NBL Presentation - FINAL
Transcript of NBL Presentation - FINAL
![Page 1: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/1.jpg)
Neuroblastoma
Elizabeth RobertsData Coordinator
CIBMTR Data Managers Mentor
Elizabeth RobertsData Coordinator
CIBMTR Data Managers Mentor
Tandem Meeting February 18
![Page 2: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/2.jpg)
Objectives
• Know what neuroblastoma is, how it is diagnosed, and how it is treated
• Complete form 2026: Neuroblastoma Pre-HSCT Data
• Complete form 2126: Neuroblastoma Post-HSCT Data
![Page 3: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/3.jpg)
About My Center• Pediatric Center, performing
average of 100 transplants per year. First transplant performed in 1981
• Three Data Coordinators, specializing in disease-specific areas
• We use Epic for our electronic records
![Page 4: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/4.jpg)
What Is Neuroblastoma?
• Starts in very early forms of nerve cells (neuroblasts) of the sympathetic nervous system
• Neuro = nerves• Blastoma = cancer
![Page 5: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/5.jpg)
Common Neuroblastoma Sites
• Adrenal glands• Abdomen (non-
adrenal)• Chest• Pelvis• Neck
![Page 6: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/6.jpg)
Neuroblastoma Statistics
• 8-10% of all childhood cancers• ~8 children per million per year under
age of 15 diagnosed• Median age of diagnosis is 22 month• More than 95% of cases are detected
by 10 years of age
![Page 7: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/7.jpg)
How Is Neuroblastoma Diagnosed?
• Blood and urine catecholamine tests• Imaging tests• Biopsy of tumor site• Bone marrow aspiration and biopsy
![Page 8: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/8.jpg)
Catecholamine Tests
• Detected in more than 85% of patients with NBL
• Homovanillic Acid (HVA)• Vanillymandelic Acid (VMA)
![Page 9: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/9.jpg)
Imaging Tests
• CT scans – usually abdomen, pelvis, and chest
• MRI scans – usually brain and spine• MIBG scans – preferred by many
doctors as the standard test• PET scans
![Page 10: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/10.jpg)
Biopsy
• Tissue is often needed to obtain all data required for risk-group assignment and treatment stratification in COG clinical trials
![Page 11: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/11.jpg)
Bone Marrow Aspiration/Biopsy
• Determine extent of disease/staging
![Page 12: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/12.jpg)
Neuroblastoma Staging – INSS System
• Stage 1: Localized tumor with complete gross excision; lymph nodes negative for tumor microscopically
• Stage 2A: Localized tumor with incomplete resection; lymph nodes negative
• Stage 2B: Localized tumor with or without complete resection; ipsilateral lymph nodes positive
• Stage 3: Unresectable unilateral tumor infiltrating across the midline; or localized unilateral tumor with contralateral positive lymph nodes
• Stage 4: Disseminated disease (except 4S)• Stage 4S: Localized primary tumor with dissemination limited to
skin, liver and/or marrow (<10%) (infants < 1yr)
![Page 13: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/13.jpg)
Neuroblastoma and the MYCN Oncogene
• Amplification of MYCN occurs in ~20% of primary untreated NB tumors, and is associated with:– Advanced stage disease (Science, 1984)– Rapid tumor progression and poor
prognosis (N. Engl. J. Med., 1985)– High levels of MYCN expression (Science,
1984)
![Page 14: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/14.jpg)
What Therapies Do We Use to Treat Neuroblastoma?
• Surgery/biopsy• Chemotherapy• Second look surgery• ASCT• Radiation therapy• Cis-Retinoic therapy• Immunotherapy
![Page 15: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/15.jpg)
Surgery
• Remove as much tumor as possible• Dependent on proximity to vital
structures or blood vessels• Chemotherapy may be used to shrink
the tumor first
![Page 16: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/16.jpg)
Chemotherapy
• Commonly used when metastases involved
• May be before (neoadjuvant) or after (adjuvant) surgery
• Most common drugs: carboplatin (or cisplatin), cyclophosphamide, doxorubicin, and etoposide in cycles
![Page 17: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/17.jpg)
Stem Cell Transplant
• High-risk patients• High doses of chemotherapy are
effective in treating NBL, but also severely damage bone marrow
• Need to give stem cells to replace those that were lost
• Use patient’s own cells (autologous transplant)
![Page 18: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/18.jpg)
Radiation Therapy
• External Beam – Aimed at tumor only– Can be used to shrink tumor before
surgery– Sometimes part of treatment regiment
before stem cell transplant• MIBG Therapy – Delivers radiation
directly to tumor via blood stream
![Page 19: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/19.jpg)
Retinoid Therapy
• Related to Vitamin A• Differentiating Agents – Help cancer
cells differentiate into normal cells• Currently use 13-cis-retinoic acid
![Page 20: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/20.jpg)
Immunotherapy
• Typically used in high-risk patients• Helps immune system recognize and
destroy cancer cells more effectively• Monoclonal antibodies given with
cytokines
![Page 21: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/21.jpg)
Filling Out the Forms
• Form 2026 is for Pre-HSCT data• Form 2126 is for Post-HSCT data
![Page 22: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/22.jpg)
Form 2026: Clinical and Laboratory Characteristics at Diagnosis
• Diagnosis Date - Pathology
![Page 23: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/23.jpg)
Form 2026: Clinical and Laboratory Characteristics at Diagnosis
![Page 24: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/24.jpg)
Form 2026: Clinical and Laboratory Characteristics at Diagnosis
![Page 25: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/25.jpg)
Form 2026: Clinical and Laboratory Characteristics at Diagnosis
• Use pathology report for sites of primary disease
• Metastases• Radiographic tests – in Epic, Radiology
tab• Biopsies – Pathology tab• Shimada Classification – Pathology
report
![Page 26: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/26.jpg)
Form 2026: Laboratory Values at Diagnosis of Neuroblastoma
• HVA and VMA in Urine Chemistry. We send ours out
![Page 27: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/27.jpg)
Form 2026: Laboratory Values at Diagnosis of Neuroblastoma
• N-myc amplification - Supplementary pathology report
![Page 28: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/28.jpg)
Form 2026: Laboratory Values at Diagnosis of Neuroblastoma
![Page 29: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/29.jpg)
Form 2026: Laboratory Values at Diagnosis of Neuroblastoma
• Cytogenetic Analysis – Our center tends to not perform these on NBL patients
• INSS – most centers use this, but if yours uses POG or Evans, report those. Noted on supplemental report
![Page 30: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/30.jpg)
Form 2026: Laboratory Values at Diagnosis of Neuroblastoma
![Page 31: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/31.jpg)
Form 2026: Laboratory Values at Diagnosis of Neuroblastoma
• Surgery• Radiotherapy• Chemotherapy – Oncology road maps• Best Response – Pathology reports
![Page 32: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/32.jpg)
Form 2026: Line of Therapy
![Page 33: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/33.jpg)
Form 2026: Disease Status Immediately Prior to Preparative Regimen
![Page 34: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/34.jpg)
Form 2126: Disease Assessment at Time of Best Response to HSCT
• Planned Therapy• Radiotherapy – End of Treatment Note• Retinoids• Immunotherapy• Chemotherapy
![Page 35: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/35.jpg)
Form 2126: Disease Assessment at Time of Best Response to HSCT
![Page 36: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/36.jpg)
Form 2126: Disease Assessment at Time of Best Response to HSCT
![Page 37: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/37.jpg)
Form 2126: Disease Assessment at Time of Best Response to HSCT
![Page 38: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/38.jpg)
Form 2126: Disease Assessment at Time of Best Response to HSCT
![Page 39: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/39.jpg)
Form 2126: Disease Assessment at Time of Best Response to HSCT
• Recurrence/Progression – Pathology• Treatment for persistent, progressive, or
recurrent disease
![Page 40: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/40.jpg)
References• American Cancer Society. Cancer Facts and Figures 2015.
Atlanta, Ga: American Cancer Society; 2015.• Cheung NK, Dyer MA. Neuroblastoma: Developmental biology,
cancer genomics and immunotherapy. Nat Rev Cancer. 2013; 13:397-411.
• Grupp SA, Dvorak CC, Nieder ML, et.al. Children’s Oncology Group’s 2013 blueprint for research: Stem cell transplantation. Pediatr Blood Cancer. 2013; 60:1044-1047
• National Cancer Institute. Pysician Data Query. Neuroblastoma Treatment. 2013. Accessed at www.cancer.gove/cancertopics/pdq/treatment/neuroblastoma/healthprofessional on 2/4/16.
• Yu AL, Gilman AL, Ozkaynak MF, et al. Anti-GD2 antibody with GM-CSF, interleukin-2, and isotretinoin for neuroblastoma. N Engl J Med. 2010; 363:1324-1334.
![Page 41: NBL Presentation - FINAL](https://reader031.fdocuments.in/reader031/viewer/2022021811/620e173874228b0ca117478c/html5/thumbnails/41.jpg)
Questions?