Questions 233 – 248: Were tumor marker analyses performed immediately prior to the preparative regimen?
Tumor markers, also known as biomarkers, are substances produced by cancer tissue or by the body in response to cancer at higher than normal levels. In certain situations, these substances can be used to detect and monitor disease due to their elevated presence in blood, urine, and/or tissue. The substances listed in questions 234–245 have been identified as potential tumor markers for neuroblastoma.
Indicate if tumor marker analyses were performed at the last evaluation prior to the preparative regimen / infusion. For each substance, indicate if tumor marker analyses were performed at the last evaluation prior to the preparative regimen. If the analysis was performed, select Known, specify the value. If the analysis was not performed or it is unknown if performed, select Not known.
- Homovanillic acid (HVA): Catecholamines (e.g. epinephrine/adrenaline) are secreted as hormones from the adrenal medulla. Neuroblastomas typically produce excessive levels of these catecholamines. After catecholamines are secreted they are broken down into metabolites including Homovanillic acid (HVA) and Vanillylmandelic acid (VMA). Both HVA and VMA are excreted in the urine. As a result, elevated levels of HVA and VMA detected by urinary analysis can be indicative of neuroblastoma.
- Neuron specific enolase (NSE): A glycolytic enzyme (enolase) specific to neuronal-type tissues. NSE may be excessively expressed by neuroblastomas and indicative of disease.
- Serum ferritin: Ferritin is a blood protein that contains iron. A ferritin level indicates how much iron a person’s body is storing. If the ferritin level is lower than normal, it indicates the body’s iron stores are low (iron deficiency). If the ferritin level is higher than normal it could indicate hemochromatosis, a condition that causes the body to store too much iron. Other causes of an elevated ferritin level include liver disease, acute and chronic inflammatory conditions, malignancy (neuroblastoma) to name a few.
- Vanillylmandelic acid (VMA): Both HVA and VMA are excreted in the urine. As a result, elevated levels of HVA and VMA detected by urinary analysis can be indicative of neuroblastoma.
- LDH: Lactate dehydrogenase is an enzyme found in the cytoplasm of almost all tissues, which converts L-lactate into pyruvate, or pyruvate into L-lactate depending on the oxygen level. For some diseases, high levels indicate active disease (e.g., lymphoma, multiple myeloma and neuroblastoma).
- Other tumor marker analysis: If testing for a tumor marker was performed at diagnosis and is not listed above, select Known, and specify the other tumor marker, value and units of measurements in questions 247 – 248. Examples of other testing can include Chromogranin A (CgA) or Neuropeptide Y (NpY).
Question 249: Specify the disease status immediately prior the preparative regimen
Indicate the disease status of Neuroblastoma at the last assessment prior to the start of the preparative regimen. Refer to the Neuroblastoma Response Criteria section of the Forms Instructions Manual for definitions of each response.
If the recipient was not evaluated at this time or the best response is unknown, report Not evaluable or Not tested / unknown, respectively.
Question 250: Specify the total number of complete remissions
Report the total number of complete remissions achieved, including the most recent CR from diagnosis until the start of the preparative regimen / infusion. If this is the report from a subsequent HCT, the number of complete remissions achieved should include all CR’s from the original diagnosis to the start of the most current preparative regimen / infusion.
Questions 251 – 268: Specify any known sites of disease immediately prior to the preparative regimen
Indicate Yes or No whether disease was identified at any of the anatomical locations listed in questions 251 – 266.
If disease was present in the recipient’s bone marrow immediately prior to the preparative regimen, report Yes and indicate if flow cytometry and immunofluorescence was performed to evaluate the bone marrow at the last evaluation prior to the start of the preparative regimen / infusion.
If a site was known to have disease at the time immediately prior to the start of the preparative regimen and is not listed as an option, select Yes, for “other site” (question 267) and specify the location of the other site.
Question 269: Specify the percent of cells positive for neuroblastoma
Report the percent of cells positive for neuroblastoma from the site(s) selected in questions 251 – 268. If multiple sites were selected, the percentage of positive cells identified in the bone marrow should be reported.
Question 270: Specify reason
If the pre-transplant disease status was Not evaluable, specify the reason why the pre-transplant disease status was not evaluated.
Question 271: Specify the date the disease status was determined
Enter the date of the most recent assessment of disease status prior to the start of the preparative regimen. The date reported should be that of the most disease-specific assessment within the pre-transplant work-up period (approximately 30 days). Clinical and hematologic assessments include pathological evaluation (e.g., bone marrow biopsy, tumor biopsy), radiographic examination (e.g., X-ray, CT scan, MRI scan, PET scan), and laboratory assessment (e.g., CBC, peripheral blood smear), in addition to clinician evaluation and physical examination. Enter the date the sample was collected for pathological and laboratory evaluations; enter the date the imaging took place for radiographic assessments.
If the exact date is not known, use the process for reporting partial or unknown dates as described in General Instructions, Guidelines for Completing Forms.
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Section Updates
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