Hema Onco Flashcards
MC extracranial solid tumor in children and the MC diagnosed malignancy in infants
Neuroblastoma
Median age of children at diagnosis of neuroblastoma is
22 mos
90% of cases of neuroblastoma are diagnosed by what age
5 yrs old
Massive involvement of the liver with metastatic disease, with or without respiratory distress in neuroblastoma
Pepper syndrome
Unilateral ptosis, myosis and anhidrosis associated with a thoracic or cervical primary tumor, these symptoms do not resolve with tumor resection
Horner syndrome
Is strongly associated with advanced tumor stage and poor outcomes in neuroblastoma
Amplification of MYCN
Confers better prognosis if the child is <18mo old at diagnosis and if amplification og MYCN is not present
Hyperdiploidy
Is MC in children < 1yr old at diagnosis, occurs through local invasion or distant hematogenous or lymphatic routes
Metastatic spread
MC site of metastasis of neuroblastoma
regional or distant lymph nodes, lone bones and skull, bone marrow, liver and skin
Neuroblastoma originating in the superior cervical ganglion can result in
Horner syndrome
Neuroblastoma can also be associated with a paraneoplastic syndrome of autoimmune origin termed
Opsoclonus-myoclonus-ataxia syndrome
These elevated tumor markers usually seen in the urine of approx. 95% of neuroblastoma can help to confirm the diagnosis
catecholamine metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA)
Neuroblastoma can be confirmed without primary tumor biopsy if
small round blue tumor cells are observed in bone marrow samples and urine VMA or HVA level are elevated
It should be used when available to better define the extent of disease
Iodine-123 metaiodobenzylguanidine (123 I-MIBG)
Staging of neuroblastoma: localized and confined to 1 body compartment without any image-defined risk factors (IDRFs)
L1 tumors
Staging of neuroblastoma: refer to localized tumors with the presence of image-defined risk factors (IDRFs)
L2 tumors
Staging of neuroblastoma: when there is disseminated tumors with metastases to bones, bone marrow, liver, distant lymph nodes and other organs
Stage M
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
Stage MS
Treatment for children with low risk NB and for stages L1 and L2
Surgery
Children with spinal cord compression at diagnosis of NB may require urgent treatment with
chemotherapy, surgery or radiation to avoid neurologic damage.
