Hema Onco

Question 1

MC extracranial solid tumor in children and the MC diagnosed malignancy in infants

Answer 1

Neuroblastoma

Question 2

Median age of children at diagnosis of neuroblastoma is

Answer 2

22 mos

Question 3

90% of cases of neuroblastoma are diagnosed by what age

Answer 3

5 yrs old

Question 4

Massive involvement of the liver with metastatic disease, with or without respiratory distress in neuroblastoma

Answer 4

Pepper syndrome

Question 5

Unilateral ptosis, myosis and anhidrosis associated with a thoracic or cervical primary tumor, these symptoms do not resolve with tumor resection

Answer 5

Horner syndrome

Question 6

Is strongly associated with advanced tumor stage and poor outcomes in neuroblastoma

Answer 6

Amplification of MYCN

Question 7

Confers better prognosis if the child is <18mo old at diagnosis and if amplification og MYCN is not present

Answer 7

Hyperdiploidy

Question 8

Is MC in children < 1yr old at diagnosis, occurs through local invasion or distant hematogenous or lymphatic routes

Answer 8

Metastatic spread

Question 9

MC site of metastasis of neuroblastoma

Answer 9

regional or distant lymph nodes, lone bones and skull, bone marrow, liver and skin

Question 10

Neuroblastoma originating in the superior cervical ganglion can result in

Answer 10

Horner syndrome

Question 11

Neuroblastoma can also be associated with a paraneoplastic syndrome of autoimmune origin termed

Answer 11

Opsoclonus-myoclonus-ataxia syndrome

Question 12

These elevated tumor markers usually seen in the urine of approx. 95% of neuroblastoma can help to confirm the diagnosis

Answer 12

catecholamine metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA)

Question 13

Neuroblastoma can be confirmed without primary tumor biopsy if

Answer 13

small round blue tumor cells are observed in bone marrow samples and urine VMA or HVA level are elevated

Question 14

It should be used when available to better define the extent of disease

Answer 14

Iodine-123 metaiodobenzylguanidine (123 I-MIBG)

Question 15

Staging of neuroblastoma: localized and confined to 1 body compartment without any image-defined risk factors (IDRFs)

Answer 15

L1 tumors

Question 16

Staging of neuroblastoma: refer to localized tumors with the presence of image-defined risk factors (IDRFs)

Answer 16

L2 tumors

Question 17

Staging of neuroblastoma: when there is disseminated tumors with metastases to bones, bone marrow, liver, distant lymph nodes and other organs

Answer 17

Stage M

Question 18

Staging of neuroblastoma: refers to neuroblastoma in children <18mo old with dissemination to liver, skin or bone marrow without bone involvement and with a primary tumor that would otherwise be stage as L1 or L2

Answer 18

Stage MS

Question 19

Treatment for children with low risk NB and for stages L1 and L2

Answer 19

Surgery

Question 20

Children with spinal cord compression at diagnosis of NB may require urgent treatment with

Answer 20

chemotherapy, surgery or radiation to avoid neurologic damage.