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)
