Neuroblastoma

Neuroblastoma is a type of cancer that originates from the immature nerve cells. It can affect any part of the body that contains such type of nerve cells [1].

The disease is related to the following processes:  neoplastic 

Presentation

Fever and feeling of malaise are the primary signs of neuroblastoma. Children also complain of poor appetite and eventually undergo weight loss. In addition to these, there are several other symptoms of neuroblastoma that vary with the site of the cancer.

When the cancer has developed in the sympathetic nerves of abdomen, individuals experience abdominal pain, swelling in legs, diarrhea and presence of a mass which is not tender when touched. Individuals in whom the cancer has affected the chest suffer from wheezing, pain in the chest, drooping eye lids and difference in the pupil size.

Workup

Neuroblastoma is diagnosed by the following methods:

  • Physical examination: A preliminary thorough physical examination is carried out for studying the signs and symptoms of the disease.
  • Blood and urine tests: Complete blood count is performed which would reveal presence of anemia. In addition, urine analysis would show elevated levels of chemicals that are released by the cancer cells. Blood test to detect the levels of catecholamine hormone is also done.
  • Imaging studies: Several imaging studies are employed to locate the primary site of the tumor and also to analyze the extent of spread of tumor. Various imaging tests that are performed include bone scan, X-rays of the bone, CT and MRI scan of the chest and abdomen and X-ray of the chest. 
  • Biopsy: Biopsy helps in designing an individualized treatment plan [7]. Bone marrow biopsy is conducted to see whether the cancer has spread to the bone marrow.

Treatment

Treatment of neuroblastoma majorly depends on the location of the tumor and stage of the disease. In many cases, surgery is the treatment of choice and is often successful. This may be combined with chemotherapy and radiation therapy to arrest the growth of the cancer cells [8] [9].

Immunotherapy is also given to children with high risk tumors. In this method, the children are given drugs to stimulate their immune system. The efficacy of stem cell transplantation is still being studied, for children with high risk tumors [10].

Prognosis

The prognosis of neuroblastomas varies with the nature of tumor cells. In some cases, the tumor disappears without any treatment; whereas in some, the tumor is benign in nature known as ganglioneuroma. Such type of tumors can be surgically removed. However, in cases, when the tumor is malignant in nature, the cancer quickly spreads which makes the treatment process more difficult [6].

Etiology

The exact factors that trigger the development of these malignant tumor cells in the nerve cells of children are not clear. Heredity or environmental factors do not seem to have any role in the development of neuroblastoma [3].

Typically, the cancer begins with a genetic mutation that signals the cells to grow in an uncontrollable fashion. The resultant reaction of cell overgrowth is a malignant tumor. Neuroblastoma begins in the neuroblasts which are essentially immature nerve cells that are naturally made during the fetal growth. In many instances, these neuroblasts mature at birth, some which fail to mature either disappear or mature eventually as the child grows. In some cases, however, tumor develops in the neuroblasts causing neuroblastoma.

Epidemiology

Neuroblastoma affects majorly the pediatric population, exclusively those aged 10 years and below. Boys are slightly at an increased risk of contracting this disease condition than girls. It has been estimated that each year about 600 cases of neuroblastoma are diagnosed in the US. Such a type of cancer is more common in whites [4].

Statistics have also shown that about 36% cases of neuroblastoma are diagnosed in infants, 79% cases are diagnosed in children less than 4 years and 97% cases are diagnosed in children aged 10 years.

Sex distribution
Age distribution

Pathophysiology

Neuroblastoma is a type of cancer that affects the immature cells of the sympathetic nervous system. This type of nervous system is a vital part of autonomic nervous system which essentially governs the functioning of majority of the body organs. In majority of the cases, neuroblastomas begin in the adrenal glands, others in the sympathetic nerves present in the abdomen.

Several type of karyotopic abnormalities occur in neuroblastoma, but deletion of short arm of chromosome 1 is a major characteristic finding in the majority (70–80%) of cases. The sequence of events that cause such a type of phenomenon to occur is poorly understood [5].

Prevention

So far there are no established guidelines for preventing development of neuroblastoma. However, there are small pieces of evidence suggesting that intake of vitamins and folic acid supplements during pregnancy can prevent the onset of such type of cancer.

Summary

Neuroblastoma mostly strikes infants and children younger than 10 years. Older children are rarely affected by this disease. Neuroblastoma is characterized by development of small, round, blue colored tumor cells in the nerve tissues. It is the 4th most common type of cancer affecting children. It commonly originates in areas such as adrenal glands. However, in many instances it is also found in abdomen, neck, and chest and in the neighboring areas of the spine [2].

Patient Information

Definition

Neuroblastoma is a type of cancer that originates in the immature nerve cells. A cancer of this type mostly strikes the infant and children population. It is considered to be the 4th most common type of cancer after lymphoma, leukemia and tumors of central nervous system.

Cause

The exact cause behind development of malignant tumors in the nerve cells is yet not known. Genetic mutations in the immature nerve cells cause uncontrolled growth of cells that finally develop into malignant tumor cells.

Symptoms

Symptoms of neuroblastoma consist of fever, malaise, wheezing, development of non–tender mass beneath the skin, diarrhea, chest pain, swelling in legs, bone pain, unexplained weight loss, changes in appetite and development of protopsis.

Diagnosis

Diagnosis of neuroblastoma begins with a preliminary physical examination followed by blood and urine tests to detect levels of chemicals and hormones. In addition, imaging studies are also carried out to locate the site of the tumor cells. Biopsy of the tumor cells are also done to analyze its nature.

Treatment

In many cases, surgery is often successful in removing the malignant cells. However, other treatment methods such as chemotherapy, radiation therapy and immunotherapy are also employed.

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References

  1. Brodeur GM, Hogarty MD, Mosse YP, Maris JM. Neuroblastoma. In: Principles and Practice of Pediatric Oncology, Pizzo PA, Poplack DG (Eds), Lippincott Williams & Wilkins, Philadelphia 2011. p.886
  2. Golden CB, Feusner JH. Malignant abdominal masses in children: quick guide to evaluation and diagnosis. Pediatr Clin North Am 2002; 49:1369.
  3. Bunin GR, Ward E, Kramer S, et al. Neuroblastoma and parental occupation. Am J Epidemiol 1990; 131:776.
  4. Goodman MT, Gurney JG, Smith MA, Olshan AF. Sympathetic nervous system tumors. In: Cancer Incidence and Survival among Children and Adolescents: United States SEER Program, 1975-1995, Ries, LA, Smith, MA, Gurney, JG, et al (Eds), National Cancer Institute, Bethesda, MD 1999. p.35.
  5. Brodeur GM, Green AA, Hayes FA. Cytogenetic studies of primary human neuroblastomas. Prog Cancer Res Ther. 1980;12:73.
  6. D'Angio GJ, Evans AE, Koop CE. Special pattern of widespread neuroblastoma with a favourable prognosis. Lancet 1971; 1:1046.
  7. Buckley SE, Chittenden SJ, Saran FH, Meller ST, Flux GD. Whole-body dosimetry for individualized treatment planning of 131I-MIBG radionuclide therapy for neuroblastoma. J Nucl Med. Sep 2009;50(9):1518-24.
  8. Strother DR, London WB, Schmidt ML, et al. Outcome after surgery alone or with restricted use of chemotherapy for patients with low-risk neuroblastoma: results of Children's Oncology Group study P9641. J Clin Oncol 2012; 30:1842.
  9. Matthay KK, Sather HN, Seeger RC, et al. Excellent outcome of stage II neuroblastoma is independent of residual disease and radiation therapy. J Clin Oncol. Feb 1989;7(2):236-44
  10. Jennings RW, LaQuaglia MP, Leong K, et al. Fetal neuroblastoma: prenatal diagnosis and natural history. J Pediatr Surg 1993; 28:1168.

  • Granulocyte colony-stimulating factor does not decrease blood transfusion requirements during induction therapy for high-risk neuroblastoma: a randomized controlled - LM Wagner, CA Billups, WL Furman - Journal of clinical , 2004 - jco.ascopubs.org
  • Acquired segmental iris dilator muscle synkinesis due to deglutition - BI Boehme, MH Graef - Archives of ophthalmology, 1998 - Am Med Assoc
  • Contamination of peripheral blood stem cell harvests by circulating neuroblastoma cells - TJ Moss, DG Sanders, LC Lasky - , 1990 - bloodjournal.hematologylibrary.org
  • Antineuronal antibodies in patients with neuroblastoma and paraneoplastic opsoclonus-myoclonus - NL Antunes, Y Khakoo, KK Matthay - Journal of pediatric , 2000 - journals.lww.com
  • Combined radionuclide and ultrasonic assessment of upper abdominal masses in children - GF Gates, JH Miller - American Journal of Roentgenology, 1977 - Am Roentgen Ray Soc
  • A Phase I dose escalation of combination chemotherapy with granulocyte‐macrophage–colony stimulating factor in patients with neuroblastoma - MC Fernandez, MD Krailo, RR Gerbing, KK Matthay - Cancer, 2000 - Wiley Online Library
  • Catheter–related infections and thrombotic events in immunocompromised children by the use of vancomycin/ciprofloxacin/heparin flush solution: a randomized, - KJ Henrickson, RA Axtell, SM Hoover - Journal of Clinical , 2000 - jco.ascopubs.org
  • Consolidating the role of* I-MIBG-scintigraphy in childhood neuroblastoma: five years of clinical experience - A Jacobs, M Delree, B Desprechins, J Otten, A Ferster - Pediatric , 1990 - Springer
  • Anti-Hu antibody in a neuroblastoma-associated paraneoplastic syndrome - PG Fisher, DS Wechsler, HS Singer - Pediatric neurology, 1994 - Elsevier
  • Assessment of lymphadenopathy in children - CJ Twist, MP Link - Pediatric Clinics of North America, 2002 - mdconsult.com
  • Autonomic dysreflexia in a paraplegic man with catecholamine-secreting neuroblastoma - KC Wright, JC Agre, BC Wilson - Arch Phys Med , 1986 - ukpmc.ac.uk
  • A multigenic program mediating breast cancer metastasis to bone - Y Kang, PM Siegel, W Shu, M Drobnjak, SM Kakonen - Cancer cell, 2003 - ctb.pku.edu.cn
  • Catecholamines and diarrhea in ganglioneuroblastoma - GB STICKLER, GA HALLENBECK - Archives of Pediatrics , 1962 - Am Med Assoc
  • Catecholamine-secreting neuroblastoma in a 4-month-old infant: perioperative management - MR Hernandez, RC Shamberger - Journal of clinical , 2009 - Elsevier
  • Basic Neurosciences - T Pacholczyk, RD Blakely, SG Amara - Nature, 1991 - Am Neuropsych Assoc
  • Blepharoptosis - AR Pimentel de Figueiredo - Seminars in Ophthalmology, 2010 - informahealthcare.com
  • Cervical neuroblastoma - H Singh, C Mohan, NK Mohindroo - Indian Journal of , 2007 - Springer
  • Antibodies against a neuron-like (HTB-10 neuroblastoma) cell in children with Tourette syndrome - HS Singer, JD Giuliano, BH Hansen, JJ Hallett - Biological , 1999 - Elsevier
  • Chronic syndrome of inappropriate antidiuretic hormone secretion and hypertension in a patient with olfactory neuroblastoma: evidence of ectopic production of - J Osterman, A Calhoun, M Dunham - Archives of internal , 1986 - Am Med Assoc
  • Cervical neuroblastoma in eleven infants—a tumor with favorable prognosis - SJ Abramson, WE Berdon, C Ruzal-Shapiro, C Stolar - Pediatric , 1993 - Springer
  • A restraint and transillumination device for neonatal arterial/venipuncture: efficacy and thermal safety - JS Curran, W Ruge - Pediatrics, 1980 - Am Acad Pediatrics
  • Are solid tumours different in children with Down's syndrome? - D Satgé, AJ Sasco, B Lacour - International journal of cancer, 2003 - Wiley Online Library
  • Abdominal masses in the newborn infant - LA Longino, LW Martin - Pediatrics, 1958 - Am Acad Pediatrics
  • Congenital cervical neuroblastoma associated with Horner syndrome - S Ogita, K Tokiwa, T Takahashi, S Imashuku - Journal of pediatric , 1988 - Elsevier
  • A Phase I/IB trial of murine monoclonal anti‐GD2 antibody 14. G2a plus interleukin‐2 in children with refractory neuroblastoma - JD Frost, JA Hank, GH Reaman, S Frierdich - Cancer, 2000 - Wiley Online Library
  • 3F8 monoclonal antibody treatment of patients with stage 4 neuroblastoma: a phase II study. - NK Cheung, BH Kushner, SD Yeh - International journal of , 1998 - ukpmc.ac.uk
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