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Encyclopedia > Neuroblastoma
Neuroblastoma, NOS
Classification & external resources
ICD-10 C74.9
ICD-9 194.0
ICD-O: M9500/3
OMIM 256700
DiseasesDB 8935
MedlinePlus 001408
eMedicine med/2836  ped/1570
MeSH D009447

Neuroblastoma is the most common extracranial solid cancer in infancy and childhood. It is a neuroendocrine tumor, arising from any neural crest element of the sympathetic nervous system. Close to 50 percent of neuroblastoma cases occur in children younger than two years old.[1] The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Cancer is a class of diseases or disorders characterized by uncontrolled division of cells and the ability of these to spread, either by direct growth into adjacent tissue through invasion, or by implantation into distant sites by metastasis (where cancer cells are transported through the bloodstream or lymphatic system). ... Neuroendocrine tumors, or more properly gastro-entero-pancreatic or gastroenteropancreatic neuroendocrine tumors (GEP-NETs), are cancers of the interface between the endocrine (hormonal) system and the nervous system. ... The neural crest, a component of the ectoderm, is one of several ridgelike clusters of cells found on either side of the neural tube in vertebrate embryos. ... Grays FIG. 838– The right sympathetic chain and its connections with the thoracic, abdominal, and pelvic plexuses. ...

Contents

Description

Neuroblastoma is a cancer of the sympathetic nervous system -- a nerve network that carries messages from the brain throughout the body. Its solid tumors, which take the form of a lump or mass, commonly begin in one of the adrenal glands, though they can also develop in nerve tissues in the neck, chest, abdomen, or pelvis.


The cause of neuroblastoma is unknown, though most physicians believe that it is an accidental cell growth that occurs during normal development of the adrenal glands.


Neuroblastoma is one of the rare human malignancies known to demonstrate spontaneous regression from an undifferentiated state to a completely benign cellular appearance. Look up Benign in Wiktionary, the free dictionary. ...

  • VIDEO - Neuroblastoma, Jeannie Yang, MD, gives an overview at the University of Wisconsin Department of Surgery Grand Rounds (2007)

Epidemiology

Neuroblastoma comprises 6-10% of all childhood cancers, and 15% of cancer deaths in children. The annual mortality rate is 10 per million children in the 0- to 4-year-old age group, and 4 per million in the 4- to 9-year old age group.[2]
The highest incidence is in the first year of life, and some cases are congenital. The age range is broad, including older children and adults, but less than 10% of cases occur in people older than 10 years of age.[3] A congenital disorder is a medical condition or defect that is present at or before birth (for example, congenital heart disease). ...


Etiology

The etiology of neuroblastoma is not well understood. Several risk factors have been proposed and are the subject of ongoing research. Due to characteristic early onset many studies have focussed on parental factors around conception and during gestation. Factors investigated have included occupation (i.e. exposure to chemicals in specific industries), smoking, alcohol consumption, use of medicinal drugs during pregnancy and birth factors, however results have been inconsistent.[4] This article is about the medical term. ... A sperm cell fertilizing an ovum This article is about reproduction in organisms. ... Gestation is the carrying of an embryo or fetus inside a female viviparous animal. ...


Other studies have examined possible links with atopy and exposure to infection early in life[5], use of hormones and fertility drugs[6] ,and maternal use of hair dye[7]. Eczema-a typical atopic manifestation Atopy (Greek ατοπία - placelessness) or atopic syndrome is an allergic hypersensitivity affecting parts of the body not in direct contact with the allergen. ... An infection is the detrimental colonization of a host organism by a foreign species. ...


Diagnosis

The first symptoms of neuroblastoma are often vague and may include fatigue and loss of appetite. Later symptoms depend on tumor locations. In the abdomen, a tumor may cause a swollen belly and constipation. A tumor in the chest may cause breathing problems. Tumors pressing on the spinal cord cause a feeling of weakness. A tumor in the head may cause the eyes to start to swell outwards and turn black due to the pressure from behind. Often because symptoms are so unclear, half of all neuroblastomas have already spread (metastasized) to other parts of the body by the time suspicions are raised and a diagnosis is made. Metastasis (Greek: change of the state) is the spread of cancer from its primary site to other places in the body. ...


The diagnosis is usually confirmed by a surgical pathologist, taking into account the clinical presentation, microscopic findings, and other laboratory tests. On microscopy, the tumor cells are typically described as small, round and blue, and rosette patterns (Homer-Wright pseudo-rosettes) may be seen. A variety of immunohistochemical stains are used by pathologists to distinguish neuroblastomas from histological mimics, such as rhabdomyosarcoma, Ewing's sarcoma, lymphoma and Wilms' tumor. In about 90% of cases of neuroblastoma, elevated levels of catecholamines or its metabolites are found in the urine or blood. Catecholamines and their metabolites include dopamine, homovanillic acid (HVA), and/or vanillylmandelic acid (VMA). Anatomic pathology is a medical specialty (a branch of pathology) that is concerned with the diagnosis of disease based on the gross, microscopic, and molecular examination of cells and tissues. ... Catecholamines are chemical compounds derived from the amino acid tyrosine that act as hormones or neurotransmitters. ... Dopamine is a phenethylamine naturally produced by the human body. ... Homovanillic acid (HOC6H3(OCH3)CH2COOH; synonyms: 3-Methoxy-4-hydroxyphenyl acetic acid; HVA; 4-Hydroxy-3-methoxy-benzeneacetic acid; 4-Hydroxy-3-methoxyphenylacetic acid; ) is a major catecholamine metabolite. ... Vanillyl mandelic acid (VMA) is a chemical end product of catecholamine metabolism. ...


Another way to detect neuroblastoma is the mIBG scan (meta-iodobenzylguanidine), but it doesn't diagnose the disease in 100% of the cases. It works like this: mIBG is taken up by sympathetic neurons, and is a functioning analog of the neurotransmitter norepinephrine. When it is radio-ionated with I-131 (a radioactive iodine), it is a very good radiopharmaceutical for diagnosis of this disease.


Identification

Other tumors also have similar origins and show a wide pattern of differentiation ranging from benign ganglioneuroma to partially differentiated ganglioneuroblastoma to highly malignant neuroblastoma. Tumor (American English) or tumour (British English) originally means swelling, and is sometimes still used with that meaning. ... Look up Benign in Wiktionary, the free dictionary. ... This article or section does not cite its references or sources. ...


The diagnosis is usually confirmed by a surgical pathologist, taking into account the clinical presentation, microscopic findings, and other laboratory tests. On microscopy, the tumor cells are typically described as small, round and blue, and rosette patterns (Homer-Wright pseudo-rosettes) may be seen. A variety of immunohistochemical stains are used by pathologists to distinguish neuroblastomas from histological mimics, such as rhabdomyosarcoma, Ewing's sarcoma, lymphoma and Wilms' tumor. The N-myc amplification is characteristic, and sometimes electron microscopy is also required. In February 2007, Althea Technologies announced the development of a molecular diagnostic capable of clearly differentiating various types of childhood cancers, developed in cooperation with the U.S. National Cancer Institute (NCI).[8]


Stages

Neuroblastoma is stratified according to its anatomical presence at diagnosis:

  • Stage 1: Localized tumor confined to the area of origin.
  • Stage 2A: Unilateral tumor with incomplete gross resection; identifiable ipsilateral and contralateral lymph node negative for tumor.
  • Stage 2B: Unilateral tumor with complete or incomplete gross resection; with ispilateral lymph node positive for tumor; identifiable contralateral lymph node negative for tumor.
  • Stage 3: Tumor infiltrating across midline with or without regional lymph node involvement; or unilateral tumor with contralateral lymph node involvement; or midline tumor with bilateral lymph node involvement.
  • Stage 4: Dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S.
  • Stage 4S: Localized primary tumor as defined in Stage 1 or 2, with dissemination limited to liver, skin, or bone marrow (less than 10 percent of nucleated bone marrow cells are tumors).

Screening

In a controlled study whose results were published in 2002, screening of all healthy infants' urine for markers of neuroblastoma was compared to deaths of infants whose urine was not tested. The overall death rate from the cancer was the same. Screening identified infants with harmless neuroblastomas that would have melted away without treatment; the infants were then subjected to surgery and chemotherapy.[9]


Treatment

Current

When the lesion is localized, it is generally curable. However, long-term survival for children with advanced disease is poor despite aggressive multimodality therapy. Skin lesions caused by Chickenpox A lesion is any abnormal tissue found on or in an organism, usually damaged by disease or trauma. ...


Recent biologic and genetic characteristics have been identified, which, when added to classic clinical staging, has allowed accurate patient assignment to risk groups so that treatment strategies can be more effectively undertaken.[10] These criteria include the age of the patient, extent of disease spread, microscopic appearance, and several other biological features, most importantly MYCN oncogene amplification, into low, intermediate, and high risk disease. The therapy for these different risk categories is very different. Low risk patients can frequently be observed without any treatment at all, while intermediate risk patients are treated with chemotherapy.[11] High-risk neuroblastoma is treated with intensive chemotherapy, surgery, radiation therapy, bone marrow / Hematopoietic stem cell transplantation and biological-based therapy with Cys-Retinoic acid (Accutane). With current treatments, patients with low and intermediate risk disease have an excellent prognosis with cure rates above 90%. In contrast, therapy for high-risk neuroblastoma results in cures only about 30% of the time. Hematopoietic stem cell transplantation (HSCT), of cells either derived from the bone marrow or peripheral blood, colloquially known as bone marrow transplantation is a medical procedure in the field of hematology and oncology that involves transplantation of hematopoietic stem cells (HSC). ...


Clinical trials for new treatments

In November 2006, DRAXIS Health received approval from the U.S. Food and Drug Administration (FDA) to run two clinical trials using radioactive Iobenguane I-131 Injection (I-131 MIBG) to treat high-risk neuroblastoma. Both will be coordinated by a group of 11 children’s hospitals and two universities in the United States known as the New Advances in Neuroblastoma Therapy (NANT) consortium, and are continuations of earlier NANT studies. The trials were expected to start in December 2006 or early 2007. [12]


In February 2007, a study in Sweden reported that a common painkiller, might inhibit the development of neuroblastoma and help make treatment of the disease more effective. Celecoxib, an analgesic, anti-inflammatory substance that works by inhibiting the effect of the inflammatory enzyme, Cox-2, and thus could affect neuroblastoma tumors, which depend on Cox-2 for their growth and proliferation. Clinical studies are now planned; research to date has been done only in animals and cell cultures. [13]


Post-treatment prognosis

After it is declared that the disease is gone, it often comes back. Further treatment is then required. This can be problematic because some treatments, such as chemotherapy, have cumulative effects and side-effects therefore can increase significantly if used again. Chemotherapy is the use of chemical substances to treat disease. ...


Intensive chemotherapy and radiation therapy have known long-term negative consequences. An estimated two of three survivors of childhood cancer will ultimately develop at least one chronic and sometimes life-threatening health problem within 20 to 30 years after the cancer diagnosis.[14][15] Clinac 2100 C100 accelerator Radiation therapy (or radiotherapy) is the medical use of ionizing radiation as part of cancer treatment to control malignant cells (not to be confused with radiology, the use of radiation in medical imaging and diagnosis). ...


References

  1. ^ Janet Sassi, "Cellular Communication: Unraveling the Secrets of Histone Proteins", Fordham University, February 16, 2007
  2. ^ Brodeur GM, Castleberry RP. Neuroblastoma. In: Pizzo PA, Poplack DG. Principles and practice of pediatric oncology, 3rd ed. 1997:761-797.
  3. ^ Franks LM, Bollen A, Seeger RC et al. Neuroblastoma in adults and adolescents: an indolent course with poor survival. Cancer 1997;79:2028-2035.
  4. ^ Olshan, AF, Bunin, GR (2000) Epidemiology of Neuroblastoma. In: Brodeur, GM, Sawada, T, Tsuchida, Y, Voute, PA eds. , Neuroblastoma, Elsevier, Amsterdam, pp 33-39. ISBN 044450222X [1]
  5. ^ Menegaux F,et al. "Day Care, Childhood Infections and Risk of Neuroblastoma", Am J Epidemiol 2004; 159:843-851.
  6. ^ Olshan AF,et al. "Hormone and Fertility Drug Use and the Risk of Neuroblastoma: A Report from the Children's Cancer Group and the Pediatric Oncology Group", Am J Epidemiol 1999;150:930-8.
  7. ^ ,McCall EE,et al. "Maternal hair dye use and risk of neuroblastoma in offspring", Cancer Causes and Control 2005; 16,6:743-8
  8. ^ "Althea Technologies Announces the Development of a Diagnostic Capable of Differentiating Multiple Forms of Childhood Cancer", press release, February 20, 2007
  9. ^ Darshak Sanghavi, "Screen Alert: How an Ounce of RX Prevention can Cause a Pound of Hurt", Slate magazine, November 28, 2006
  10. ^ Neuroblastoma: biological insights into a clinical enigma. Brodeur GM. Nature Reviews Cancer. 2003 Mar;3(3):203-16. PMID 12612655
  11. ^ Current aspects of biology, risk assessment, and treatment of neuroblastoma. Haase GM, Perez C, Atkinson JB. Semin Surg Oncol. 1999 Mar;16(2):91-104. PMID 9988866
  12. ^ "DRAXIS Radiopharmaceutical Unit Approved to Run 2 Clinical Trials to Treat Neuroblastoma", DRAXIS Health Inc. press release, November 22, 2006
  13. ^ "Painkiller Helps Against Child Cancer", medicalnewstoday.com, February 8, 2007, accessed March 8, 2007 (source apparently is a press release from the Karolinska Institutet in Sweden)
  14. ^ "Childhood Cancer Survivors Face Increased Sarcoma Risk", HealthDay News, February 21, 2007
  15. ^ Oeffinger et al., "Chronic Health Conditions in Adult Survivors of Childhood Cancer", New England Journal of Medicine, October 12, 2006

External links

mode=image_finder&action=search&srchstr=neuroblastoma&srch_type=any&labels=&details=-1&no_filter=-1&plane_id=&capt_id=&filter_m=&filter_o=&acr_pre=&filter_p=&acr_post=#top Medical Image Database] at Uniformed Services University of the Health Sciences The Open Directory Project (ODP), also known as dmoz (from , its original domain name), is a multilingual open content directory of World Wide Web links owned by Netscape that is constructed and maintained by a community of volunteer editors. ... The National Cancer Institute (NCI) is part of the United States Federal governments National Institutes of Health. ... The American Cancer Society (ACS) is a medical organization with a corporate attitude in the United States. ... The Collaborative Hypertext of Radiology (or CHORUS) is a reference database that is free of use. ... The National Cancer Institute (NCI) is part of the United States Federal governments National Institutes of Health. ... The original New York Cancer Hospital[1], first built between 1884 and 1886, now converted to luxury condominiums, at 455 Central Park West and 106th St. ... The Uniformed Services University of the Health Sciences (USU), established by the United States Congress in 1972 and graduating its first class in 1980, is a center for military medical education and research. ...


  Results from FactBites:
 
MedlinePlus Medical Encyclopedia: Neuroblastoma (571 words)
Neuroblastoma is a malignant (cancerous) tumor that develops from nerve tissue.
Neuroblastoma most commonly begins in the abdomen in the tissues of the adrenal gland, but it may also occur in other areas.
In very young children with neuroblastoma, the tumor may go away on its own, without any treatment, or the tissues of the tumor may mature and develop into a benign ganglioneuroma that can be surgically removed.
Neuroblastoma Treatment - National Cancer Institute (967 words)
Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord.
Neuroblastoma often begins in the nerve tissue of the adrenal glands.
Neuroblastoma may also begin in the chest, in nerve tissue near the spine in the neck, or in the spinal cord.
  More results at FactBites »

 
 

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