by dysfxnl immune cells (pain - expansion of so many WBCs, thrombocytopenia and anemia b/c of decrease in platelets and RBCs)
- bone pain, anemia, fatigue, fevers, and infection, petechiae and bleeding
- infection: because immune cells are dysfxnl
22
Q
Sxs of brain tumors in children, infants and the specific sites of tumor with correlated sxs?
A
- infant: large head, bulging fontanelle, anorexia, FTT, loss of milestones, irritable, shrill cry - child: HA (morning) vomiting decreased academics personality changes
- specific sites:
vision change
seizure (less than 99% not a tumor)
altered speech, handwriting or gait (clumsy)
23
Q
IICP findings and management?
A
- neuro-onc emergency: 10-18 mmHg: normal ICP 25-40: decreased blood flow and sxs greater than 40: unconsciousness - stroke, herniation, death - late findings: cushings triad: HTN, bradycardia, and wide pulse pressure, cheyne-stokes respirations
management:
- oxygenation: increased CO2 levels lead to dilation of cerebral vessels and more ICP
- steroids if edema present
- no prolonged hyperventilation: will lead to blood vessel constriction and decreased perfusion
- EVD: drain into lateral ventricle
- surgical decompression
24
Q
Sxs of lymphoma?
A
- localized: painless enlarged lymph nodes abdominal pain or mass emergent: compromise of vital structure - systemic: fevers, night sweats, wt loss, fatigue, pruritus, pain with ETOH ingestion
25
Q
Mediastinal mass Complications? Management?
A
- anterior mediastinal mass: compress trachea, pleural effusion - this can lead to tamponade
- SVC or superior mediastinal syndromes are medical EMERGENCIES
- proceed with extreme caution:
small tumors can be sx, all depends on locations, sedation can cause fatal resp failure - anesthesia can aggravate SVC/airway compression, difficult to intubate past obstruction - obtain tissue by least invasive means possible
- emergency management may include steroids or radiation: bx may be uninterpretable in as little as 48 hrs
26
Q
Signs and sxs of abdominal tumors?
A
- distension, palpable mass (clothes won’t fit)
- N/V/D, constipation, wt loss, fever
- obstruction, pain, jaundice, blood in urine, renal failure
27
Q
Signs and sxs of bone tumors?
A
- pain/tenderness/limp
- soft tissue mass
- night pain
- “growing pains”
- hx of minor trauma
- pathologic fracture
- pain often present for 2-3 months
28
Q
Diff types of leukemia?
A
ALL:
- precursor B cell (B-ALL)most common in young kids
- Burkitts (mature B-ALL, tx like lymphoma)
- T cell (T ALL)
AML:
- FAB (old) and WHO classifications:
FAB: based on morphology/phenotype. M0-M7, marrow with 30% blasts or more
WHO: clinical/molecular dx, marrow with 20% blasts or more, prior therapy, down syndrome, MDS, cytogenics
29
Q
How common is leukemia in peds?
A
- leukemia 32% of childhood cancers among those less than 15 yrs old
- 13% of cancers among those 15-19.99
- 0-5 yrs: ALL 81%, AML 14%, CML 2%, others 3%
- 15-19: ALL 51%, AML 36%, CML 9%, other 4%
- leukemia causes 30% of cancer deaths among those less than 20
30
Q
Predisposing conditions lead to higher risk of leukemia?
A
- cancer predisposition syndromes:
- Li Fraumeni (SBLA): Tp53 oncogene and other genes
- ataxia telangectasia (T ALL, T NHL)
- Neurofibromatosis type 1: AML more common than ALL
- bloom syndrome (leuk, lymphoma, AML more than ALL)
-congenital anomaly syndromes:
klinefelters
- congenital and acquired immunodeficiences:
ataxia telangectasia, Wiskott aldrich syndome, chronic immunosuppresison, AIDs
31
Q
Down sydrome and leukemia?
A
- 10-20x increased risk of leukemia
- relative risk of AML higher but ALL more common during first year of life
- risk period limited to first 3 decades
- increased risk of toxic death
- DS-ALL, DS-AML
32
Q
ALL prevalence?
A
- 80-85% cases are B-precursor
- less than 2% cases are mature B-ALL (burkitt): assoc with t(8;14)
- 15% cases are T ALL
- incidence peaks at 2-4 yrs in developing countries
- incidence in whites almost 2x higher than blacks
- little known about causes
33
Q
Presentation of leukemia?
A
- bone pain!!! (35%): due to expansion of marrow cavity, young children may just be irritable, stop walking, favor painful site if localized
- adenopathy (diffuse)
- hepatosplenomegaly
- thymic expansion: mediastinal mass esp T ALL
- CNS sxs: HA, neck pain, CN palsy, seizures
- testicular involvement: painless, enlarged, usually rock hard testes, must confirm with bx
34
Q
Heme findings and assoc clinical findings in leukemia?
A
- WBC can be high, normal or low
- decreased neutrophil + and fxn: infection
- risk of tumor lysis syndrome with high WBC (more than 50)
- hyperleukocytosis (WBC greater than 250-500K) may be assoc with CNS and pulmonary sx but much lower risk with ALL compared to AML
- decreased platelets: bruising, bleeding, and petechiae
- decreaed RBC: pallor and fatigue
35
Q
DDx of pancytopenia finding?
A
- leukemia
- aplastic anemai (acquired, congenital, viral)
- metastatic solid tumor
- myelodysplatic syndrome
- hemophagocytic lymphohistocytosis
- infection, post infectious (EBV especially)
- SLE
- hypersplenisim
- B12 or folate deficiency
36
Q
DDx of hepatosplenomegaly and pancytopenia?
A
- leukemia: AML and ALL
- hepatitis assoc aplastic anemia
- metastatic solid tumor
- MDS
- HLH
- SLE
37
Q
What can ALL mimic? Stongest factors predictve of ALL?
A
- ALL can mimic juvenile RA
- strongest factors: low WBC (less than 4000), low-normal platelet count (150-250) and night time pain. Rash and ANA not helpful
38
Q
ITP findings?
A
- most ITP kids are very healthy and have acute onset of brusing
- ITP typically nml Hgb and WBC and platelets less than 50K, no circulating blasts
39
Q
DDx for acute lymphocytosis?
A
- infection (esp pertussis, EBV, CMV)
- ALL
- thyrotoxicosis
- neutropenias (relative lymphocytosis)
- Addison’s disease (relative lymphocytosis)
40
Q
DDx of lymphadenopathy?
A
- infection
- metastatic disease
- leukemia
- lymphoma
- autoimmune (JRA, SLE)
41
Q
Labs for dx leukemia?
A
- CBC with diff, peripheral smear (see lymphoblasts, all monoclonal WBCs, larger than usual)
- bone marrow aspirate
- LP
- flow cytometry, morphology, and cytogenetics
- sanctuary sites (CNS and testes): LP, testicular exam
42
Q
Tx for standard risk ALL?
A
- 4 week 3 drug induction
- 4 week oral consolidation with IT
- 8 wk Int maintenance
- 8 wk delayed intensification
- 2nd int maint
- maintenance (2 + years)
43
Q
Tx for HR ALL and T ALL?
A
- 4 wk 4 drug induction
- 8 wk IV consolidation
- 8 wk Int maint
- 8 wk delayed intens
- 2nd interim maint
- maintenance (2+ years)
44
Q
Tx adjustment if poor response to therapy?
A
- alters tx regimen
- CNS 3 disease typically receives cranial irradiation, many T ALL also receive regardless of CNS status. Intrathecal chemo has been key to improving outcomes
45
Q
Relapse site and time importance?
A
- site of relapse impt:
bone marrow worse than extra medullary (resistant) - time to relapse: earlier much worse than later, worse if less than 18 months since dx
- age at initial dx: very poor outcomes for teens who then relapse
- genetic features: marrow relapse of T ALL has very poor outcome, subset of low/st risk ALL does relatively well
46
Q
How common is AML? Prognosis compared to ALL?
A
- 5% of childhood leukemia (0-14)
- peaks in infancy and teen years
- classification based on old system (FAB) and WHO definitions
- tx related (t-AML) diff than de nova AML
- cytogenic lesions impt
- worse prognosis than ALL, 5 y EFS 60-70%
47
Q
Presentation of AML?
A
- fever (33%)
- bone pain
- LAD
- HSM
- extramedullary disease: leukemia cutis (rash), gingival hypertrophy, and CNS disease (infants, , M4, M5, high WBC)
- chloromas
- sepsis
- hpyerleukocytosis
- anemia
- thrombocytopenia
- hemorrhage: epistaxis, menorrhagia, either due to thrombocytopenia or DIC
- DIC: assoc with acute promyelocytic leukemia
48
Q
What is key feature of AML that you will see on peripheral smear?
A
- Auer rod in AML blast cell
49
Q
Risk groups of AML?
A
- based on cytogenetics
- down syndrome less than 4, tx with less intense chemo
- APML tx with All trans retinoic acid (ATRA) and arsenic trioxide
- favorable four courses of intense chemo
- unfavorable to bone marrow transplant if acceptable donor found after 2 rounds of chemo
50
Q
Tx of AML?
A
- remission induction: 2 course intense chemo, very high risk of invasive infection
- post remission consolidation: transplant for high risk, or 2 courses of intense chemo
- CNS prophylaxis: much less intense than ALL due to low rate of CNS relapse. If CNS positive weekly IT chemo until clears
- maintenance: survival benefit never demonstrated so no main course
- no irradiation even with CNS disease: emergent if life threatening chloroma (spinal cord compression)
51
Q
Different types of lymphomas?
A
Hodgkin lymphoma:
- classic HL: nodular sclerosing, mixed cellularity, lymphocyte predominant, lymphocyte depleted
- nodular lymphocyte predominant
non-hodgkin lymphoma:
- Burkitt, lymphoblastic, diffuse large B cell, anaplastic large cell, many others
52
Q
Epidemiology of lymphoma?
A
- 11% of childhood cancers (0-14)
- 25% b/t 15-19
- NHL more common in young male white children
- overall good prognosis
53
Q
Presentation of Hodgkin lymphoma?
A
- painless lymphadenopathy, mediastinal mass, and or constitutional sxs
- B sxs: (prognostic) wt loss (greater than 10% in 6 months), drenching night sweats, unexplained fevers of greater than 38 C for 3 consecutive days
- other sxs (not prognostic): fatigue, anorexia, mild wt loss, pain following ETOH ingestion, and pruritis
54
Q
Lab findings in hodgkin lymphoma?
A
- elevated inflammatory markers: CRP, ESR, ferritin, copper, anemia of chronic inflammation and possible immune dysregulation (autoimmune neutropenia, autoimmune hemolytic anemia, immune thrombocytopenia, or nephrotic syndrome)
55
Q
DDx for hodgkins?
A
non-malignant:
- normal thymus
- infection: EBV, atypical mycobacterium, histoplasmosis, toxoplasmosis
- lymphoproliferative disorder
- progressive transformation of germinal centers
malignant: NHL, germ cell tumor, soft tissue sarcoma, metastasis
56
Q
Eval of pt with suspected HL?
A
- H&P: key to ask about B sxs and eval respiratory/CV status
- CBC, CMP, inflammatory markers
- CXR
- CT neck and chest
- CT or MRI of abdomen and pelvis
- FDG-PET
- if higher stage will need bilateral bone marrow bx and aspirate to eval for metastatic disease
57
Q
Risk stratifiction for HL?
A
- based on staging, presence or absence of B sxs and bulk disease
- bulk disease is more than 1/3 thoracic diameter on PA CXR or greter than 6 cm nonmediastinal mass
- bx of tissue is Key!!
- malignant cells: Hodgkin Reed sternberg cells, lymphocytic and histiocytic cells
- immunostaining of bx very impt, phenotype can determine histology
- EBV implicated in some HL
58
Q
Tx for HL?
A
- multimodal: multi-agent chemo and radiation std, survival over 90% for advanced disease
- risk adapted: disease severity determines intensity, used to decrease, increase or omit modalities
- new tials designed to minimize late effects: decrease doses of chemo, decrease radiation fields or omit in some pts
59
Q
NHL incidence?
A
- more common in young (under 10) white male children
- 800 cases/yr in US
- risk factors: EBV (for some), immunodeficieny, immunosuppression, pesticides
- tx depends on exact dx
- B sxs not prognostic
60
Q
Emergent presentations of NHL?
A
- these are frequent
- compression of airway due to superior mediastinal syndrome
- pulmonary effusions
- CV: SVC syndrome, tamponade, arrhythmia
- paraspinal/epidural mass complications
- electrolyte abnormalities due to lumor lysis
- organ failure: renal compromise due to compression 2/2 mass
- GI obstruction, intussusception, bleeding, perforation, jaundice, and pancreatitis
- cytopenia due to marrow infiltration
61
Q
Diff NHL presentations?
A
- endemic burkitt: jaw swelling, abdominal or orbital swelling, paraspinal mass, CNS or marrow involvement
- sporadic burkitt: rapidly expanding abd mass, spontaneous tumor lysis, non specific GI sx, CNS involvement, marrow infiltration
- DLBCL: more diverse (LN, liver, spleen, marrow mediastinum, retroperitoneum)
- lymphoblastic: mediastinal mass, pleural/pericardial effusion, pain, dysphagia, dyspnea
- ALCL: slowly progressive, systemic sx, organs and skin can be involved
62
Q
Tx of NHL?
A
- depends on type and staging
- all receive CNS ppx
- lymphoblastic lymphoma tx like ALL, long therapy with maintenance
- most don’t receive iradiation (exception: CNS + lymphoblastic lymphoma)
63
Q
Prognosis of NHL?
A
- overally survival is high
- Burkitt 90%
- DLBCL: 90%
- ALCL: 85%
- burkitt leukemia: 80%
64
Q
How common is neuroblastoma in kids?
A
- 10% of childhood cancers
- second most common solid neoplasm in childhood
- median age: 22 months (not common over 5)
- originates from neural crest tissue
- can met to bone, bone marrow, lymph nodes
65
Q
Genetics and neuroblastomas (NBL)?
A
- familial neuroblastoma assoc with an anaplastic lymphoma kinase (ALK) germline mutation
- Noonan syndrome
- congenital cardiac defects
- constitutional deletion of chr 1p
- neurofibromatosis
- neurocrsitopathy syndrome
66
Q
Presentation of NBL?
A
- coincidental finding (30%)
- periorbital echymosis (racoon eyes), proptosis
- neck mass
- GU sx
- neuro abnorm
- paraneoplastic syndromes: opsoclonus/myoclonus (4%), secretory diarrhea due to vasoactive intestinal peptide
- ill appearing: malaise, irritable, wt loss, anorexia
- pain: ill defined (40%)
- abdominal distension (35%)
- fever, resp distress
67
Q
Dx NBL?
A
- CT/MRI scan (spine): eval primary and nodal met sites
- bilateral bone marrow bx and aspirate
- radiolabeled MIBG: if non avid then do PET
- catecholamine metabolites: vanillymandelic acid (VMA) and homovanillic acid (HVA) 90%
- tumor bx: histologic grading, tumor genetic analysis
68
Q
Prognostic criteria for NBL?
A
- independent variables
- all poor prognostic characteristics
- age over 18 months
- advanced stage dz
- Myc-N amplification
- poorly or undiff tumor histology
- diploid DNA content
69
Q
What is a Wilm’s tumor? Incidence?
A
- nephroblastoma
- most common renal tumor in kids (95%)
- 4th most common childhood cancer
- 8/1mill annual incidence
- 50% are less than 3, median age is 43 month girls and 37 months boys
- slight female predominance
- 5-10% have bilateral tumors
70
Q
Assoc malformations and genetic predisposition related to Wilm’s tumors?
A
- aniridia
- hemi-hypertrophy
- GU malformation
- malformation of any type
- children with assoc malformations or bilateral disease are dx earlier
- genetic predisposition:
WAGR (wilms, aniridia, GU malf, mental retardation)
Beckwith-Wiederman, Dneys-Drash syndreom (nephropathy, male pseudohemaphroditism, 90% develop wilms), perlman syndrome, sotos syndrome, simpson-golabi-behmel
71
Q
Presetnation of Wilm’s tumor?
A
- usually presents as asx upper abdominal mass
- abdominal pain (20-30%)
- fever
- anemia (bleed into tumor - BAD!)
- hematuria (tumor extension into renal pelvis)
- HTN - distortion of renal vasculature
- hx esp family hx and congenital defects
- physical: BP and congenital anomalies
- lab: CBC, UA, BUN, Cr, T bili, alk phos, albumin, SGPT
- encapsulated tumor - claw sign
72
Q
Dx of wilm’s tumor?
A
- resection if able: if bx considered and tumor spills - upstaged to at least stage III
- CT chest
- Abd US (eval for vascular invasion)
- CT abdomen
- further imaging if tissue not consistent with wilms
73
Q
DDx of wilm’s tumor?
A
- clear cell sarcoma of kidney
- rhabdoid tumor
- renal cell carcinoma
- congenital mesoblastic nephroma
74
Q
Pathology of wilm’s tumor?
A
- favorable histology: 92%, 5-10% will have bilateral disease, 10% multifocal loci in solitary kidney, overall survival is 95% stage 1-III
- anaplasia: 8%, focal or diffuse, marker of increased resistance to chemo, overall survival 20-80% depending on stage
75
Q
Staging of wilm’s tumor?
A
- stage 1: confined to kindey
- stage II: confined to renal fossa
- stage III: gross residual disease - unresectable primary, lymph node mets, positive surgical margins, tumor spill
- stage IV disseminated
- stage V: bilateral tumors
76
Q
Prognostic factors of wilm’s tumors?
A
- stage: stage II or greater do worse
- histology: diffuse anaplasia unfavorable
- gene expression profile: LOH 1p or 16q
- tumor size: wt of over 550 g worse
- age: older than 2 worse
77
Q
Tx of wilms tumors?
A
- surgical resection if possible
- multiagent chemo
- radiation for higher stages, pulmonary mets
- higher stages get more intense therapy
78
Q
Incidence of retinoblastoma?
A
- 300 new cases per yr in US
- 3% of all ped cancers
- 63% less than 2
- 95% less than 5
- 2 clinical forms: non-hereditary or heriditary (more likely to be BL)
79
Q
Clinical presentation of retinoblastoma?
A
- intraocular disease: leukocoria (65-75%), strabismus, nystagmus
- advance intraocular disease: buphthalmos, glaucoma, periorbital cellulitis
- extraocular disease: proptosis + LN, metastases
80
Q
Dx retinoblastoma?
A
- exam under anesthesia: direct visualization of tumors, eval of corneal diameters and IOP
- Ocular US
- MRI orbits and brain: eval of optic nerve prior to enucleation, eval of CNS (trilateral disease)
- bone scan, bone marrow and CSF for advanced disease
- genetic counseling: all patients, heritable:25-30%, and non-heritable: 70-75%
- family hx, multifocal dz, or identified RB1 germline mutation
81
Q
DDx of retinoblastoma?
A
- congenital cataracts
- coat’s disease (inflammatory process)
- persistent fetal vasculature
- retinitis
- medulloepithelioma (tumor of ciliary pigment epithelium(
- astrocytic hamartoma (TS or NF1)
- choroidal osteoma
82
Q
Staging and tx of retinoblastomas?
A
- multiple staging systems: size of tumor and extension are key pts
- tx is very individualized, determined by staging, grouping and laterality of disease
- goals of tx: cure - eye salvage - vision preservation
83
Q
Prognostic factors of retinoblastoma?
A
- high risk histology
- massive choroidal replacement
- ciliary body or iris involvement
- optic nerve extension beyond lamin cibrosa
- Extra-CNS disease
- CNS disease has dismal prognosis
- survival overall good if no CNS disease
84
Q
Incidence of brain tumors in peds?
A
- 1/50000 kids
- 3500 new dx in US annually
- 2nd most common ped malignancy
- presentation differs in peds than adults: seizure is common adult presentation - not so in peds
- overall survival: 65% with variability, less than 20% survival for brainstem gliomas, greater than 80% survival for pilocytic astrocytomas
85
Q
Types of childhood CNS tumors?
A
- ependymoma
- high grade glioma
- germ cell tumor
- medulloblastoma
- pilocytic astrocytoma
86
Q
Genetic asociations with CNS tumors?
A
- neuorfibromatosis 1: optic pathway gliomas, nonneoplastic hamartomas
- tuberous sclerosis: subependymal giant cell astrocytoma
- retinoblastoma: risk for trilateral disease, region of pineal gland (pineoblastoma)
87
Q
What is a craniopharyngioma? Tx?
A
- epithelial tumor
- arises from Rathke cleft: can occur anywhere along course of the duct, pharynx to sella turcica and 3rd ventricle
- adamantinomatous type: cystic appearance with solid component and Ca deposits
- 5 yr survival: 80%
- tx: surgery and radiation
88
Q
Presentation of cranipharyngioma?
A
- most common presentation is growth failure and HA in peds
- interference with pituitary fxn: hypothyroid, obesity, diabetes insidious
- interfere with visual fxn: amblyopia, temporal heminaopsia, blindness, optic atrophy
- mass effect: HA, N/V, papilledema
89
Q
What is a medulloblastoma? Presentation?
A
- most common malignant peds tumor: males greater than females, 40% occur prior to age 5
- 1/3 present with CSF mets
- presenation: HA, N/V., papilledema is most common finding
- staging: low vs high risk
- survival is greater than 50%, low risk: 80%
90
Q
What is an ependymoma? Presentation, survival?
A
- 10% of ped brain tumors
- males = females
- glial tumors: arise in ependymal cells, 4th ventricle and spinal cord, 50-80% have calcifications
- common presentation:: IICP, HA, ataxia, nystagmus, seizure, increased head circumference
- overall survival: 50%, with full resection - 70%, extent of resection is strongest predictor of outcome, radiation is useful, role of chemo unclear
91
Q
What is a pilocytic astrocytoma?
A
- 50% of ped CNS tumors
- Can occur throughout CNS
- increased incidence 5-14
- increased incidence assoc with NF-1
- with GTR: 10% year survival is greater than 90%
with subtotal prognosis still 50%
92
Q
How common are spinal cord tumors? sxs and signs?
A
- relatively rare in peds
- heterogenous group: neurodevelopmental, astrocytomas, or neuroblastoma
- intramedullary, intradural, or extradural.
astrocytomas, ependymomas, neurofibromas,
extradural: neuroblastoma, bone tumors other mets - signs/sxs: chronic back or neck pain most common, scoliosis assoc with 1/3 of spine tumors, progressive weakness: decreasing motor skills, numbness, paralaysis
Eval:
PE - strength, sensation, reflexes, and MRI
93
Q
Ped cancers that have greater than 90% 5 yr survival rates? Other cancers with lower survival rates
A
- ALL
- Wilms
- hodgkins
- retinoblastoma
- NHL: 80%
- bone tumors: 68%
- CNS tumors: 65%
- AML: 50%
94
Q
Why must survivors of cancer be screened for late effects of therapy down the road?
A
- children receive more chemo and surgery than adults do
- more vulnerable to effects on growth, fertility, heart and neuropsychological fxn
95
Q
Delayed and chonic toxicities effects on the body?
A
- chemo: secondary AML
- anthracyclines: myocardial dysfxn
- corticosteroids: osteonecrosis
- nephrectomy: HTN
- radiation: breast/thyroid neoplasms
- 75% of childhood cancer survivors will develop disabilities that alter quality of life
