Neuroblastoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Historical Perspective
In 1910, the term neuroblastoma was first used to describe the mass by Dr. James Homer Wright, an American patholgist at Massachusetts General Hospital.[1]
Classification
Neuroblastic tumors may be grouped according to International Neuroblastoma Pathology Classification (Shimada classification) into two groups which include a schwannian stroma rich group and a schwannian stroma poor group. Based on the degree of the cellular maturity and composition, neuroblastoma may be further classified into three subtypes according to the International Neuroblastoma Pathology Classification which include undifferentiated neruoblastoma, poorly differentiated neuroblastoma, and differentiating neuroblastoma.[2][3][4]
Pathophysiology
Neuroblastoma arises from neural crest cells, which are normally involved in the development of the sympathetic nervous system and adrenal glands. Neuroblastoma is frequently observed along the sympathetic nervous system structures. Genes involved in the pathogenesis of neuroblastoma include NBPF10 gene, KIF1B gene, and ALK gene. Neuroblastoma is associated with a number of syndromes that include neurofibromatosis type 1, Beckwith-Wiedemann syndrome, and DiGeorge syndrome. On gross pathology, a well defined, bulky, and tan colored mass is a characteristic finding of neuroblastoma. On microscopic histopathological analysis the presence of round blue cells separated by thin fibrous septa are characteristic findings of neuroblastoma.[5][1][6]
Causes
There are no known direct causes for neuroblastoma.[1]
Differentiating Multiple Myeloma from other Diseases
Intra-abdominal neuroblastoma must be differentiated from other diseases that cause abdominal swelling and constipation such as Wilms tumor and ganglioneuroma. Whereas intra-thoracic neuroblastoma must be differentiated from other diseases that cause shortness of breath and chronic cough such as intrathoracic lymphoma and extra lobar pulmonary sequestration.[5][1]
Epidemiology and Demographics
Neuroblastoma is the most common extracranial solid cancer in childhood and the most common cancer in infancy.[1] The overall incidence rate of neuroblastoma is approximately 4.9 per 1,000,000 individuals in the United States. The incidence of neuroblastoma decreases with age; the highest incidence is in the first year of life. Males are slightly more commonly affected with neuroblastoma than females. The male to female ratio is approximately 1.12 to 1. Neuroblastoma usually affects individuals of the Caucasian race. African American, Native Indian and Asian individuals are less likely to develop neuroblastoma.[7]
Risk Factors
There are no established risk factors for neuroblastoma.[1]
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for neuroblastoma.[8]
Natural History, Complications and Prognosis
Neuroblastoma patients may initially present with vague symptoms making the diagnosis of such patients difficult. If left untreated, patients with neuroblastoma may progress to develop fatigue, loss of appetite, joint pain, and fever. Complications of neuroblastoma may include treatment-resistant diarrhea, Horner's syndrome, and transverse myelopathy. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis of neuroblastoma is generally regarded as poor.[5][1][6][2]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 4 2015
- ↑ 2.0 2.1 Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015
- ↑ Neuroblastoma, Ganglioneuroblastoma and Ganglioneuroma. Stanford Medicine Surgical Pathology Criteria(2015) http://surgpathcriteria.stanford.edu/srbc/neuroblastoma-ganglioneuroblastoma-ganglioneuroma/ Accessed on October, 5 2015
- ↑ Shimada H, Umehara S, Monobe Y, Hachitanda Y, Nakagawa A, Goto S; et al. (2001). "International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group". Cancer. 92 (9): 2451–61. PMID 11745303.
- ↑ 5.0 5.1 5.2 Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015
- ↑ 6.0 6.1 Neuroblastoma Treatment for health professionals. National Cancer Institute (2015) http://www.cancer.gov/types/neuroblastoma/hp/neuroblastoma-treatment-pdq#link/_534_toc Accessed on October, 7 2015
- ↑ Navalkele P, O'Dorisio MS, O'Dorisio TM, Zamba GK, Lynch CF (2011). "Incidence, survival, and prevalence of neuroendocrine tumors versus neuroblastoma in children and young adults: nine standard SEER registries, 1975-2006". Pediatr Blood Cancer. 56 (1): 50–7. doi:10.1002/pbc.22559. PMC 4251713. PMID 21108439.
- ↑ Recommendations. US Preventive Services Task Force(2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=neuroblastoma Accessed on October, 5 2015