Oncology

Question 1

2 most common primary malignant bone tumors during childhood and adolescence

Answer 1

Osteosarcoma and Ewing sarcoma

Question 2

Unilateral pain. Not relieved by NSAIDS. Most common distal femur or proximal tibia then humerus. Occurs in metaphyseal region of long bones

Answer 2

Osteosarcoma

Question 3

Xray demonstrates periosteal reaction (sunburst pattern). Can also see codman triangle (reaction at junction of mass and periosteum)

Answer 3

Osteosarcoma

Question 4

Group of small, round-cell undifferentiated tumors of neural crest origin. PRIMARILY bone tumor, but can arise from soft tissue

Answer 4

Ewing sarcoma

Question 5

Present with pain and swelling of flat bones (ribs, pelvis) and diaphyses of long bones. Can have systemic findings (fever, weight loss)

Answer 5

Ewing sarcoma

Question 6

Xray demonstrates lytic lesion with lamellated/onion skin periosteal reaction

Answer 6

Ewing Sarcoma

Question 7

Common benign cartilage-forming bone tumor. Often asymptomatic and in metaphysis of long bones

Answer 7

Osteochondroma

Question 8

On xray - stalks of broad-based projections from the surface of the bone. usually a cartilage “cap” which can be as thick as 1cm

Answer 8

Osteochondroma

Question 9

Benign tumor. Presents with unremitting and worsening pain that is relieved by ASA and NSAIDs. Usually proximal femur and tibia

Answer 9

Osteoid osteoma

Question 10

On xray - round or oval metaphyseal or diaphyseal lucency surrounded by sclerotic bone

Answer 10

Osteoid osteoma

Question 11

Diseases that are risk factors for CNS tumors

Answer 11

Neurofibromatosis type 1, neurofibromatosis type 2, tuberous sclerosis, li-fraumeni syndrome, turcot syndrome, nevoid basal cell carcinoma syndrome, von hippel-lindau disease

Question 12

Parinaud syndrome

Answer 12

triad of impaired upward gaze, dilated pupils with better reactivity to accomodation than light and retraction or conversion nystagmus with lid retraction. Due to compression of midbrain tectum

Question 13

Side effect methotrexate

Answer 13

myelosuppression, renal and hepatic toxicity

Question 14

side effect cyclophosphaide

Answer 14

hemorrhagic cystitis

Question 15

side effect doxorubicin

Answer 15

cardiotoxciity

Question 16

side effect bleomycin

Answer 16

pulonary fibrosis

Question 17

side effect vincristine

Answer 17

peripheral neuropathy

Question 18

side effect cysplatin

Answer 18

nephrotoxic, ototoxic, neurotoxic

Question 19

presents with headache, vomiting, seizures, motor symptoms, behavioral abnormalities. Account for 40% of all childhood brain cancers - 25% are GBM

Answer 19

High grade astrocytoma

Question 20

presents with clumsiness and usteadiness of hte arms and legs. Headaches and vomiting also occur. Tend to have best prognosis of intracranial malignancies

Answer 20

Cerebellar astrocytoma

Question 21

present with morning headache, ovmiting and lethargy. Ataxis of trunk/limbs. Often have hydrocephalus at diagnosis. Can have head-tilt due to 4th cranial nerve dysfunction or impending herniation

Answer 21

medulloblastoma

Question 22

Symptoms depend on tumor location - but relate to blockage of CSF flow

Answer 22

Ependymoma

Question 23

present with parinaud syndrome (imapired upward gaze, dilated pupils with better reactivity to acoomodation than light and retraction/conversion nystagmus with lid retraction) can also have hormonal secretion

Answer 23

Germ cell tumors

Question 24

present with headache and vomiting. Often have visual changes and can have endocrinologic changes. Often have personality or sleep pattern changes as well

Answer 24

craniopharyngioma

Question 25

Rare, except in children with neurofibromatosis type 2 OR long term survivors of other brain tumors who have received radiation

Answer 25

Meningioma

Question 26

Neuroblastoma primary sites

Answer 26

40% arise within the abdomen (adrenal medulla) and 30% in nonadrenal abdomen (paravertebral ganglia, pelvic ganglia and organ of zuckerkandl). 20% occur in paravertebral ganglia of chest/neck

Question 27

Common neuroblastoma metastasis sites (often metastasized already at presentation)

Answer 27

lymph nodes, bone, bone marrow, liver, skin (subQ bluish nodules), orbital (raccoon eyes - differentiate from NAT)

Question 28

Neuroblastoma diagnosis

Answer 28

biopsy with histology of neural origin tumor. Neural crest cells so metabolize catecholamines - increased urinary metabolites HVA and VMA

Question 29

Wilms tumor associated disorders

Answer 29

GU anomalies (cryptorchidism and hypospadias), hemihyperplasia, sporadic aniridia

Question 30

Wilm’s associated syndromes

Answer 30

WAGR (wilms, aniridia, GU anomalis, reduced intellect), beckwith wiedmann, denys-drash (wilms, nephropathy, male undervirilization)

Question 31

Tumor that would lead to bitemporal vision loss

Answer 31

pituitary tumor or craniopharyngioma

Question 32

loss of visual perception in a single visual field

Answer 32

occipital tumor

Question 33

Risk factors for hepatoblastoma?

Answer 33

Pre-term birth and beckwith-wiedemann syndrome

Question 34

Differential time course presentation for HCC versus hepatoblastoma?

Answer 34

Hepatoblastoma more common in children < 3, HCC more common in children > 3

Question 35

Most common sites of involvement for langerhans cell histiocytosis

Answer 35

skull, femur, ribs, vertebra and humerus

Question 36

a lytic, “punched out” circular lesion on xray

Answer 36

Langershans cell histiocytosis

Question 37

Various things that can present with langerhans cell histiocytosis

Answer 37

bony lesions, cutaneous lesions (seborrheic like often, but can be variable), lymph node enlargement, hepatomegaly, pituitary dysfunction (with DI)

Question 38

Typically high grade in pediatrics, originating in peyers patches of the GI tract (Ileocecal junction most common)

Answer 38

burkitt’s lymphoma