Oncology Flashcards
The second most common malignancy in childhood and adolescence
Primary CNS
glial cells - 60-70%
Most frequent location of Primary CNS tumor
Supratentorial region
Cerebellum
Important for diagnosis of germ cell tumors
B HCG
AFP
Originate from astrocytes
Grade 1 pilocytic most common in children in cerebellum
Enhancing nodule within the wall of a cystic mass
Rosenthal fibers - condesed glial filaments
Astrocytoma
Most common highly malignant tumor in children (90% embryonal CNS) in cerebellar vermis
Males (5-7 y/o)
Headache, vomiting, truncal ataxia
Homer wright rosettes
+ synaptophysin
Heterogenously enhancing, solid, 4th ventricular mass
Medulloblastoma
Outside the brain
Has same histopathologic configuratjob with neuroblastoma
Primitive Neuroectodermal Tumor
Most common site in the 4th ventricle
Derived from ependymal lining
Male
2-5 months of unsteadiness, headache, double vision and facial asymmetry
Perivascular pseudorosette
Ependymal rosette
Monomorphic nuclear morphology, nonpalisading foci of necrosis
Enhancing 4th ventricle mass with cerebellopontine predilection
Ependymoma
From Rathke’s duct
Seen in hypophyseal stalk in 3rd ventricle
Cystic, solid, large and heterogenous
Can infiltrate brain tissue despite lack of malignant features (adamantinous)
Endocrinologic abnormalities
Craniopharyngioma
Intraventricular tumor
Large with abundant blood vessel and produce great amount of CSF
Choroid plexus papilloma
10% choroid carcinoma
Embryonal malignancy
Children younger than 5 years
As in medulloblastoma but primarily in infants
Facial weakness, strabismus
Heterogenous pattern of cell including rhabdoid cell that express epithelial membrane antigen and neurofilament antigen
Mutation ib INI1 gene
Atypical teratoid
Rhabdoid tumor
Affects adrenal glands or midline stuctures (paraspinal sympathetic) including the pituitary
Most common extracranial solid tumor in children arising from SNS
Most commonly diagnosed malignancy in infants
Arises from primordial neural crest cells
Elevated catecholamines (VMA or HVA)
Calfications
N-myc gene amplification
Neuroblastoma
Amplified protooncogene in Neuroblastoma
N-myc
mutation PHOX2B, ALK
Small round blue cell tumor in bone marrow
Increased vanillylmandelic acid or homovanillic acid in utine
Iodine 123 metaiodobenzylguandine to define extent
Neuroblastoma
Most common primary renal tumor in children
Second most common malignant abdominal tumor
Autosomal dominant
Arise from undifferentiated mesodermal tumors: complex, mixed embryonal neoplasm (blastema, epithelia, stroma)
Loss of function mutation of tumor supressor gene
Peaks at 3-4 years old
Wilms tumor
Mets:
Lungs
Regional lymph nodes
Liver
Hemihypertrophy Aniridia GU anomalies Beckwith-Wiedmann syndrome Denys-Drash syndrome
Wilms tumor
Confirmatory: biopsy, discourages as it results to upstaging
Use CT
Wilms
Aniridia
GU abnormalities
Mental retardation
WAGR
Early onset renal failure
Renal mesangial sclerosis
Male pseudohermaphroditism
Denys-Drash syndrome
Organomegaly of liver, kidney, adrenal, pancreas
Macroglossia
Omphalocoele
Hemihypertrophy
Beckwith-Wiedmann syndrome
Adolescents
Spindle cell producing osteoid
Metaphysis of long bones
Sclerotic destruction sunburst pattern
Loss of heterozygosity of Rb
Germline p53 mutation Li-Fraumeni
Previously irradiated bone
Increased in Hereditary Retinoblastoma
Osteosarcoma
Rare syndrome associated with short stature
Skin telangiectasia
Poikiloderma
Small hands and feet
Hypoplasticity or ABSENCE OF THUMB
High risk of osteosarcoma
Rothmund-thomson Syndrome
Undifferentiated small round cell
Diaphysis of long bones and flat bones
Primarily lytic, multilaminar periosteal reaction “onion skinning”
Ewing sarcoma
Primary tumor arising in the chest wall of Ewing sarcoma
Askin tumors
IHC: Neuron specific enolase (NSE)
S-100
Neuroblastoma
IHC: desmin, actin, vimentin
Rhabdomyosarcoma
