Oncology
Terms
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- Tumors of infancy
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neuroblastoma
wilms
retinoblastoma
primitive neuroectodermal tumor
myeloproliferative disease (Downs, increases risk of leukemia later) - Philadelphia chromosome
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9;22 tyrosine kinase protein always on.
CML, ALL - genetic conditions increasing leukemia risk
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Downs, Klinefelter, Turner, NF
Fanconi, Bloom, Diamond-Blackfan anemia, Schawachman, ataxia-telangiectasia, SCID, paroxysmal nocturnal hemoglobinuria, Li-Fraumeni S. - CML
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all have Philadelphia chromosome (9;22)
initial chronic phase, , big spleen causing pain, leukocytosis, mild anemia and thrombocytopenia, in 3-4 yrs blast crisis phase begins. all counts rise rapidly with terrible tumor lysis sx - Hodgkins
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Lymphoma, mostly teens. childhood, teen, and older adults.
Reed-Sternberg cell (large multiple nuclei) same as seen in mono. painless firm cervical adn supraclavicular lymphadenopathy.ant. mediastinal mass - non-Hodgkins lymphoma
- T or B cells. incidence increases steadily throghout life. (Burkitt is most common type). intrathoracic tumor. wheezing teen with mediastinal mass.
- Brain tumors
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medulloblastoma
astrocytoma
ependymomoa
craniopharyngioma - astrocytoma
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40% pediatric brain tumor
can occur anywhere
cerebellum most common
contrast enhancing nodule in wall of cystic mass
Low grade most common (juvenile pilocytic astrocytoma) not aggressive - ependymal tumors
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ependymoma is most common
posterior fossa, 6yr
non-invasive - Choroid plexus tumors
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most common brain tumor if <1yr
intraventricular from chorioid plexus
increased ICP - Primitive neuroectodermal tumors (PNET)
- metastisize within CNS. medullloblastoma, supratentorial PNET, ependymoblastoma.
- Medulloblastoma
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midline cerebellar
males
5-7yrs
increased ICP and cerebellar dysfxn - Neuroblastoma
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embryo tumor of sympathetic NS. #3 most common
most common cancer of infants
90% are <5yr
prognosis based on histo. most in abdomen, adrenal or retropertoneal ganglia. firm nodular mass causingt abdominal discomfort. Calcified on CT (distinguishes from Wilms) - Renal tumors
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Wilms (nephroblastoma)
congenital mesoblastic nephroma
nephroblastomatosis
multicystic nephroblastoma
renal cell carcinoma - Wilms Tumor
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no fam hx
favorable and unfavorable histo
WAGR, Denys-Drash, Beckwith-Wiedemann
abdominal mass, no sx , smooth firm - Diff dx abdominal tumors
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Wilms (preschool)
neuroblastoma (preschool)
non-Hodgkins lymphoma (>1yr)
rhabdomyosarcoma (all)
germ cell/teratoma (preschool, teens)
hepatoblastoma (school age, teens)
hepatoma - Wilms syndromes
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WAGR (Wilms, aniridia, GU abnorm, MR)
Denys-Drash early renal failure, pseudohermaphrodism in males
Beckwith-Wiedemann - Rhabdomyosarcoma
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most common soft tissue tumor
occur anywhere, most common in head and neck
presents as mass w/ sx of mass effect - Osteosarcoma
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teen during growth spurt
metaphysis of long bones
local pain and swelling, often hx of injryu
sunburst on XR - Ewings sarcoma
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teens
diaphyses of long bones and flat
local pain, fever
onion skinning on XR, mets to lungs, bones. - osteochondroma
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benign bone tumor
manhy asx and unrecognized.
childhood metaphysis of long bones distal femur, prox humerus, prox tibia, enlarges as child grows. presents 5-15yr. bony nonpainful mass. - enchondroma
- benign hyaline cartilage lesion centrally in bone. asx and occur in hands
- chondroblastoma
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rare
epiphysis long bones
teens
pain in adjacent joint. hip shoulder knee - osteoid osteoma
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small benign bone tumor
5-20yr
chronic worsening pain, worst at night
any bone. most common prox femur and timbia - osteoblastoma
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progressive growing destructive lesion
vertebrae preferred, but anywhwere
dull aching pain. - fibromas
- fibrous lesions of bone in 40% of kids over 2yrs. defect in ossification, no neoplasm. incidentaloma
- unicameral bone cysts
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3yr-skeletal maturity
fluid filled lesions. some go away. most asx but may cause pathologic fx.
prox humerus or femur - aneurysmal bone cyst
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up to teens
cavernous space filled with blood and tissue.
femur, tibia, spine.
progressively growingt
pain and swelling - fibrous dysplasia
- developmental abnormality with fibrous replacement of cancellous bone.
- eosinophilic granuloma
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boys 5-10
skull, but any bone possible
local pain and swelling
tenderness and warmth - retinoblastoma
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60% unilateral and nonhereditary
only 10% found by screening
leukocoria, strabismus, orbital inflammation, hyphema
95% cured - Germ Cell tumors
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gonadoblastoma: gonadal dysgenesis adn Y chromosome, failure to fully masculinize external genitalia
Teratoma: masses.
Germinomas: intracranial, mediastinum, testes.
Sertoli-Leydig, granulosa cell cause feminization or precocious puberty - Hepatoblastoma
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<3yrs beckwith-wiedemann
large asx abdominal mass. R lobe of liver. weight loss, anorexia, v, abdmoina pain later. mets to regional lymph nodes and lungs.
AFP elevated in most
bili, LFT normal
anemia common, thrombocytosis common
check HBV, HCV - Hepatocellular carcinoma
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teens associated with chronic HCV HBV
hepatic mass, abdominal distension, anorexia, weight loss, pain. acute abdominal crisis with rupture and hemoperitoneum. AFP elevated in some.bili normal. FLTs abnormal - Hemangiomas
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more in girls, preterm, and S/P CVS. arrise in first yr and grow fast. involute by 15yrs
Kasabach-Merrit: rapidly enlarging lesion, thrombocytopenia, hemolytic anemia. coagulopathy (plt, RBC stuck in hemangioma). - Kasabach-Merrit
- rapid enlarging lesion with thrombocytopenia, hemolytic anemia, coagulopathy.
- lymphangiomas and cystic hygromas
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benign lymphatic malformations
most in head and neck
intrathoracic lymphatic malfomations acan present as mediastinal mass, pericardial effusion