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Pediatrics > Oncology > Flashcards

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)
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2
Q

Most common cancers 0-14 yos?

A
  • leukemia: 32%
  • CNS 20%
  • lymphoma: 11%
  • neuorblastoma 8%
  • soft tissue sarcomas: 7%
  • kidney (wilms): 6%
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3
Q

Common cancers in 15-19 yos?

A
  • lymphoma: 25%
  • germ cell: 14%
  • leukemia: 12%
  • CNS: 10%
  • soft tissue sarcoma: 8%
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4
Q

Down syndrome is assoc with higher risk of getting what cancers?

A
  • ALL (12x risk)
  • AML (46x risk)
  • AMKL (466x risk)
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5
Q

Beckwith-Wiedeman assoc with higher risk of getting what cancers?

A
  • Wilm’s (4-20%)
  • hepatoblastoma
  • rhabdomyosarcoma
  • adrenocortical carcinoma
  • neuorblastoma reported
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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
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7
Q

Brain tumors are assoc with what genetic syndromes?

A
  • neuorfibromatosis
  • tuberous sclerosis
  • turcot syndrome
  • von hipple lindau
  • gorlin
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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
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9
Q

Kidney tumors are assoc with what genetic syndromes?

A
  • congenital aniridia
  • hemihypertrophy
  • GU malformations
  • WAGR
  • Denys-Drash
  • Frasier
  • Beckwith-Wiedemann
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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
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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)
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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
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13
Q

Common causes of isolated cytopenia?

A
  • infection, ITP, toxin
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14
Q

Posisble cause of recurrent fever and bone pain?

A
  • leukemia
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15
Q

Possible cause of morning HA with vomiting?

A
  • CNS tumor
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16
Q

possible cause of neck mass not responding to abx?

A
  • lymphoma
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17
Q

Possible cause of chronic bone pain, swelling and limp?

A
  • bone tumor
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18
Q

Possible cause of progressive abdominal distension?

A
  • abdominal tumor
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19
Q

Possible cause of bleeding, bruising, pallor and fatigue?

A
  • leukemia
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20
Q

Possible cause of combined cytopenia?

A
  • leukemia, marrow failure
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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
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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)
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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

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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
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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
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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
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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
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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

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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
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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
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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
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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
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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
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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
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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
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36
Q

DDx of hepatosplenomegaly and pancytopenia?

A
  • leukemia: AML and ALL
  • hepatitis assoc aplastic anemia
  • metastatic solid tumor
  • MDS
  • HLH
  • SLE
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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
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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
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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

Pediatrics (20 decks)

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