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73 Cards in this Set
- Front
- Back
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How is neuroblastoma defined?
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A malignancy of the sympathetic nervous system
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Where does neuroblastoma develop from?
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Neural crest cells
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How is neuroblastoma usually identified upon assessment?
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A mass is found in the neck, chest, and/or abdomen, most arising from the adrenal gland
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What constitutes a neuroblastoma?
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Solid soft mass w/microscopic nests of small tumor cells separated by fibrovascular septa w/areas of hemorrhage, calcification, & necrosis
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What is a neuroblastoma cell composed of?
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Small round cells w/blue granules w/varied maturation
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What are the malignant forms of neuroblastoma?
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1. Neuroblastoma
2. Ganglioneuroblastoma |
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What type of neuroblastoma is the most mature/benign form w/out metastatic potential?
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Ganglioneuroma
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How does one differentiate neuroblastoma from other small, round, blue-cell tumors pathology?
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Using electron microscopy & immunohistochemistry Pseudorosettes can be seen in 15-50% of cases
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What are key factors in developing an appropriate treatment plan for a neuroblastoma diagnosis?
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Biology of each tumor
Age of child at diagnosis |
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What is N-myc amplification associated with for neuroblastoma patients?
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Advanced stages
Rapid tumor progression Poor prognosis |
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N-myc amplification refers to what?
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Less than 10 copies of the N-myc oncogene
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What is High-TrkA expression associated with?
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1. Favorable group biologically & clinically
2. Infants up to 1yr old 3. Stages 1,2, & 4S |
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What also may High-TrkA gene expression be associated with?
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Cell differentiation
Tumor regression |
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Then what does lack of TrkA expression and a high expression of the TrkB gene associated with?
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Unfavorable NB (stage 4)
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What chromosome deletions & gains have been linked to a loss of heterozygosity and of "neuroblastoma suppressor genes"?
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Deletion 1p and 11q
Gain of 17q |
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What has a more favorable prognosis hyperdiploidy or diploidy in Neuroblastoma tumor cytogenetics?
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Diploid (DNA index of 1) = more likely to have advanced disease & less favorable outcomes
*(Hyperdiploid = DNA index >1) |
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T or F: Accounting for approx. 8% of all childhood cancers, Neuroblastoma is the most common extracranial solid tumor in children.
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True
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What age group is it considered the most common malignancy?
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Infants = accounts for 50% of all malignancies in newborns
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What phenomenon occurs that make it difficult to assess incidence accurately?
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Spontaneous tumor regression and maturation
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What is the average age of diagnosis for Neuroblastomas?
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2 years old; 90% are diagnosed by age 5yrs;
Rare after 10yrs of age |
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What is the neuroblastoma tumors nickname and why?
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"the silent tumor" - b/c presents w/widespread metastatic disease at diagnosis in 60% of patients
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What other conditions/diseases are known to have an increased incidence of NB?
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1. Neurofibromatosis Type 1
2. Hirschsprung disease 3. Central hypoventilation (Ondine's curse) |
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What percentage of NB patients report a family history?
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1 to 2% - typically occurs sporadically
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T or F: The small population that reports familial history of NB have transmission through an autosomal recessive gene
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False: Autosomal Dominant Gene
*Incr'd incidence among siblings & identical twins |
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Are screening studies beneficial in reducing the incidence of late-appearing advanced-stage disease?
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No - 6mo or younger - screening studies were found to NOT reduce incidence
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Are signs & symptoms of NB always specific to tumor location and metastatic sites?
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No, they can be generalized - as tumors can occur anywhere along sympathetic nervous system.
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In more than 50% of NB cases, the primary site of disease arises from what gland?
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Adrenal Gland
(30% occur in the abdomen) (15% are thoracic in origin) |
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What location of a tumor is generally associated with localized disease?
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Thoracic primary tumor (Stages 1-3)
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An adrenal primary tumor is associated with what stage of disease?
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Stages 4 and 4S
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What sites are considered metastatic?
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- Lymph nodes
- Bone - Bone Marrow - Liver - Subcutaneous tissue |
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What are other sites NB can be located?
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Head
Posterior Mediastinum Pelvis Neck |
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In the rare occasion NB spreads to the lung and/or brain, what should be considered?
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Relapsing or End-stage disease
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What should one consider if a patient develops malaise, low-grade fever, or limp?
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Bone or bone marrow metastases
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What is a common cause of hypertension in a patient w/NB?
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Pressure from tumor on the renal artery, therefore activating the renin-angiotensin system
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What is being described when someone uses the term a "blueberry muffin" sign?
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A subcutaneous tumor = hardened bluish nodule seen or palpated
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What type of tumor is often mistaken for child abuse?
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Periorbital tumor that has caused proptosis and/or periorbital ecchymoses
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What location of tumor tends to form a dumbbell-shaped mass and causes cord compression?
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Intervertebral ganglion tumor that has grown into the intervertebral foramena
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If a patient develops paralysis, weakness in extremities, incontinence, and pain, what is happening?
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Cord Compression = Oncologic emergency
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What is Horner syndrome?
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Unilateral ptosis
Myosis anhydrosis |
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What location is a tumor if a patient develops Horner syndrome?
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High thoracic and cervical masses
2.4% of diagnoses patients |
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What unique paraneoplastic syndromes present at an early age & have been associated predominantly w/localized NB?
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Opsomyoclonus = myoclonic jerking and random eye movement
Cerebellar ataxia 1.3% of NB patients |
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What casues 5% of patients to have intractable diarrhea, resulting in hypoK+ & dehydration?
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Secretion of VIP (vasoactive intestinal polypeptide) by tumor
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Tumors that secrete VIP are mostly type?
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Mature histologically (Ganglioneuroblastoma or ganglioneuroma) = favorable outcomes
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At time of diagnoses, what urine test should be done prior to start of therapy?
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Urine catechloamines - increased in 90-95% of cases
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What percentage of NB patients have disease identified on BM biopsy?
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11-30% biopsies Positive when aspirates were negative
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Must biopsy the tumor to confirm diagnosis & what?
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Determine N-myc & ploidy status
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What scan is extremely useful in determining metastatic NB disease?
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MIBG (Metaiodobenzylguanidine)
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Then what is a PET/CT for?
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assessing metastatic disease not sensitive to MIBG
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What cooperative group has developed a universal staging system?
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International Neuroblastoma Staging System (INSS)
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Read page 34 for NB stages Table 2-9
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Stages 1, 2A, 2B, 3, 4, 4S
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What factors must one consider when evaluating prognosis for a NB patient?
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1. INSS stage
2. age at diagnosis 3. N-myc status 4. tumor histology/DNA ploidy |
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What stages are considered low risk NB?
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Stages 1 & 2
Stage 4S (younger than 1yr of age) Greater than 90% EFS |
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What stages are Intermediate Risk?
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Stage 3
Stage 4 (younger than 1year of age) Stage 4S w/large liver mets or diploid DNA index |
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What stages are High Risk?
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Stage 4 (older than 1yr of age)
N-myc amplification regardless of age or stage Overall 5-yr survival = 30-40% |
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What are the 7 treatments offered to varying degrees of NB diagnoses?
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1. Observation alone
2. Surgery 3. Chemo 4. Raidiation Therapy 5. BM w/HD chemo & SCR 6. Immunotherapy 7. Retinoid compounds |
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What is treatment based on for patients w/NB?
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Staging & risk factors at time of diagnosis
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What 4 phases of treatment do high-risk NB patients receive?
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1 - Induction chemo
2 - primary site, local control (surgery & radiation) 3 - high-dose marrow ablative therapy 4 - manage min residual disease |
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When is observation alone considered as a treatment option?
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Pt's <1year with stage 4S disease & favorable biology
- poss spontaneous regression |
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When is surgery alone a curative treatment?
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Low-stage disease (stage 1&2)
- offers 5yr EFS >90% |
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What is the common chemo regimen when treating intermediate-risk NB?
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Low dose chemo for 4-8 cycles
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What is the common chemo regimen when treating high-risk NB?
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High-dose induction chemo
includes - Cytoxan, Adraimycin, VCR, Cisplatin & VP16 |
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When can radiation therapy be used as treatment in NB?
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Administered to:
- primary site after surgical resection - metastatic sites that don't resolve w/chemo alone |
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What treatment is used in emergency situations such as proptosis or cord compression?
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Radiation before surgery to decrease surgical risks
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What chemo is typically used in a high-risk patients receiving a bone marrow transplant?
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Carboplatin
Etoposide Melphalan Cyclophosphamide Thiotepa |
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Is TBI always used as part of the treatment with HD chemo and BMT w/SCR?
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No
If it is used w/a tandem BMT, it is administered w/second treatment |
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When is immunotherapy especially considered as part of NB treatment?
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Minimal residual disease
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How does immunotherapy work in treating NB?
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monoclonal antibdy produced from WBC of mice, attaches to NB specific cell surface ganglioside GD2 & used w/cytokines
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What normal process does the monoclonal antibody signal to help treat NB?
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Own immune system attacks & kills the NB cell
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When is isotretinoin used in the treatment of NB?
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Standard of care for high-risk pt's w/MRD.
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How does isotretinoin (retinoid compound) treat MRD?
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Induces apoptosis & differentiation
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In low- or intermediate-risk disease w/local relapse what treatment option may be curative?
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Second surgery
(or w/ or w/out chemo &/or radiation) |
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Pt. w/high-risk relapse or recurrent disease what are some current approaches to treatment?
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1. Cytotoxic agents - Cytoxan, topotecan & irinotecan
2. Radionuclides - w/MIBG, somatostatin analogs radiation is delievered directly to NB cells 3. Retinoid compounds - fenretinide induces cytotoxicity in NB cells |
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What are the 4 goals of nursing assessment & intervention with a NB diagnosis?
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Pt. will experience minimal complications related to 1) onset of tumor; 2) treatment.
Pt. & family will 3)be adequately informed re:dx, disease & treatment; 4) cope adequately w/ the diagnosis. |
