Pediatric Oncology Flashcards
Leukemias
- types
lymphomas
- types
quick review of the blood cell lines
Blood Cell Lines
Myeloid Stem Cell becomes
- RBCs
- platlets
- monocytes
- granulocytes (neutrophils, eosinophilis, basophils)
Lymphoic Stem Cell
- bcomes a lymphoblast
- then becomes T cell OR B cell
Leukemia: cancers of the myleoid and lymphoid line
AML and ALL more common in kids
CML (philly chromosomes) and CLL (smuged cells) more common in adults
Lymphomas
- non-hodkins
- hodgkins (reed steernburg cells)
- from T, B lymphocytes and in lymph
Acute Lymphoblasci Leukemia (ALL)
- etiology
- symptoms/signs
Etiology: ALL
- cancer affecting the immature lymphcytes (T and B cells: T-ALL or B-ALL) = creating lymphoblasts the immature version of the T and B cells
- most common pediatric cancer
- commonly affected those age 2-5 years; 85% survival
Symptoms/Signs
- easy brusing (since low platelets)
- fatigue
- recurrent infections (immunocomp.)
- easily bleeding, nose bleed
- bone pain and pallor
ALL
Diagnosis and Labs
Risk Classes by type of ALL
DIagnosis ALL
CBC and smear:
- will see increased WBC and low platelets (thrombocytopenia)
- on smear: can see blasts (marrow pusing out immature cells into the blood because the cancer is ruining the others)
Unilateral bone marrow biopsy and aspirate
- see >20% of blasts
LP and need to IT chemo
- LP to asses if CNS disease: if there is IT chemo will be used
Flow Cytometery
- to differentiate between myleoid and lymphoid cells
Risk Classes
85% of ALL is B-cell : which can be high or low risk
HR: those 10+ years old OR those with WBC > 50,000
LR: those 1-10 years old AND WBC < 50,000
“infantile ALL” those < 1 year
MRD: minimal disease residual plays a role too in HR or LW
ALL
Treatment Stragety
ALL Treatment APproach
2-3 years of treatment
induction thearpy: chemo for 28 days
- goal here = induce remission with chemo off the bat
consolidation thearpy: chemo
- goal here = treat any CNS infection (even if we dont see it)
interim maintenence
Delayed intensification
- when they are the most prone to infection: immune system so wiped
maitnenence
- using 6-MP
CNS + pt : get LPs to monitor disease
- IT methotrexate used as IT chemo
relpae = chemo and stem cell transplant
Acute Myleoid Leukemia (AML)
etiology
symptoms
Etiology: AML
- less common than ALL, poorer prognosis
- usually effects those 65+ but can still effect kids
- AML: affects myleoid cells: diagnosis made by the presence of myleoblasts
- subtype: APML
symptoms
- fatigue
- brusing
- recurrent infection
- swollen gingiva
- easy bleed/brusing!!
- bone pain
- pallor
AML
diagnostic labs
Diagnosis
CBC and smear
- CBC = increased WBC, low platlets
- smear: sometimes will show blasts
unilateral bone marrow biopsy and aspirate
- will find > 20% blasts = diagnositic
- will find aurer rods!!!
LP and IT chemo
- assess CNS disease and treat via IT (IT chemo of choice is cytarbine)
Flow cytometery
- differentiate between myleoid and lymphoid cells
AML
Treatment
Treament
- shorter, more intesne than ALL
- lasts 6-9 months ; depends on mutations and specific aspects of the CA
those with intermediate risk = 5 cycles of chemo
those with high risk = chemo + stemm cell transplant
APML specific = ATRA and ARAC (trans-retinoic acid and cytarbine)
relapse =
- those without a history of STC will get donor transplant
- those with a histry of transplant: get a second one
Hodgkin’s Lymphoma
etiology
symptoms
Etiology
- lymphoma type
- can be associated with EBV
- bimodal age distribution: late teens and then older adults
- most common subtype of HL = nodular sclerosing
symptoms
- the constitutional B symptoms: classic hallmar: fever, night sweats, weight loss
- often a medistinal mass and concurrent respiratory symptoms + itching
- lymph node enlargement
Hodgkin’s Lymphoma
diagnosis
treatment
Diagnosis
- lymph node biopsy reveas: Reed Sternburg cells
- bone marrow aspirate: look for involvement here
- CBC and CMP/mag/phos
- chest CT/MRI and PET = assess for METS
Treatment
- a combination of chemo and radiation
- immunotherapy used
- stagec accoding to above and below the diaphragam
- stage 1: 1 spot above
- stage 2: 2 + spots (lymph nodes) above
- stage 3: involvement above nad below diaphgram
- stage 4: on organs too
Non-Hodgkin’s Lymphoma
etiology
subtypes
symptoms
Etiology
- multiple subtypes
- MC subtype: Diffused large B-cell: see a mediastinal mass growing rapidly ; be aware of quick decompensattion
other subtypes
- burkitt: tumor lysis syndrome, rapid growth
- lymphoblastic (T and B-cell involved)
- follicaulr: slow
Symptom s
- B sx. so common : WL, fatigue, night sweats,
- more common in those who are immunosuppressed
- lymph node enlargement
Non-Hodgkin’s Lymphoma
Diagnosis
Treatment
Diagnosis
- CBC, CMP, mag, phosphate, tumor lysis labs = phos, uric acid, calcium, K, LDH
- Lymph node biopsy
- bone marrow aspiration and biopsy (posterior illiac crest)
- chest ct/MIR and PET for METS
Treatment
- chemo (belomycine)
- steroids
- immunotherapy (rituximab)
3 types of neuro-onc tumors
medulloblastoma
atypical rhabdoi tumor (ATRT)
Diffuse midline glioma
Medulloblastoma
etiology
symptoms
diagnosis
Medulloblastoma
- Most common CNS malignancy in kids
- occurs: at cerevellum in posterior fossa
symptoms
- headache: especially in the AM and not getting better with meds
- vomiting
- fatigue
- cerebellar dysfunction: ataxia
- neurological changes
Diagnosis
- stable pt = MRI - no increasd ICP = LP = surgery
- unstable pt = surgery 1st (to remove mass) - MRI and LP (10-14 days post -op once swelling has decreased)
Medulloblastoma
treatment
prognosis
Complications; to know!!!
Treatment
- surgery
- chemothearpy
- radiation: only if child is > 3 years old (under 3- stunted growth)
Prognosis
- depends on lots of things
- SHH and WNT : > 75% prognoss
- others: less
Complication = posterior fossa syndrome
after surgical resection of the tumor due to location
- emotional liability, mutism , atxia
- intensive rehab helps to decresed severility of sx.
Atypical Teratroi Rhabdoid Tumor (ATRT)
etiology
symptoms
diagnosis
treatment
Etiology
- rare: 18 month olds
- occur in posterior fossa or supertentorial areas
- survival = 17 months
Symptoms
- increased ICP (HA, vomiting, vision changes)
- motor changes
- ataxia
Diagnosis
- MRI
- biopsy via surgery
- LP
- genetic testing
Treatment
- surgical resection
- chemo and auto SCT
- IT chemo if CNS disease
- radiation if over 3
Diffuse Midline Glioma (DMG)
etiology
symptoms
diagnosis
treatment
DMG: etiology
- inucrable cancer due to location
- involves pons and brainstem: thus cannot operate as they might injury basic functioning
Symptoms
- difficulty swallowing
- facial drop, slurred speech
- balance issues
- HA, nausea, vomiting
- CN6 palsy
Treatment
- radiation: to control
- no cure
Retinoblastoma
etiology
symptomssigns
diagnosis
treatment
Etiology
- cancer of the retina
- average age at dx. 18 months
- relatively common
Symptoms
- leukocoria (white pupil) or red
- stabismus
- mydriasis
- vision changes
- eye pain
Diagnosis
- optho exam
- MRI of brain and orbit
- CBC.CMP and genetcs
Treatment
- cryotherapy, thermo and plaque thearpy
- chemo: intraarterial chemo + radiation
Complications which can arise from the CNS tumors
Increased ICP + hydrocephalus
- some kids may need VP shunts
Endocrine Issues
- Diabetes insipidus
- adrenal insufficency
- hypothyroidism
usually due to tumor location near hypothalmus/pituitary
Wilm’s Tumor (Nephroblastoma)
etiology
symptoms
diagnosis
treatment
Etiology
- most commony abd. cancer in kids
- associated with prematurity and low birth weight
- beckwith-widemann syndrome
- nephroblastoma; of the kidney
symptoms
- bloating/abd distenstion and pan
- constipation
- fevers and fatigue
- hematuria, hypertension or hypotension
- WILL NOT CROSS MIDLINE
Diagnosis
- cbc, cmp, mag, phos
- beta-HCG, AFP anf U/A to Rule out other cancers
- abd US, CT chest CT/xray (find any mets)
Treatment
- partial/total nephrectomy + chemo + proton radiation
- prognosis depends on favorable v unfavorable histology
Neuroblastoma
etiology
symptoms
Etiology
- tumor of the neruonal tissues: sympathetic
- in the adrenal medulla and para-spinal region
- risk: depends on age at diagnosis, (18months + is high risk) MCYN amp and differntiation
Sympotms
- MASS WILL CROSS MIDLINE
- abd Distention and blating with pain
- constipation OR diarrhea
- fevers, fatigue
- hematuria, hypertension or hypotension
- horners syndrome: if involved in paraspinus at the cerical/throacic levels
- raccoon eyes if orbit involved
Neuroblastoma
DIagnosis
Treatment
Diagnosis
- CBC, CMP, mag, phos, LDH, ferritin
- bHCG, AFP, U/A, urine catecholamines (VMA and HVA)
- Curie Scorevia MIBG: with radioactive iodon to see where the cells are
- abd US/CT, MRI and PET
- bone biopsy and aspirate
- tissue biopsy
Treatment
- low risk: surg v obs.
- high risk : see below
- 5 cyclesof chemo
- surgical resection if possible
- 2 cycles of Stem cell rescue (their own cells!)
- immunotherapy + accutane
what is MIBG treatment
MIBG: high does of the radioactive iodine = treatment
lower does = can be used for diagnosis of neuroblastome
priot to radioactive iodine: pt. given SSKI (potassium iodide) to preotect thyroid
the MIBG is taken up by the neurobalstoma cells and destroyed
Treatment protocols
- level of radioactive must be < 7 to discahrge
- track levels of all who enter room
- everything is radioactive: wrappe in plastic
- Q15min vitals during infusion
Osteosarcoma
etiology
symptoms
etiology: osteosarcoma
- MC cancer of bone in kids
- MC location = femur or tibia: close to the knee
symptoms
- bone pain
- swelling or mass at site
- pain waking them at night
- WL, limping
mc location to spread is lungs
Osteosarcoma
diagnosis
treatment
Diagnosis
- xray-mri-biopsy
- xray: codmans triangle & sunburst appearnace
- looks for mets via: CXR, Ct and PET
- get CBC, CMP, U/A, LFts, LDH: elevated usually
treatment
- 10 weeks MAP chemo: methotrex, doxorub, cisplat.
- surgery
- 17 weeks of chemo (osteosarcoma doesnt respond to radiation)
Ewing Sarcoma
Etiology
Symptoms
diagnosis
treatment
Etiology
- teens MC
- sarcoma = of the bone (but can be the tissue surrounding as well)
Symptoms
- bone pain: especially waking them up at night
- limping, fatigue, fever
- chills
- possible swelling/tumor at site
- likely to MET to lungs
Diagnosis
- CBC, CMP, LFTS, U/A
- xray – then MRI — then biopsy
- onion skin appearance on imaging
- chest xray/ct/pet for mets
Treatment
- 14 cycles of chemo
- surgery and/or radiation starting at 6 weeks of chemo
Rhabdomyoscarcoma
etiology
two types
symptoms
Etiology
- a small round blue cel tumor
- rare: but most common soft tissue sarcoma
- unfavorable locations = prostate and bladder
types
Embryonal
- better prognosis
- often mets to Lungs
- fusion (-)
Alvolar
- aggressive
- see mets everywehre
- fusion (+) needing radiation
Symptoms = depend on the location of the disease (sarcome = soft tissue and bone)
throat = difficult to swallow/eat
eye = sweling
vagina= mass or bleeding
bladder = hematuria, bowel or bladder obstruction, difficult/painful urinaion
Rhabdomyosarcoma
Diagnosis
treatment
Diagnosis
- chest CT, whole body PET/MRI
- biopsy, lumbar puncture
- bone marrow biopsy to assess for mets to bone
Treatment
- Chemo +/- radiation
- local control surgery at 11-13 weeks of chemo
Oncologic Emergencies : Neutropenic Fever
definition
why we care
treatment abx.
treatment for those febrile without anc < 200
Neutorpenic Fever
definition
- ANC < 200
- temperature > 38.5
- or temo over 38.5 3times in 24 hours
we monitor neuro fever, because during chemo we expected ANC to go to 0, but when its like less tahtn 200 we are super concerened for opportunistic infections = as the body has virtuall no immune system at this point
Treatment: for febrile + ANC < 200 = cefepime + vanco (and get culutres)
treatment for those febrile without ANC < 200
- PICC = cefepime + vanco
- port: ceftriaxone
- no line = no treatment
Oncologic emergency: Tumore Lysis Syndrome
what is it
symptoms
labs!!!!!!
TLS: occurs usually after 12-72 hours of treatment
- resultes because during treatement youre destroying cells and there is a metablic derrangement in the contents being released from the cells
Symptoms
- N/V, fatigue
- diarrhea
- decreased UOP
- hypocalcemia: weakness, muscle cramps/spasm, tetany, seizures
Diagnosis: Labs
- increased uric acid
- increasd phosphrous
- decreased calcium
- increased potassium
- increased LDH
Tumor Lysis Syndrome
treatment
Prophlyatic treatment = hyperhydration + allopurionol
Treatment
hyperhydratio: 2x maitnence fluids
if K > 6 = dialysis, insulin or kaylexalate (becuase increase K = arrythmia risk)
Ca < 8 = bolus or fix the phosphate
phosphate > 6.5 = sevlamer or amphgel
uric acid > 8 = allopurinol, rasburicase
Onc Emergencies: Spinal Cord Compression
SCC
- seen in thsoe with mets to the spinal cord and progression of the disease
Symptoms
- urinary retention, loss of bladder/bowel function
- wekaness, numbenss and tingling extremities
- back or neck pain
DIagnosis
- STAT full spine MRI
Treatmen t
- steroids: dexamethasone, pain control +/- surgery to remove the mass
- emergency spinal radiation can be used
Onc emerg: Spetic Shock
Ped Onc pt. = high risk of septic shock
Symptoms
- current infection
- fever, fatigue, chills
- hypotension, tachycardia, tachypnea
- no immune system + central line access = bugs love
- therefore, SIRS criteris met = sepsis + end organ damange/poor perfusion
Diagnosis
- STAT blood cultures
- CBC, CMP, mag./phosphate
- blood gases and lactate
- CRP
- chest xray (PNA and resp. disorderS)
Treatment
- fluids: normal salinein boluses
- abx. cefepime and vanco meropenedm for super broad spectrum
Onco Standards of care
abx prophaylaxis for everyone
agents to suppert difficult checmo SE
At risk for PJP/PCP : all pt. get bactrum prophylatically
fungal coverage: levoflox. or caspofungin in AML pt. with ANC < 200
Bowel Regimen
- miralax, senna, colase, etc.
Antiemetics
- ondansetron (zofran), benydraly, etc.
Pain
- tylenol, oxycodone, morphine, dilaudid
Chemo SE
cisplatin
vincristine
cylophosphamide
methotrexate
dinutuximab
doxorub.
Cisplatin
- ototoxic + hearing loss
Vincristine
- constipation severely, peripheral neuropathy, SIADH, jaw pain
Cyclophosphomide
- hemorragic cystitic
- nephrotoppic: lots of fluids
methotrexate
- mucositis (sores)
- nephrotoxic
Dintuximab
- capillary leak syndrome, hypotension
- lots of se: monitor weithgts and fluids closely
Doxorubicin
- cardiotoxicity
