Q2 – 61: Clinical and Laboratory Characteristics at Diagnosis

Questions 2 – 31: Specify the site(s) of primary tumor(s) at diagnosis

For each site, specify if the primary tumor was identified at this location at diagnosis. If the primary tumor was identified at the site, select Yes and report the number of tumors present.

If the primary tumor was located at site is not listed, indicate Yes for Other site, report the number of tumors present at diagnosis and specify the location.

If the location of primary tumor(s) at diagnosis is unknown, select No for each site, and report Yes for Location of primary tumor(s) unknown.

Question 32: Were metastases present at diagnosis?

Metastases can develop when cancer cells break away from the main tumor and enter the bloodstream or lymphatic system. Indicate if metastases were present at diagnosis.

The Unknown option should be used sparingly and only when no diagnostic information is available.

Questions 33 – 47: Specify the site(s) of metastases

For each site, specify if the site of metastatic neuroblastoma present at diagnosis.

If metastatic disease was present at a site not listed, report Yes for Other site and specify the site.

Questions 48 – 52: Specify radiographic tests used to evaluate the disease status at diagnosis

Radiologic assessments are imaging techniques used to assess disease at diagnosis, typically for lymphomas or solid tumors. A multimodal imaging approach is often taken when evaluating neuroblastoma. CT, MRI, scintigraphy, and skeletal surveys are commonly used.

For each radiologic assessment, indicate if any of the listed radiologic tests were performed to evaluate the disease status at diagnosis.

See below for definitions and examples of each method of assessment:

• CT scan: Uses computer processing to turn numerous x-rays taken from many different angles into cross-sectional images; these scans can be useful for identifying abnormal structural features or pathologically enlarged organs or tissues.
• Magnetic resonance imagining (MRI): Magnetic resonance imaging (MRI) is an imaging technique used to form pictures of the anatomy and the physiological processes of the body.
• I-meta-iodobenzylguanidine scan (MIBG): Uses radioactive tracers for imaging +/- tissue destruction. These scans are specific to adrenergic tissues, which are those nervous system tissues involved in epinephrine and norepinephrine production and reception.
• Skeletal survey: Or “bone survey”, is comprised of a series of x-rays taken to examine all the bones in the body.
• Technetium scan: Technetium (Tc-99m) is an isotope commonly used as a tracer in imaging scans to detect how certain parts of the body are functioning. Tc-99m scans are often used to detect a wide range of conditions such as tumors, heart disease, thyroid abnormalities and kidney conditions.
  

Question 53: Were any biopsies performed at diagnosis?

Indicate if any biopsies were performed at diagnosis or prior to the first treatment for neuroblastoma. If biopsies were not performed at diagnosis or prior to the first treatment or it is not known if one was performed, select No.

Questions 54 – 58: Specify the biopsy site(s) positive for neuroblastoma

For site, specify whether each biopsy was positive for neuroblastoma. If a biopsy site was tested and negative for neuroblastoma, the site was not biopsied, or unknown if biopsied, indicate No.

If a biopsy was positive for neuroblastoma at a site not listed, indicate Yes for Other site and specify.

Question 59: Specify the histologic findings by Shimada classification

The Shimada classification divides tumors into Stroma-rich and *Stroma-poo*r categories according to their organizational pattern. Stroma refers to Schwann cells associated with neuroblastoma cells.

Specify the Shimada classification for the positive biopsy(ies) specified above using the following below:

• Stroma-rich: Percentage of tumor stroma ≥ 50%
• Stroma-poor: Percentage of tumor stroma < 50%

This information is likely to be found in a histopathology report from a tissue biopsy; however, if the Shimada classification is unclear, seek physician clarification.

If the histologic findings by the Shimada classification are not known, select Not classified / unknown.

Question 60: Specify histology

Specify the histology of the stroma-rich biopsy as either Nodular or Well differentiated / intermixed. This classification is based on the morphology of immature elements and might be included in a histopathology report. However, clinician clarification may be necessary.

Question 61: Specify histology

Specify if histology of the stroma-poor biopsy is considered Favorable or Unfavorable. This classification is typically based on the recipient’s age at diagnosis, degree of maturation, and the mitosis-karyorrhexis index (count of cells undergoing mitosis or karyorrhexis) of the neuroblastic cells. This information might be included in a histopathology report; however, clinician input may be necessary if the histology is not documented within the biopsy report.

Section Updates

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