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20 Cards in this Set

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1. Paraneoplastic Syndrome-presentation and pathophys?
a. HTN and Secretory diarrhoea.
b. Related to tumour production of catecholamines and vasoactive intestinal peptide.
2. Opsoclonus-myoclonus syndrome?
a. Characterized by chaotic eye movements and myoclonic jerks.
b. Described as “dancing eyes, dancing feet”
c. Related to autoantibodies produced against neuronal elements.
3. Neuroblastoma?
a. Comprised of primitive neuroendocrine tissue.
b. Its aetiology is poorly understood, but believed to be multifactorial.
c. It is the most prevalent solid, extracranial tumour in children and accounts for more than half of all cancers in infancy.
4. Where do most Neuroblastomas arise?
a. In the abdomen from the adrenal gland
b. Other origins:
i. Intrathoracic and paraspinal neuronal ganglia.
5. S/S of Neuroblastoma?
a. Depend on tumour location.
b. Cervical ganglia tumours may cause Horner’s syndrome.
c. Intrathoracic tumours (most commonly seen in infancy) may be associated w/wheezing and respiratory distress.
d. Paraspinal tumours: May cause compressive neuralgias, back pain, and urinary retention or stool retention.
e. Abdominal masses are typically non-tender, irregular, and cross the midline.
6. What does Metastatic disease with Neuroblastoma typically involve?
1. Skin
2. Lungs
3. Liver
4. Bone
7. Skin involvement w/neuroblastoma?
a. Bluish skin discolouration (most often seen in infancy) represents subcutaneous infiltration.
8. Lung Involvement w/neuroblastoma?
a. Pulmonary involvement can promote increased work of breathing, dyspnea, and pneumonia.
9. Bone marrow involvement w/neuroblastoma?
a. Bone pain and pancytopenia
b. Petechiae, bruising, pallor, fatigue may occur.
c. If orbital bones are involved: Proptosis and bluish periorbital discolouration “raccoon eyes”.
d. Generalized lymphadenopathy is also common.
10. 2 Conditions which may also occur as a result of neuroblastoma?
a. Paraneoplastic syndrome related to tumour neuroendocrine mediators.
b. Opsoclonus-myoclonus syndrome (autoimmune-mediated phenomenon that may be characterized by cerebellar ataxia).
11. The major differential diagnostic consideration w/neuroblastoma in children?
a. Wilm’s tumour.
12. Presentation of Wilm’s tumour?
a. Haematuria
b. HTN
c. Localized abdominal mass that rarely crosses the midline.
d. In general, pts w/neuroblastoma are slightly younger and sicker than pts w/Wilms tumour.
13. Tests for assessing the extent of Neuroblastoma?
a. CT or MRI.
14. Lab tests for assessing the extent of Neuroblastoma?
a. Elevated vanillymandelic acid and homovanillic acid levels (catecholamine metabolites).
b. Seen in 90% of neuroblastoma pts.
15. Other markers for Neuroblastoma?
a. Elevated Enolase
b. Ferritin
c. Lactate Dehydrogenase.
16. Tx of Neuroblastoma?
a. Surgical excision of the tumour, usually after chemo and/or radiation therapy to decrease tumour size.
b. Combined multi-agent chemo and radiotherapy often is used in pts w/advanced-stage neuroblastoma.
17. Staging of Neuroblastoma?
a. Classically dependent on tumour location and extent, w/risk assessment and therapeutic decision-making based on variable such as age at diagnosis and staging (eg, stage 2 disease localized to the abdomen of a 1-yr old requiring only limited post-excision chemo vs. stage 4 disease w/bony mets in a toddler mandating multi-agent chemo and bone marrow transplantation.
18. Cure rates for Neuroblastoma?
a. >90%. w/infants typically having better prognosis than older children.
19. What features of neuroblastoma denote a poor prognosis?
a. Skeletal mets.
b. N-myc oncogene amplification at the cellular level.
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