What is the most common childhood cancer ages 0-14?
age 15-19?
leukemia (0-14)
Lymphoma (15-19)
Uncommon sx that might suggest cancer
- recurrent fever and bone pain(leukemia)
- mmorning headache with vomiting(cns tumor)
- neck mass not responding to abx(lymphoma(
- chronic bone pain, swelling, limp(bone tumor)
- progressive abdominal distension(abd tumor)
- bleeding, bruising, pallor, fatigue(leukemia)
- combined cytopenias (leukemia)
Brain tumors
- sx caused by what
- sx
- what is normal ICP?
Sx caused from increased pressure and location
Sx
- HA
- Vomiting
- Decreased academics
- personiality changes
- large head, bulging fontanelle
- anorexia
- vision changes
- seizure
Normal ICP is 10-18 mmHg
Lymphoma
- localized sx
- systemic sx
localized
- painless enlarged lymph nodes
- abd pain or mass
- emergent: compromise of vital structure
Systemic
- fevers, night sweats, weight loss, fatigue
- pain with alcohol consumption
Superior vena cava syndrome and superior mediastinal syndrome are medical emergencies, true or false?
True
-emergency management may include steroids or radiation
Abdominal Tumor
sx
sx
- distension, palpable mass
- nausea, vomiting, diarrhea, constipation, weight loss, fever
- obstruction, pain, jaundice, blood in urine
Bone tumor
sx
- pain/tenderness/limp
- soft tissue mass
- night pain
- growing pain
- history or minor trauma
- pathologic fracture
- pain often present for 2-3 months
Leukemia
ALL types
AML type of dx (clinical/molecular)
ALL types
- precursor B cell (80-85% of ALLs)
- Burkitts
- T cell
AML is a clinical AND molecular dx
Cancer Predisposition syndromes
Li Farumeni
Ataxia Telangectasia
Neurofibromatosis Type 1
Bloom Syndrome
Down Syndrome pts are 10-20x more likely to develop what type of cancer?
leukemia
-and they respond to chemo differently because they have different immune systems
AL Leukemia
- presentation
- heme findings
- dx
Presentation-
- Bone pain (35%)
- adenopathy
- hepatosplenomegaly
- thymic expansion
- CNS sx (HA, neck pain, CN palsy, seizures)
- Testicular Involvement
Heme findings-
- WBC can be high, normal, or low
- decreased neutrophil number and function
- hyperleukocytosis (WBC greater than 250-500K)
- decreased platelets
- decreased RBCs
Dx
- CBC with diff, peripheral smear
- bone marrow aspirate, LP
- Flow cytometry, morphology, and cytogenetics
- sanctuary sites (CNs and Testes): LP, testicular exam
Leukemia
Tx
What has a worse prognosis, aLL or AML?
Tx
- 4 week 3 drug induction
- 4 week oral consolidation with IT
- 8 week Int maint
- 8 week delayed intensification
- 2nd int maint
- maintenance (2yrs +)
- this is for standard risk ALL
-CNS 3 disease and T ALL typically receives cranial irradiation
AML has a worse prognosis than ALL. AML 5yr survival is 50% compared to more than 90% in ALL
AML
presentation
smear findings
treatment
Presentation
- fever
- bone pain
- LAD
- hepatosplenomegaly
- gingival hypertrophy
- sepsis
- anemia
- thrombocytopenia
- hemorrhage
- DIC
Smear findings
-Auer Rod
Treatment
- remission induction (two course intense chemo, very high risk of invasive infection)
- post remission consolidation
- CNS prophylaxis (lower rate of CNS relapse than ALL)
- maintenance
- no irradiation even with CNS disease
Hodgkin Lymphoma presentation lab findings Dx Tx
Presentation -painless lymphadenopathy -mediastinal mass -constitutional sx -B symptoms (weight loss, drenching night sweats, unexplained fevers for 3 consecutive days) fatigue -anorexia -mild weight loss -pain following alcohol ingestion
Lab findings
-elevated CRP, ESP, ferritin, copper, anemia of chronic inflammation
Dx
- lymph node bx
- Reed Sternberg cells**
- CXR/CT/MRI/PET
Tx
-chemo and radiation
Non-hodgkin lymphoma common in who presentation Tx Prognosis
Common in
-young while male children (less than 10)
Presentation -Similar to HL -different types have different presetation (ex. endemic burkitt has jaw swelling, abd or orbital swelling, paraspinal mass, CNS involvement...think Africa) -painless lymphadenopathy -mediastinal mass -constitutional sx -B symptoms (weight loss, drenching night sweats, unexplained fevers for 3 consecutive days) fatigue -anorexia -mild weight loss -pain following alcohol ingestion
Tx
- depends on type and staging
- all get chemo
- most receive CNS ppx
Prognosis
-overall survival is high (burkitt is 90%)
Neuroblastoma
presentation
Presentation
- Periorbital echymosis (think of the picture of the 2 year old with purple shiners on both eyes)
- neck mass
- GU sx
- malaise, irritable, weight loss, anorexia
- il defined pain
- abd distension
- fever
- raccoon eyes
Dx
- CT/MRI (spine)
- bilateral bone marrow bx
- radiolabelled MIBG
- Tumor bx
What is the most common renal tumor of childhood?
Wilms Tumor
Wilms tumor Presentation Dx Staging Tx
Presetation
- usually an asymptomatic upper abd mass
- abd pain
- fever
- anemia
- hematuria
- HTN
- Hx of FMx
Dx
- resection and bx
- CT of chest/abdomen
- Abd ultrasound
Staging
- confinded to kidney
- confined to renal fossa
- gross residual disease: unresectable primary, lymph node mets, positive, surgical margins, tumor spill
- disseminated
- bilateral tumors
Tx
- surgical resection
- chemo
- radiation for higher stages
Retinoblastoma
Presentation
Dx
Tx
Presentation
- intraocular disease (strabismus, leukocoria=white reflex)
- advanced intraocular disease (glaucoma)
- extraocular disease(metastases)
Dx
- exam under anestesia
- ocular ultrasound
- MRI of orbits and brain
- genetic counseling-ALL pts
Tx
- tx determined by staging, grouping, and laterality of disease
- goals: cure, then eye salvage, then vision salvage
Craniopharyngioma
- what
- presentation
- 5 year survival
- tx
What-
- arises from the Rathke cleft
- can occur anywhere along the course of the duct- pharynx to sella turcica and third ventricle
Presentation-
-interference with pituitary function (hypothyroid, obesity, diabetes insipidus)
-Interfere with visual function (amblyopia, blindness)
-mass effect (HA, n/v)
5 year survival: 80%
Tx
-surgery and radiation
What is the most common malignant pediatric brain tumor?
Medulloblastoma
-papilledema is MC finding
Ependymoma
- glial tumors arise from what cells
- common presentation
Glial tumors arise from ependymal cells
Common Presentation
- increased ICP
- HA, ataxia, nystagmus, sz
50% of pediatric CNS tumors are…
Pilocytic Astrocytoma
Spinal cord tumors
types
signs and sx
dx
Types: astrocytomas, ependymomas, neurofibromas, bone tumors
Signs and sx
- chronic back or neck pain MC*
- scoliosis
- progressive weakness
Dx
- PE for strength, sensation, reflexes)
- MRI
