Children's Neuroblastoma Cancer Foundation

CNCF 2008 - Cell and Vaccine Therapies for Neuroblastoma At Texas Children's Hospital

Mark Dungan (Sydney's Daddy) — Thu, 09 Oct 2008 19:28:00 GMT

Dr. Chrystal Louis from Texas Children's Cancer Center Center for Cell and Gene Therapy presents information regarding cell and vaccine therapies for neuroblastoma. This presentation was made at the Children's Neuroblastoma Cancer Foundation Neuroblastoma Conference on July 19, 2008

Download iPod or PSP version here.

Online Seminar - Preventing Hearing Loss

Mark Dungan (Sydney's Daddy) — Mon, 25 Aug 2008 11:46:00 GMT

Dr. David Freyer from Children's Hospital of Los Angeles is the study chair for the new COG study "Sodium Thiosulfate in Preventing Hearing Loss in Young Patients Receiving Cisplatin for Newly Diagnosed Germ Cell Tumor, Hepatoblastoma, Medulloblastoma, Neuroblastoma, or Osteosarcoma." The chemotherapy drug cisplatin is a very important part of treatment regimens used for a variety of childhood cancers, including high-risk neuroblastoma. Unfortunately, cisplatin frequently causes the...(read more)

We need your recipes!

Mark Dungan (Sydney's Daddy) — Mon, 18 Aug 2008 10:38:00 GMT

This year we will be compiling another cookbook to raise money for the CNCF (Children's Neuroblastoma Cancer Foundation) for much needed neuroblastoma research , education, awareness, support, and advocacy initiatives. The Lunch for Life Cookbook is a joint effort between Lunch for Life , The L.O.V.E. Club , and all of the families that have been touched by neuroblastoma . Several families have gotten together to work on this project to get it ready over the next month. We need favorite family...(read more)

The Neuroblastoma Handbook

Mark Dungan (Sydney's Daddy) — Fri, 01 Aug 2008 17:00:00 GMT

For those of you who were unable to attend the recent CNCF conference, we'd like to let you know that the NB Parent Handbook is now available on the CNCF website at www.nbhope.org/handbook. The Handbook has been prepared by NB parents, with the goal of sharing with others what they have learned during their children's NB treatments. The list of contributors is too numerous for me to thank individually ...(read more)

Caroline Pryce Walker Conquer Childhood Cancer Act

Mark Dungan (Sydney's Daddy) — Wed, 30 Jul 2008 11:12:00 GMT

Pryce applauded the signing of the legislation she authored titled the Caroline Pryce Walker Conquer Childhood Cancer Act. The statement said the legislation authorizes $150 million over five years for pediatric cancer research and outreach, and reaffirms our nation's commitment to eradicating the number one cause of death by disease for children. ...(read more)

Help Spread the Word about the NB Handbook

Mark Dungan (Sydney's Daddy) — Fri, 25 Jul 2008 17:00:00 GMT

We are asking everyone to help us spread the word. We are calling for a "bum rush" of all of the social media you can get your hands on, ultimately directing people to the CNCF's Neuroblastoma Handbook. We are asking you to blog about the handbook, write entries on your caringbridge sites, and to include links and badges on your websites. The idea is to create as much awareness as we possibly can about the handbook. Ideally we want it to be in every family's hands but we need your help to let everyone know that it exists. It isn't just for us but for all families both now and in the future who land in our footsteps....(read more)

Senate Appropriations Committee encourages FDA to prioritize review of treatments and clinical trials for high-risk neuroblastoma

Mark Dungan (Sydney's Daddy) — Wed, 23 Jul 2008 14:31:00 GMT

The Senate Appropriations Committee passed its 2009 funding bill for the FDA late last week (Senate Report 110-426). In the bill, they encourage FDA to prioritize review of new treatments and clinical trials for high-risk neuroblastoma and other pediatric cancers and report back to Congress on progress made. The House version of the bill includes a similar provision. ...(read more)

Autographed Guitar on ebay benefiting Neuroblastoma Research

Mark Dungan (Sydney's Daddy) — Tue, 22 Jul 2008 18:51:00 GMT

An online charity auction on ebay is benefiting the CNCF. This autographed guitar was donated in Caleb Larson's memory to benefit neuroblastoma research through the Children's Neuroblastoma Cancer Foundation. Donated in support of the 1st Annual Cowboyin' for a Cure in Graham, Texas, this guitar is signed by many of country music's greats. Pleas help spread the word about this auction and make sure to bid....(read more)

2008 NB Conference Program

Mark Dungan (Sydney's Daddy) — Wed, 09 Jul 2008 16:01:00 GMT

2008 NB CONFERENCE SCHEDULE Thursday, 7/17/08 Event Time Room Early Registration 6:00 pm - 9:00 pm REGENCY EFG Welcome Dinner Buffet (Everyone) 6:00 pm - 9:00 pm REGENCY EFG Friday, 7/18/08 Event Time Room Registration 7:00 am - 9:00 am REGENCY FOYER EFG Breakfast Buffet 7:00 am - 9:00 am REGENCY FOYER EFG General Session 9:00 am - 5:00 pm REGENCY EFG Morning Break (Adults only) 11:15 am - 11:30 am REGENCY FOYER EFG Kids Room 9:00 am - 5:00 pm THE TERRACE ROOM Angel Parent Session 9:00 am - 5:00...(read more)

Editors — Thu, 03 Jul 2008 19:43:00 GMT

Chapters [1] [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14]

Download entire Handbook in single PDF file: NB Handbook 7-2008

CNCF Handbook for Parents of Children with Neuroblastoma

Disclaimer

Acknowledgments

Navigating Neuroblastoma and This Handbook (Read This First!)

Chapter 1 Confronting the Diagnosis

What is Neuroblastoma?

101    NB: Description, Diagnosis, and Staging

102    NB: Tumor Pathology and Genetics

103    NB: Risk Assignment

104 Questions for Your Doctors

105 U.S. Neuroblastoma Specialists

106 What is a Clinical Trial?

107 The World of Hospitals

108 Patients' Rights & Responsibilities

109 Reaching Out and Accepting Help

Chapter 2 Understanding the Basics of Frontline Treatments

201 Overview of Low- and Intermediate-Risk Treatment

202 Overview of High-Risk Treatment

Induction Phase

    Chemotherapy

    Stem Cell Harvest

    Surgery

Consolidation Phase

    Stem cell transplant

    Radiation

Treating Minimal Residual Disease

    Accutane

    Antibodies

Chapter 3 Coping with Treatment: Side Effects, Comfort, and Safety

301 What is Palliative Care?

302 Getting Through Chemotherapy

303 Surviving Neutropenia

304 Special Issues with Stem Cell Transplant(s)

305 Surgery

306 Central Venous Lines: Broviacs, Hickmans, & Ports

307 Radiation: From Tattoos to Side Effects

308 Coping with ch14.18 Antibodies

309 Coping with 3F8 Antibodies

310 Coping with 8H9 Intrathecal Antibodies

311 Coping with Accutane

312 Coping with MIBG Treatment

313 Advocating for Your Child

314 Special Issues for Teenagers and Adults

Chapter 4 Getting Through Tests & Scans

Understanding the Purpose of the Test

401 Blood Tests:

CBC – Complete Blood Count

Liver & Kidney Function

Electrolytes (Chemistries)

Cultures, Drug Levels

403 Urine tests: HVA/VMA, other

404 Bone Marrow Biopsies/Aspirates

405 CT/CT-Assisted Biopsy

406 Bone Scan

407 MIBG I-123 and I-131

408 MRI

409 PET

410 Ultrasound

411 Cytogentics

412 Other Tests and Scans: Heart, Lung, Auditory, and more

Chapter 5 Reaching Remission (No Evidence of Disease)

501 What Does No Evidence of Disease Really Mean?

502 Eradicating Minimal Residual Disease

503 Short-term Side Effects

504 Follow-up Scans and Other Tests

505 Re-vaccination

506 Getting Your Treatment Summary

507 Returning to School and Life

Chapter 6 Living With Long-Term Survivorship Issues

601 Hearing Loss

602 Dental Care

603 Scoliosis

604 Neurocognitive Issues

605 Psychological Issues

606 Horner's Syndrome

607 Neuropathy

608 Joint and Bone Pain

609 Hair Loss

610 Growth

611 Heart Issues

612 Sexuality and Infertility

613 Secondary Cancers

Chapter 7 Treating Refractory NB

701 Treating Refractory NB

Chapter 8 Dealing with Relapse

801 Dealing with Relapse

What are the Symptoms of Relapse?

Relapse Treatment Rationale

Investigating Available Protocols

Enrolling in Clinical Trials

Maximizing Your Treatment Options

Weighing Quality of Life Issues

Reaching Second Remission (NED again)

Chapter 9 Managing Emotions

901 One Family's Insights

902 Parents Coping with Relapse

903 Informed Consent

904 Grooming a Pill Popper

Chapter 10 Keeping Records

1001 Why Keep Records?

1002 Diagnosis Information Chart

1003 Drug and Test Chart

1004 Monitoring Response to Treatment

1005 Daily Record Chart

1006 Treatment Summary

Chapter 11 Utilizing Complimentary & Alternative Medicine

1101 What is Complimentary, Alternative, and Integrative Medicine?

1102 Learning about CAM

1103 Precautions

Chapter 12 Turning to End of Life Care

1201 Turning to End of Life Care

How do I know when it is the time to stop treatment? To stop transfusions?

What do I consider in choosing whether to have my child on hospice at home or in the hospital?

What sort of care is available for my child on hospice? What do I need to know about hospice to get the best care?

How can I most effectively manage my child's pain?

How do I make the most of my child's time?

How do I talk to my child with neuroblastoma?

What do I tell the siblings of my NB child?

How to I get the support I need from family, friends & others?

What do I need to know about the dying process (since no one is telling me about this)?

What do I need to know about planning and making final arrangements?

How do I let go? What is my role in releasing my child to a journey I have not experienced?

What thoughts do you have about the time ahead without our children?

Chapter 13 Support Resources

1301 Directory of On-Line Resources

1302 Travel Guides to Selected Cities with Neuroblastoma Specialists

1303 Houston

1304 National Institutes of Health (Bethesda, Md.)

1305 New York City/MSK

1306 San Francisco/UCSF

1307 Philadelphia/CHOP

1308 Chicago/CMH

1309 Los Angeles/CHLA

1310 Boston/Children's and DFCI

1311 Burlington, Vermont
1312 Ann Arbor, Michigan

1313 Tips for Navigating the Insurance Maze

1314 Finding Other Families: Listservs and on-line communities

1315 Keeping Family and Friends Informed

1316 Reading List

Chapter 14 Neuroblastoma Terminology

1401 Common Abbreviations

1402 Glossary of Medical Terms

102 NB: Tumor Pathology and Genetics

Editors — Wed, 02 Jul 2008 21:43:00 GMT

Download printer-friendly version: 102 NB Tumor Pathology and Genetics

WHAT IS NEUROBLASTOMA?

Part 2: Tumor Pathology and Genetics

NB is sometimes referred to as a “heterogeneous” disease because of the wide range in its behavior in different children—some NB tumors go away on their own (regress), some mature into a benign growth (ganglioneuroblastoma), and some grow and spread rapidly. The pathologist’s job is to try to identify which type of NB tumor your child has. This information, together with the child’s age and the stage of his or her disease, is used to determine the degree of risk for the child’s specific situation. The resulting risk assignment enables the oncologist to prescribe the right treatment for your child’s disease—not too much, but not too little. The scientific information used to determine risk assignment for NB is very technical in nature; understandably, some parents are content just knowing their child’s risk assignment and the resulting treatment required. The various categories of “risk assignment” are described in Section 3 following. For those who wish to know more, this section summarizes the various types of pathology and genetic information used to determine the risk category of specific disease profiles. We have tried to define each of the scientific terms used in the criteria for risk assignment. NB risk assignment is dependent on four distinct factors:

age of the child;
stage of the disease;
pathology of the tumor; and
genetic make-up of the tumor.

Each factor has significance (favorable or unfavorable) but no factor alone can determine prognosis. The combination of this information dictates the risk group assigned and the treatment your child will receive. Since it takes some time to get the pathology and genetic analysis (sometimes referred to as “cytogenetics”) from the biopsy of the primary tumor, oncologists will often begin treating (surgery and/or chemo) very sick children before all of this information is obtained, rather than waiting for a final risk assignment, which can take a week or more.

Generally, the younger the patient is, the lower the risk assignment. For example, infants under 12 months with a certain pattern of metastasized disease are classified as low risk. Similarly, children aged 12-18 months with metastasized disease and other certain favorable tumor characteristics are classified as intermediate risk. In contrast, children over the age of 18 months with the same factors would be classified as high risk.

2. Stage.

In children over 18 months old, the stage of the disease is one of the most important determinants of the child's risk group. Generally, localized disease is better, since getting rid of systemic disease can be more difficult.

3. Pathology of the tumor.

Another type of information used to determine risk classification is the pathology of the tumor after a sample (biopsy) is obtained. The pathologist determines whether the tumor has favorable or unfavorable “histology”--an analysis based on what the primary tumor cell structure looks like to the pathologist’s eye and under a microscope. (Note that the “pathology,” “histology” and “biology” of the tumor are often used as interchangeable terms.) Determination of histology as favorable or unfavorable is based on two factors: tumor grade and MKI. Tumor Grade. NB is classified as one of the “peripheral neuroblastic tumors” or pNTs. Although they arise from the same tissue type, these tumors can behave very differently depending on the structure and genetics, and are graded accordingly. They range in character from highly malignant neuroblastoma to benign ganglioneuroma. Tumor grade is determined from a biopsy of the tumor obtained before treatment and based on the International Neuroblastoma Pathology Committee (INPC), also referred to as the “Shimada Classification,” developed in 1999[1] and revised in 2003.[2] The pathologist looks for the proportion of non-cancerous structural cells called “stroma” (also known as “Schwannian” cells) and the degree of maturity (differentiation) of neuroblastic cells, and assigns one of the following tumor grades:

NB (Neuroblastoma): stroma-poor, undifferentiated, poorly differentiated, or differentiating;
GNBi (Ganglioneuroblastoma intermixed): stroma-rich, intermixed with Neuroblasts;
GNBn (Ganglioneuroblastoma nodular): stroma-rich, nodules of neuroblasts; or
GN (Ganglioneuroma): stroma-dominant, benign.

Ganglioneuroma (GN) is a benign tumor. Ganglioneuroblastoma (GNB) is essentially a benign tumor with less than half cancerous cells mixed in (GNBi) or nodules (GNBn) of cancerous cells visible--this tumor “mix” is considered malignant. NB is similar, but has less than half the proportion of benign stroma present in GNB, or none at all, and so is a malignant tumor.

MKI. In addition to tumor grade (NB, GNBi, GNBn, GN), information about cell division and activity is determined, referred to as the mitosis-karyorrhexis index (MKI). Dividing cells (mitosis) and cells with nuclear fragmentation (karyorrhexis) are counted and the MKI determined as follows:

low MKI: Less than 2% (100 cells in a sample of 5000 tumor cells);
intermediate MKI: 2-4% (100-200 cells per 5000 tumor cells); or
high MKI: 4% or more (200 or more cells per 5000 tumor cells).

Histology. The above two factors--tumor grade and MKI--together with the child’s age, allow INPC classification into two groups: favorable histology (FH) and unfavorable histology (UH):[3]

Favorable Histology

all ganglioneuroma (GN) and ganglioneuroblastoma intermixed (GNBi);
ganglioneuroblastoma nodular (GNBn) with 50% or more Schwannian cells;
neuroblastoma (NB), under 18 months; or
neuroblastoma (NB), under 5 years old, differentiating, low MKI.

Unfavorable Histology

ganglioneuroblastoma nodular (GNBn) with less than 50% Schwannian cells;
neuroblastoma (NB), undifferentiated;
neuroblastoma (NB), high MKI;
neuroblastoma (NB), poorly differentiated or differentiating, intermediate MKI, over 18 months; or
neuroblastoma (NB), differentiating, low MKI, over 5 years old.

This complex scheme enables pathologists to decide if the tumor has favorable or unfavorable characteristics, but this information alone does not determine prognosis or treatment intensity. It is used along with age, stage, and genetics to assign risk for treatment purposes. The next section, “Risk Assignment,” shows that favorable or unfavorable histology makes a difference in risk assignment in some situations, such as stage 2 or 3 disease, but not in others, such as stage 1 and some stage 4 diagnoses.

4. Genetic Make-up of the Tumor.

NB tumors act differently depending on genetic information coded inside the nucleus of the tumor cell. A cell has very long chains (about 5 feet) of compacted DNA within its nucleus. The DNA is wound up into strands like coiled rope and packaged in the chromosomes. Each chromosome has two short “p arms” and two longer “q arms,” and hence is shaped like an X. Each place on a chromosome holds genetic information that pertains to the expression of a trait, and each piece of information on a chromosome is a gene. See illustrations below.

The presence or absence of certain genetic information in an NB tumor cell is another factor used to determine risk category. Two types of genetic information currently considered relevant are “ploidy” and “MYCN.”

Ploidy. As noted, all dividing cells share genetic information through chromosomes, the “package” for DNA. Two copies of the DNA is normal in healthy cells and is called “diploidy” or a DNA index of 1.0. Diploidy is a poor prognostic factor for neuroblastoma and indicates higher risk disease. Three copies of DNA (DNA index 1.5 or DNA index > 1) in an NB cell is favorable, and is called triploidy (or hyperdiploidy).

MYCN. Another genetic factor considered in risk assignment is MYCN (also written N-myc). MYCN is a type of oncogene--a gene with a DNA sequence that causes cancer. MYCN is unfavorable factor—when there are more than 10 copies present, the NB tumor is referred to as MYCN amplified. MYCN is commonly multiplied, by 100 times, and has been found as high as 700 times in an NB cell. About 20% of all NB cases have MYCN amplification. It is more common in widespread disease than localized tumors. MYCN amplification is found in less than 10% of stage 1 & 2 cases, in about 10% of stage 4s, and in about 30% of stage 3 & 4 cases. (Note: MYCN amplification is often found in other cancers such as retinoblastoma, medulloblastoma, and small-celllung cancer.)[5]

MYCN is an important prognostic factor. Younger children and children with lower stage disease will be treated as high-risk (see the following section on risk assignment) if their tumor is MYCN amplified, but MYCN does not necessarily contribute to a poorer prognosis in high-risk cases because one or more unfavorable characteristics are present in all high-risk cases.[6]

Other genetic and molecular factors. Other genetic variables believed to have prognostic value for NB have been identified, but not all are currently used in risk assignment. For example, tumor suppressor genes are believed to be associated with 1p and 3p chromosomes, and deletion of either in the NB is considered an unfavorable prognostic factor, as well as loss of 11q or gain of 17q.[7] Currently 1p and 11q are used to further define treatment for intermediate risk.[8] The INRG (International Neuroblastoma Risk Group) will include 11q status in the new risk assignment schema.[9]^,[10]

A host of potential prognostic factors have been studied over the past 25 years, and current consensus is that none add anything significant to current use of age, stage, pathology, ploidy, and MYCN amplification. For example, lack of “trkA” and “CD44” expression on the NB cell surface are also considered unfavorable, but not independently prognostic.[11] TrkA is a high-affinity nerve growth factor receptor, and CD44 is a cell surface glycoprotein (antigen) involved in cellular interactions and homing to bone marrow. Overexpression of CD44 has been noted in the growth and spread in different types of malignancies, such as lymphomas. In neuroblastoma, however, unfavorable tumors often have low CD44 expression.[12]

Summary

The information involved in the diagnosis, staging, and prognosis of a rare disease like NB can be overwhelming This section identifies and introduces important factors used to determine the most critical treatment issue: risk assignment, which dictates treatment intensity. The next section, “Risk Assignment,” explains how these pieces fit together to determine whether a child receives treatment for low, intermediate, or high risk NB. However your child’s oncologist is the definitive source for learning how all of this information relates to his or her particular case.

Please contact editors@nbhope.org with any comments

[1] Shimada H, Ambros IM, Dehner LP, et al (July 1999). "The International Neuroblastoma Pathology Classification (the Shimada system)". Cancer 86 (2): 364–72. PMID 10421273.

[2] Peuchmaur M, d'Amore ES, Joshi VV, et al (November 2003). "Revision of the International Neuroblastoma Pathology Classification: confirmation of favorable and unfavorable prognostic subsets in ganglioneuroblastoma, nodular". Cancer 98 (10): 2274–81.

[3] Peuchmaur M, d'Amore ES, Joshi VV, et al (November 2003). "Revision of the International Neuroblastoma Pathology Classification: confirmation of favorable and unfavorable prognostic subsets in ganglioneuroblastoma, nodular". Cancer 98 (10): 2274–81.

[4] Cheung & Cohn, Neuroblastoma, Springer (2005), p. 79

[5] Williamson,D et al “Relationship Between MYCN Copy Number and Expression in Rhabdomyosarcomas and Correlation With Adverse Prognosis in the Alveolar Subtype” Journal of Clinical Oncology, Vol 23, No 4 (February 1), 2005: pp. 880-888

[6] Cheung & Cohn, Neuroblastoma, Springer (2005), p. 80

[7] Stallings RL. Origin and functional significance of large-scale chromosomal imbalances in neuroblastoma. Cytogenet Genome Res. 2007;118(2-4):

110-5.

[8] National Cancer Institute, Phase III Study of Response- and Biology-Based Combination Chemotherapy and Surgery With or Without Isotretinoin in Young Patients With Intermediate-Risk Neuroblastoma, COG-ANBL053, opened 2007

[9] Friedman GK, Castleberry RP. Changing Trends of Research and Treatment in Infant Neuroblastoma Pediatr Blood Cancer 2007;49:1060–1065

[10] S. L. Cohn, W. B. London, T. Monclair, K. K. Matthay, P. F. Ambros, A. D. Pearson, for the INRG Working Group. “Update on the development of the international neuroblastoma risk group (INRG) classification schema” Journal of Clinical Oncology, 2007 ASCO Annual Meeting Proceedings Part I. Vol 25, No. 18S (June 20 Supplement), 2007: 9503

[11] Vasudevan SA, Nuchtern JG, Shohet JM. Gene profiling of high risk neuroblastoma. World J Surg. 2005 Mar;29(3):317-24. Review.

[12] Kramer K, Cheung NK, Gerald WL, LaQuaglia M, Kushner BH, LeClerc JM, LeSauter L, Saragovi HU. Correlation of MYCN amplification, Trk-A and CD44 expression with clinical stage in 250 patients with neuroblastoma. Eur J Cancer. 1997 Oct;33(12):2098-100.

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1401 Common Abbreviations

Editors — Sat, 21 Jun 2008 18:25:00 GMT

Quick Reference Chart of Abbreviations and Acronyms

Download printer-friendly version: 1401 Common Abbreviations

123-I MIBG	isotope of iodine with quick half-life (13 hours) connected to MIBG, used for scanning
131-I MIBG	isotope of iodine with longer half-life (8 days) connected to MIBG, used for scanning and therapy to kill NB
131I-3F8	“hot antibodies” used for radioimmunotherapy
1p, 3p, 4p, 11q, 17q	genetic aberrations in NB: unbalanced loss and/or gain of these chromosomal arms are subjects of research for prognostic significance
3891	randomized study published in 1999: compared results with and without transplant and accutane
3973	randomized study (closed 2006) to compare with and without purged stem cells
3F8	antibody for neuroblastoma at Sloan-Kettering
4HPR	fenretinide
8H9	antibody used at Sloan-Kettering especially for NB relapsed in CNS
ABMT	autologous bone marrow transplant
ABT-751	anti-angiogenesis drug in phase 1
ACS	American Cancer Society
ANBL0032	randomization study for ch14.18 antibody
ANC	absolute neutrophil count
ASCT	autologous stem cell transplant
BMT	bone marrow transplant (or blood and marrow transplant)
BSO	buthionine sulfoximine
BUN	blood urea nitrogen
BX or BMX	bone marrow biopsy
CAM	complementary and alternative medicine
CBC	complete blood count
CCG	Children’s Cancer Group
CD34+	antigen expressed on healthy blood-forming stem cell, used to count stem cell collection
CD44	cell surface glycoprotein, advanced neuroblastomas often have low CD44 expression
CEM	carboplatin, etoposide, melphalan
CEM-LI	carboplatin, etoposide, melphalan, and local irradiation
CEP-701	receptor tyrosine kinase inhibitor, lestaurtinib
CEV	carboplatin, etoposide, vincristine
ch14.18	chimeric anti-GD2 antibody (part human, part mouse)
CHLA	Children’s Hospital Los Angeles
CHOP	Children’s Hospital of Philadelphia
cisRA	13-cis retinoic acid, brand name Accutane, Amnesteem, Claravis
CMV	cytomegalovirus
CNCF	Children’s Neuroblastoma Cancer Foundation
CNS	central nervous system
COG	Children’s Oncology Group
COJEC	“Rapid COJEC” European protocol: cisplatin, vincristine, carboplatin, etoposide cyclophosphamide given in rapid delivery schedule (10 day cycles)
CPT-11	irinotecan
CR	complete response
CT	computerized tomography
CVL	central venous line, Broviac or Hickman
DFCI	Dana-Farber Cancer Institute
DMSO	preservative used in frozen stem cells
DNA index	ploidy, copies of DNA
DX	diagnosis, diagnosed
EFS	event free survival
EKG or ECG	electrocardiogram
ESIOP	European pediatric oncology study group
ESR	erythrocyte sedimentation rate (also called “sed rate”)
FISH	Fluorescent in situ hybridization
GCSF or G-CSF	granulocyte colony stimulating factor
GD2	antigen expressed on the surface of neuroblastoma
GM-CSF	granulocyte macrophage colony stimulating factor
GNB	ganglioneuroblastoma
GPOH	German pediatric oncology study group
HAMA	human anti-mouse antibodies
HLA	human leukocyte antigens
HSCT	hematopoietic stem cell transplant
hu14.18-IL2	humanized anti-GD2 antibody fused to interleukin-2
ICE	ifosfamide, carboplatin, etoposide
IL2	interleukin-2
INRG	International Neuroblastoma Risk Group
INSS	International Neuroblastoma Staging System
IORT	intraoperative radiation therapy
IRB	institutional review board
IV	intravenous
LD or LDH	lactate dehydrogenase
LI	local irradiation
LOH	loss of heterozygosity
LP	lumbar puncture
MIBG or mIBG	meta-iodobenzylguanidine
MKI	mitosis-karyorrhexis index
MRD	minimal residual disease
MRI	magnetic resonance imaging
MSKCC or MSK	Memorial Sloan-Kettering Cancer Center
MYCN	myc myelocytomatosis viral related oncogene, when amplified (more copies) unfavorable prognostic factor; same as N-myc
N9	Memorial Sloan-Kettering neuroblastoma protocol
NANT	New Approaches to Neuroblastoma Therapy
NB or NBL	neuroblastoma
NCI	National Cancer Institute
NED	no evidence of disease
N-MYC or N-myc	myc myelocytomatosis viral related oncogene, when amplified (more copies) unfavorable prognostic factor; same as MYCN
NP	nurse practitioner
NSAIDs	nonsteroidal anti-inflammatory drugs
NSE	neuron-specific enolase
OMS	opsoclonus-myoclonus syndrome
OS	overall survival
p53	tumor suppressor gene
PA	physician’s assistant
PBSCT	peripheral blood stem cell transplant
PD	progressive disease
PET	positron emission tomography
PET-CTPET/CT	positron emission tomography and computerized tomography performed at the same time; images fused
PFS	progression-free survival
pNTs	peripheral neuroblastic tumors
POG	Pediatric Oncology Group
PR	partial response
RA	retinoic acid
RT-PCR	reverse transcription-polymerase chain reaction
S-100	stain used to identify neuroblastoma in biopsies
SAHA	suberoylanilide hydroxamic acid
SCT	stem cell transplant
SD	stable disease
SIOP	Society International Oncology Pediatrics
SSKI	Lugol’s potassium iodine solution
TBI	total body irradiation
TMI	total marrow irradiation
TPN	total parenteral nutrition
trkA, trkB, trkC	tyrosine kinase family of neurotrophin receptors: high levels of trkA expression favorable, high levels trkB unfavorable, high level trkC favorable
TVD	topotecan, vincristine, and doxorubicin
VGPR	very good partial response
VOIT	temozolomide, oral irinotecan and vincristine
VP-16	etoposide
VZV	Varicella-zoster virus
WBC	white blood cells

Please contact editors@nbhope.org with any comments

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The more we learn the more things change

Mark Dungan (Sydney's Daddy) — Sat, 07 Jun 2008 12:36:00 GMT

One of the most interesting revelations of the 2008 ANR in Chiba, Japan from a parent of a child with neuroblastoma was the realization that the rules are always changing. I can not tell you how many times something I had previously accepted as fact was now coming into question. In the neuroblastoma research world the old adage "the more you learn the less you know" is more fact than fiction. Throughout the conference there were several examples of this type of revelation coming to fruition. None was more interesting than an abstract and presentation by Thorsten Simon from Children's Hospital, University of Cologne, Germany. ...(read more)

Reflections on the ANR

Mark Dungan (Sydney's Daddy) — Fri, 30 May 2008 15:10:00 GMT

As many parents know, the Advances in Neuroblastoma Research (ANR) Congress was held in Chiba, Japan during May 21-24, 2008. This was the first time it has been held in Asia. The ANR is held every two years in a new location throughout the world. In 2010 in will be held in Sweden. The ANR is an opportunity for neuroblastoma researchers and clinicians to meet, discuss and share the most current research in the world of neuroblastoma. During the 2008 ANR over 390 neuroblastoma research abstracts were submitted. All of them were reviewed and scored by at least 7 authorities. The event was attended by almost 500 researchers and clinicians from over 35 countries. The ANR is, by far, the world's largest neuroblastoma specific research conference and usually attracts all of the world's leading neuroblastoma researchers....(read more)

104 Questions for Your Child's Doctors at Diagnosis

Editors — Wed, 28 May 2008 14:24:00 GMT

Download printer-friendly version: 104 Questions for Doctors at Dx

You have the right to have all questions about your child’s diagnosis, treatment, prognosis, and other available treatments answered to your satisfaction, keeping in mind that many questions about neuroblastoma have no definitive answer. The questions below are some that we wish we had asked at the time of our child’s diagnosis, so that we would have been better informed in facing the situation. We hope these questions will help you get the information you need to understand better your child’s diagnosis and the treatment options.

Understanding Your Child’s Diagnosis

How long will it take you to determine my child's diagnosis?
What tests will be done to diagnose my child?
Will all tests be performed at this facility?
Are there tests to determine neuroblastoma diagnosis and prognosis that are not available at this center?
What are the results of my child’s tests and scans?
Where is my child’s cancer? Has my child’s cancer metastasized? To what extent?
What stage of disease does my child have and what risk assignment?
How do I get copies of my child’s test results?

Understanding Your Child’s Prognosis

What is the survival rate for children with my child’s stage of disease and risk assignment?
What are the prognostic factors for NB and how are they determined?
Which ones does my child have and what is their significance?
What is my child’s chance of surviving cancer-free for five years? For ten years?

Exploring Your Hospital’s Experience with Neuroblastoma

How many children have you/this hospital treated with my child’s stage of disease and risk assignment in the past year? In the past five years?
What cancer does each doctor in the department specialize in? What other cancers besides NB do they treat?
Which specific doctors at this center will treat my child? Which doctor is primarily responsible for my child’s care?
Do the same doctors care for my child in both the day clinic and the hospital?
Who will perform my child’s surgery? What types of surgery does this doctor perform? How many NB surgeries does this doctor perform per year?

Getting a Second Opinion

Are there NB experts in the country that I might consult? Who would you recommend?
Which centers in the country treat the most patients with NB?
Who are the leading NB surgeons in the country?

Exploring Treatment Options

What is the treatment for my child’s disease stage and risk assignment?
What are the realistic goals of this treatment?
How many children have been treated on this protocol?
How long has this treatment protocol been in use?
Is this treatment a clinical trial (open or closed)? What are the purposes of this trial? Is randomization involved? What specific rules or limitations do I need to know about this trial?
What is the survival rate of children treated on this protocol?
Are there other treatment options for neuroblastoma and where are they available?
How do the survival rates vary for the different treatments?
Will my child undergo a stem cell transplant? What type?
What transplant alternatives are offered elsewhere?
How does the regimen offered here differ from regimens at other centers? What are the risks and benefits of each?
Will stem cells be collected? When?
When will surgery be performed? How does this compare with other centers?
Do we have any choices about the treatment?
If this were your own child, what would you do and why?

Understanding the Demands of NB Treatment

How long does this treatment regimen generally take to complete?
Can you give me a detailed outline and schedule for my child’s treatment?
Realistically speaking, what are the caretaking demands of this treatment on parents?
How often will my child be hospitalized? How many days per week will we be in clinic?
Can we do any parts of this treatment at our local hospital? What are the risks of doing so?
How will my child feel during treatment?
Which treatments are done on an out-patient basis, and which on an in-patient basis?
How do I keep my child safe during treatment?

Learning About the Risks of Treatment

What are the risks and short-term side effects of this treatment?
What are the long-term side effects of this treatment?

Understanding Tests to Determine Response to Treatment

How often will my child’s response to treatment be evaluated?
Which tests will be used?
Where will these tests be done?
Do other cancer centers use different tests to determine a child’s response to NB treatment?

Finding Information and Support

Where can I find information to learn about NB?
Where can I find the scientific studies about this treatment?
Have you published any studies about this treatment?
Where can I find scientific studies about other available NB treatments?
What support services are available at this center for my child? For my family?
Who do I contact about insurance and payment issues?
Could I speak with the parent of a child who has completed this treatment?

Please contact editors@nbhope.org with any comments

Download printer-friendly version: 104 Questions for Doctors at Dx

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TABLE OF CONTENTS

Download entire Handbook in single PDF file: NB Handbook 7-2008

CNCF Handbook for Parents of Children with Neuroblastoma

Navigating Neuroblastoma and This Handbook (Read This First!)

Chapter 1 Confronting the Diagnosis

Chapter 2 Understanding the Basics of Frontline Treatments

Chapter 3 Coping with Treatment: Side Effects, Comfort, and Safety

Chapter 4 Getting Through Tests & Scans

Chapter 5 Reaching Remission (No Evidence of Disease)

Chapter 6 Living With Long-Term Survivorship Issues

Chapter 7 Treating Refractory NB

Chapter 8 Dealing with Relapse

Chapter 9 Managing Emotions

Chapter 10 Keeping Records

Chapter 11 Utilizing Complimentary & Alternative Medicine

Chapter 12 Turning to End of Life Care

Chapter 13 Support Resources

Chapter 14 Neuroblastoma Terminology

102 NB: Tumor Pathology and Genetics

Download printer-friendly version: 102 NB Tumor Pathology and Genetics

WHAT IS NEUROBLASTOMA?

Part 2: Tumor Pathology and Genetics

1401 Common Abbreviations

The more we learn the more things change

Reflections on the ANR

104 Questions for Your Child's Doctors at Diagnosis