oncology Flashcards
most common cancer in children, accounting for 30% of all childhood malignancies
acute leukemia (mostly ALL)
- Second most common of all childhood malignancies
- most common peds solid organ tumor
Primary malignant CNS tumors
most prevalent solid tumor in kids outside of the cranium
neuroblastoma
age group at risk for neuroblastoma, ALL, osteosarcoma, ewing sarcoma, hogdkins
- 0-2 years: neuroblastoma
- 2-5 years: ALL
- 10-15 years: osteosarcoma, Ewing sarcoma
- 15-19 years: Hodgkin lymphoma
top 3 most common cancers in kids
- leukemia
- CNS tumors
- lymphoma
trisomy 21 increases risk of ___
leukemia
fanconi anemia increases risk of ____ and ____
what two cancers
AML
solid tumors
what disorder is associated w/ increased risk of all these cancers?
- Leukemia
- Adrenocortical carcinoma
- CNS tumors
- Rhabdomyosarcoma
- Osteosarcoma
Li fraumeni syndrome
Von hippel-lindau dz increases the risk of what 3 cancers
- Retinal and CNS Hemangioblastoma
- Clear cell renal cell carcinoma
- Pheochromocytoma
The overall risk of malignancy is up to 4x higher than that of the general population with which predisposition disorder?
neurofibroma type 1 (NF1)
2 common tumors with NF1
neurofibroma; mostly benign
optic pathway glioma
3 common tumors associated with NF2
- vestibular schwannoma
- meningioma
- spinal tumors
Consecutive genetic lesions results in arrest in development and abnormal proliferation; more in B cell
* Aberrant lymphoblast cell proliferation and survival are hallmarks
this describes the pathogenesis of what cancer
ALL
- age 2-5
- nonspecific sx
- bone pain/refusal to bear weight
- fever, pallor, bruising, wt loss, hepatosplenomegaly, etc
- CNS & testicular involvement and mediastinal mass
ALL
procedure to evaluate the presence of leukemic blasts in the CSF; can give first dose of intrathecal chemotherapy this way too
lumbar puncture
gold standard for dx of ALL
bone marrow biopsy
over 25% blasts in marrow is leukemia
under 25% blasts in marrow is indicative of …
lymphoma
tx of ALL
- multi-drug remien + CNS directed IT chemo (to prevent leukemic meningitis)
takes 2.5 yrs to complete
3 goals, post-induction for ALL
- Prevention of leukemic regrowth
- Reduction of residual tumor burden
- Prevention of the emergence of drug-resistance in the remaining leukemic cells
genetic changes in the HPC that alter hematopoietic proliferation and differentiation so theres a lot of abnormal, immature myeloid cells in the bone marrow and peripheral blood
AML
which cancer has auer rods + blue ring outside
AML
- nonspecific sx
- Gingival hypertrophy, chloroma
- bleeding/DIC
AML presentations
labs in AML vs ALL
- AML: Elevated D-dimer, PTT, and PT/INR & Decreased fibrinogen
- ALL: low ANC, high LDH & uric acid
- BOTH: thrombocytopenia
between AML and ALL which one has scant cytoplasm
ALL
what post-remission therapy is offered to children with high-risk cytogenetics in first CR or patients with treatment-related AML
allogenic BMT
tx of APML
all-trans retinoic acid (ATRA) to reduce risk of DIC; started if APML is suspected
- Clonal transformation of cells of B-cell origin
- Pathognomic bi-nucleated Reed-Sternberg cells
hodgkins lymphoma pathogenesis
bimodal age distribution and more in females
- rubbery, firm lymphadenopathy
- pruritis, fatigue, anorexia
- B symptoms
- mediastinal mass esp in kids over 12 yo w/ associated sx
Hodgkins lymphoma
what are the B symptoms
- night sweats x 1 month
- weight loss (over 10% in 6 mo)
- persistent unexplained fevers (over 38 C) x 1 month
6 associated sx of mediastinal mass
- SVC syndrome
- dysphagia
- dyspnea
- orthopnea
- cough
- stridor
3 imaging modalities used in HL evaluation
CXR
CT w/ contrast
PDG-PET CT skull base to thigh
gold standard for HL diagnosis
excisional biopsy bc needle doesnt give enough tissue
aspirates for select patients
lugano staging of HL (1-4)
- localized; single node or organ
- 2+ nodes on same side of diaphram
- 2+ nodes above & below
- widespread
low vs intermediate vs high risk chemo tx of HL
- low: 2-4 cycles +/- radiation
- intermediate: 2-6 cycles + response-based radiation
- high: 3-5 cycles + response-based radiation
5th most common diagnosis of pediatric cancer in children < 15 years
NHL
Congenital and acquired immunodeficiency syndromes and DNA damage repair syndromes place affected patients at risk for this kind of cancer..
includes HIV, WAS, CVID
NHL
- painless lymphadenopathy (most common)
- mediastinal mass
this descirbes what kind of cancer
NHL
- painless lymphadenopathy (most common)
- mediastinal mass
AND - belly pain similar to appendicitis
- abdominal mass
- sick
- intussusception (part of intestine slides into another part)
what kind of cancer (specific)
Burkitt lymphoma
3 imaging modalities to evaluate NHL
- CT
- PDG-PET scan
- skeletal scintigraphy for bone involvement
2 procedures to evaluate for NHL
- bone marrow biopsy
- lumbar puncture
tx for burkitt lymphoma
multi-agent chemo +/- immunotherapy
NO RULE FOR SURGERY OR RADIATION
tx of T-lymphoblastic leukemia
combo chemo based on ALL
most common low grade glioma
pilocytic astrocytoma
name a type of high grade glioma
DIPG
- Most cases are sporadic (random) but can be associated with various genetic conditions like Neurofibromatosis, Von Hippel Lindau, Tuberous Sclerosis
- incresed risk in ppl exposed to ionizing radiation to head or spine
CNS pathogenesis
- early morning headache relieved w/ vomiting
- clumsiness, worse handwriting
- CN palsies
- vision changes, seizures
- endocrinopathies
CNS tumor presentation
most common presenting sx of CNS tumors in infants
macrocephaly
CNS tumor with N/V, HA, gair & coordination issues is likely located where?
posterior fossa
CNS tumor with CN palsies, gait & coordination issues is likely located where?
brain stem
CNS tumor with back pain, weakness, gait issues is likely located where?
spinal cord
most common sx of supratentorial and central CNS tumors
HA
tx of CNS tumors (2)
- near total surgical resection
- chemo therapy to delay or replace radiation
Abnormal growth of embryonic neural crest cells that normally make up the sympathetic ganglia and adrenal medulla
neuroblastoma (NBL)
median age and gender of NBL diagnosis
17 months; males
- metastasis to marrow, liver, skin causing constitutional sx, blueberry muffin rash
- spinal cord compression– pain, numbness limb weakness
- horners syndrome– ptosis, miosis, anhydrosis
- opsoclonus myoclonus syndrome
- raccoon eyes
NBL clinical presentation
opsoclonus myoclonus syndrome (3)
Rapid multidirectional eye movements
involuntary muscle spasms
irritability
Urine HVA and VMA elevated in almost all patients with this cancer
NBL
2 imaging for NBL
- MRI > CT (can still do CT)
- I-123 MBIG scan (follow / PET if neg.)
biopsy shows Homer-Wright rosettes (small clusters of small round blue cells separated by a fibrillar matrix)
what cancer?
NBL
management of NBL (2)
- observation– can resolve; can do US or urine test
- if over 12 months– intense multimodal therapy including radiation & surgery & (MBIG therapy?)
- Thought to arise from a mesenchymal stem cell that differentiates towards fibrous tissue, cartilage, or bone
- more in Black males
- more in rapid bone growth or in prior cancer tx
osteosarcoma (OSt)
most common primary malignant bone tumor
OSt
- localized pain esp after an injury
- generally no systemic sx
- large, TENDER to palpation, soft tissue mass
OSt presentation
4 imaging for OSt eval
- plain films– sunburst, Codman’s triange, pathologic fracture
- MRI of long bone
- CT for chest
- PET/radionuclide scan for whole body
biopsy is for definitive diagnosis
tx of OSt (3)
- surgery is standard
- chemo
- radiation if incomplete resection or unresectable
- mesenchymal progenitor cell origin
- Nonrandom chromosomal translocations that involve breakpoints within the EWSR1 gene
- Can develop in any bony or soft tissue but tend to develop in the diaphysis of long bones and the axial skeleton
ewing sarcoma (EWS)
- 2nd most common primary bone malignancy
- more in white males
EWS
- localized pain or swelling; often worse at night or w/ exercise
- minor trauma may initiate it
- constitutional sx
EWS presentation
first image obtained when evaluating for EWS
plain film xray
XRAY findings below match up with what cancer
- “moth eaten” bony lesion
- codman’s triangle– displaced periosteum and cortex expansion
- onion skin apperance
EWS
other imaging used in EWS evaluation
- MRI or CT of primary site
- CT for pulmonary metastases
- radionuclide bone scan
way to evaluate for bone marrow metastases with EWS
- bone marrow biopsy
- tumor biopsy w/ CT
EWS tx (3)
- chemo
- surgery > radiation
- adjuvant radiation
effects of cancer survival
- neurologic– memory, coordination, academic performance, etc issues
- endocrine– impaired growth, obesity, thyroid dysfunction
- effect on fertility depends on age of therapy and what kind (radiation, alkylating agents); gonadal function and pre puberty ok
- AML tx with anthracyclines can cause cardiomyopathies
- higher risk of secondary malignancies if tx with radiation
family centered care
- respect for family preferences
- flexibility and custamization
- partnership an dcommunication
- inforamtion sharing
