Flashcards in Neuroblastoma Deck (17):
What are cancer stem cells?
Name a specific marker
Subpopulation of cells within a tumor with the ability to self-renew, generate differentiated progeny and create heterogenous cancer population, with increased resistance to chemo and propensity to metastatize
Genetic disorders associated with NBL (6)
Noonan (PTPN11 mutations)
Neuro-cardio-facial syndrome, e.g. Costello syndrome
Simpson-Golabi-Behmel syndrome type 1
Germline mutations associated with hereditary NBL
PHOX2B (chr 4)
What is associated with heterozygous PHOX2B mutation?
Neuroblastoma, ganglioneuroblastoma, congenital central hypoventilation syndrome, Hisrchprung disease
Genetic aberrations seen in sporadic NBL? (4)
ALK amplification or mutation
Chromosomal anomalies seen in sporadic NBL? (6)
Chr 1p LOH
Chr 11q LOH
Chr 14q LOH
Chr 17q gain
VIP tumor secretion:
- What are the symptoms?
- What is the treatment?
- What is the prognosis?
Chronic watery diarrhea, failure to thrive
Surgical resection of primary tumor
What is the prognosis of Ospoclonus Myoclonus Ataxia syndrome?
70-80% with long-term neurological sequelae; immunosuppresive treatments useful fr acute symptoms but none associated with improved long-term outcome
What is ROHHAD?
Syndrome including: Rapid-onset obesity, hypothalamic dysfunction, hypoventilation and automonic dysregulation;
Thought to be paraneoplasic (1/3 associated with neural crest tumors)
Poor prognosis, at least 25% mortality 2nd to respiratory insufficiency
- What is their sensitivity?
- Technique used
- Role in NBL staging
Only recommended in cases in which primary tumor is not MIGB avid, or MIBG-avidity unknown (e.g. resected before MIBG)
Sensitive for bone mets but less specific
List differences between INSS and INRG staging system?
1. INRG uses radiologic factors rather than resectability
2. INRG doesn't include midline and LN status
3. INRG 4s includes a 18 months-old age cutoff and L2 tumors (INSS: 12 months, localized tumors)
List elements included in the INRG classification?
- INRG stage
- Tumor differenciation
- MYCN status
- 11q status
- Tumor ploidy
Stage 4S disease: who should be treated?
Leading causes of deaths (3)
If evidence of worsening organ function, respiratory compromise, and/or significant hepatomegaly
- Respiratory insuffiiciency
- Hepatic dysfunction
Radiation therapy in HR NBL:
Volume: pre-surgical volume (post-induction chemo)