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Pedia > Oncology > Flashcards

Oncology Flashcards

1
Q

The second most common malignancy in childhood and adolescence

A

Primary CNS

glial cells - 60-70%

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2
Q

Most frequent location of Primary CNS tumor

A

Supratentorial region

Cerebellum

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3
Q

Important for diagnosis of germ cell tumors

A

B HCG

AFP

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4
Q

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

A

Astrocytoma

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5
Q

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

A

Medulloblastoma

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6
Q

Outside the brain

Has same histopathologic configuratjob with neuroblastoma

A

Primitive Neuroectodermal Tumor

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7
Q

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

A

Ependymoma

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8
Q

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

A

Craniopharyngioma

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9
Q

Intraventricular tumor

Large with abundant blood vessel and produce great amount of CSF

A

Choroid plexus papilloma

10% choroid carcinoma

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10
Q

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

A

Atypical teratoid

Rhabdoid tumor

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11
Q

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

A

Neuroblastoma

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12
Q

Amplified protooncogene in Neuroblastoma

A

N-myc

mutation PHOX2B, ALK

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13
Q

Small round blue cell tumor in bone marrow

Increased vanillylmandelic acid or homovanillic acid in utine

Iodine 123 metaiodobenzylguandine to define extent

A

Neuroblastoma

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14
Q

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

A

Wilms tumor

Mets:
Lungs
Regional lymph nodes
Liver

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15
Q 
Hemihypertrophy
Aniridia
GU anomalies
Beckwith-Wiedmann syndrome
Denys-Drash syndrome
A

Wilms tumor

Confirmatory: biopsy, discourages as it results to upstaging

Use CT

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16
Q

Wilms
Aniridia
GU abnormalities
Mental retardation

A

WAGR

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17
Q

Early onset renal failure
Renal mesangial sclerosis
Male pseudohermaphroditism

A

Denys-Drash syndrome

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18
Q

Organomegaly of liver, kidney, adrenal, pancreas
Macroglossia
Omphalocoele
Hemihypertrophy

A

Beckwith-Wiedmann syndrome

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19
Q

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

A

Osteosarcoma

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20
Q

Rare syndrome associated with short stature

Skin telangiectasia

Poikiloderma

Small hands and feet

Hypoplasticity or ABSENCE OF THUMB

High risk of osteosarcoma

A

Rothmund-thomson Syndrome

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21
Q

Undifferentiated small round cell
Diaphysis of long bones and flat bones
Primarily lytic, multilaminar periosteal reaction “onion skinning”

A

Ewing sarcoma

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22
Q

Primary tumor arising in the chest wall of Ewing sarcoma

A

Askin tumors

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23
Q

IHC: Neuron specific enolase (NSE)

S-100

A

Neuroblastoma

24
Q

IHC: desmin, actin, vimentin

A

Rhabdomyosarcoma

25
Q

IHC: CD 99

A

Ewing sarcoma

26
Q

IHC: CD 30 (Lymphocyte common antigen)

A

Lymphoma

27
Q

Most common pediatric soft tissue sarcoma

Most common in H&N

From embryonic mesenchyme of striated ms

FISH + PAX FOX01
Small round blue cell tumor

A

Rhabdomyosarcoma

28
Q

AFP is elevated with

A

endodermal sinus tumors

29
Q

Elevated BHCG in

A

Choriocarcinoma

Germinoma

30
Q

Occur intracranially
mediastinum
gonads

A

Germinoma

In ovary = dysgerminoma
In testis = seminoma

31
Q

Tumors that cause virilization, feminization or precocious puberty

A

Sertoli-Leydig cell
Granulosa cell

Non-germ cell gonadal tumor

32
Q

Children <3
Familial adenomatous polyposis

Alreration in antigen-presenting cell B catenin

Associated with Beckwith-Wiedemann Syndrome, hemihyperplasia

Large asymptomatic, abdominal mass of right lobe, anemia, thrombocytopenia

Prematurity and low birthweight

AFP as monitoring

A

Hepatoblastoma

33
Q

Adolescents

Hepa B, Hepa C

Chronic form of hereditary tyrosinemia, galactosinemia, glycogen storage disease, alpha-1 antitrypsin and biliary cirrhosis

Aflatoxin B

Arises in cirrhotic cells

Fibrolamellar variant in young adult and adolescent

Hepatic mass, weight loss, anorexia and abdominal pain

A

Hepatocellular carcinoma

34
Q

Can differentiate benign from malignant hepatic CA

A

UTZ

35
Q

Wilms
Aniridia
GU abnormalities
Mental retardation

A

WAGR

36
Q

Early onset renal failure
Renal mesangial sclerosis
Male pseudohermaphroditism

A

Denys-Drash syndrome

37
Q

Organomegaly of liver, kidney, adrenal, pancreas
Macroglossia
Omphalocoele
Hemihypertrophy

A

Beckwith-Wiedmann syndrome

38
Q

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

A

Osteosarcoma

39
Q

Rare syndrome associated with short stature

Skin telangiectasia

Poikiloderma

Small hands and feet

Hypoplasticity or ABSENCE OF THUMB

High risk of osteosarcoma

A

Rothmund-thomson Syndrome

40
Q

Undifferentiated small round cell
Diaphysis of long bones and flat bones
Primarily lytic, multilaminar periosteal reaction “onion skinning”

A

Ewing sarcoma

41
Q

Primary tumor arising in the chest wall of Ewing sarcoma

A

Askin tumors

42
Q

IHC: Neuron specific enolase (NSE)

S-100

A

Neuroblastoma

43
Q

IHC: desmin, actin, vimentin

A

Rhabdomyosarcoma

44
Q

IHC: CD 99

A

Ewing sarcoma

45
Q

IHC: CD 30 (Lymphocyte common antigen)

A

Lymphoma

46
Q

Most common pediatric soft tissue sarcoma

Most common in H&N

From embryonic mesenchyme of striated ms

FISH + PAX FOX01
Small round blue cell tumor

A

Rhabdomyosarcoma

47
Q

AFP is elevated with

A

endodermal sinus tumors

48
Q

Elevated BHCG in

A

Choriocarcinoma

Germinoma

49
Q

Occur intracranially
mediastinum
gonads

A

Germinoma

In ovary = dysgerminoma
In testis = seminoma

50
Q

Tumors that cause virilization, feminization or precocious puberty

A

Sertoli-Leydig cell
Granulosa cell

Non-germ cell gonadal tumor

51
Q

Children <3
Familial adenomatous polyposis

Alreration in antigen-presenting cell B catenin

Associated with Beckwith-Wiedemann Syndrome, hemihyperplasia

Large asymptomatic, abdominal mass of right lobe, anemia, thrombocytopenia

Prematurity and low birthweight

AFP as monitoring

A

Hepatoblastoma

52
Q

Adolescents

Hepa B, Hepa C

Chronic form of hereditary tyrosinemia, galactosinemia, glycogen storage disease, alpha-1 antitrypsin and biliary cirrhosis

Aflatoxin B

Arises in cirrhotic cells

Fibrolamellar variant in young adult and adolescent

Hepatic mass, weight loss, anorexia and abdominal pain

A

Hepatocellular carcinoma

53
Q

Can differentiate benign from malignant hepatic CA

A

UTZ

54
Q

Embryonal malignancy of the retina
Most common intraocular tumor

Cat’s eye reflex / White pupil reflex (leukocoria)

Inactivation of retinoblastoma gene1 RB1

Small round blue cell with rosette formation

Chalky white gray retinal mass with soft, friable consistency

A

Retinoblastoma

55
Q

Small round blue tumor with rosette formation

Retinoblastoma

A

Flexner-wintersteiner rosette

56
Q

Marker for fibrolamellar HCC

A

neurotensin

IL 8

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