Neuroblastoma overview: Difference between revisions
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==Natural History, Complications and Prognosis== | ==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.<ref name="radio">Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015</ref><ref name="wiki">Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015</ref><ref name="gov"> 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</ref><ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref> | 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.<ref name="radio">Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015</ref><ref name="wiki">Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015</ref><ref name="gov"> 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</ref><ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref> | ||
==Diagnosis== | |||
===Staging=== | |||
The staging of neuroblastoma is based on the International Neuroblastoma Staging System (INSS). According to a newly proposed International Neuroblastoma Risk Group Staging System (INRGSS), there are four stages of neuroblastoma based on the extent of dissemination and image findings. According to the Children Oncology Group (COG) risk stratification system neuroblastoma patients are risk stratified into a low risk group, an intermediate risk group, and a high risk group based on the [[tumor]] INSS stage, the patient's age, tumor grade, and the amplification of ''MYCN'' gene.<ref name="wiki">Neuroblastoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015</ref><ref name="pmid22248965">{{cite journal| author=Davidoff AM| title=Neuroblastoma. | journal=Semin Pediatr Surg | year= 2012 | volume= 21 | issue= 1 | pages= 2-14 | pmid=22248965 | doi=10.1053/j.sempedsurg.2011.10.009 | pmc=PMC3261589 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22248965 }} </ref><ref name="pmid21736987">{{cite journal| author=Colon NC, Chung DH| title=Neuroblastoma. | journal=Adv Pediatr | year= 2011 | volume= 58 | issue= 1 | pages= 297-311 | pmid=21736987 | doi=10.1016/j.yapd.2011.03.011 | pmc=PMC3668791 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21736987 }} </ref><ref name="gov">Neuroblastoma treatment–for health professionals. National Cancer Institute (2015) http://www.cancer.gov/types/neuroblastoma/hp/neuroblastoma-treatment-pdq#section/_1 Accessed on October, 8 2015</ref> | |||
===History and Symptoms=== | |||
Symptoms of neuroblastoma include fever, abdominal distension, constipation, cough and weakness.<ref name="wiki"> Neuroblastoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 7 2015</ref><ref name="O"> Neuroblastoma Childhood: Symptoms and Signs. Cancer.net http://www.cancer.net/cancer-types/neuroblastoma-childhood/symptoms-and-signs Accessed on October, 7 2015</ref><ref name="pmid21736987">{{cite journal| author=Colon NC, Chung DH| title=Neuroblastoma. | journal=Adv Pediatr | year= 2011 | volume= 58 | issue= 1 | pages= 297-311 | pmid=21736987 | doi=10.1016/j.yapd.2011.03.011 | pmc=PMC3668791 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21736987 }} </ref> | |||
===Physical Examination=== | |||
A positive history of palpable [[abdominal mass]], [[fever]] and purple skin patches is suggestive of neuroblastoma.<ref name="wiki">Neuroblastoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 7 2015</ref><ref name="O"> Neuroblastoma Childhood: Symptoms and Signs. Cancer.net http://www.cancer.net/cancer-types/neuroblastoma-childhood/symptoms-and-signs Accessed on October, 7 2015</ref><ref name="gov"> 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</ref><ref name="pmid21736987">{{cite journal| author=Colon NC, Chung DH| title=Neuroblastoma. | journal=Adv Pediatr | year= 2011 | volume= 58 | issue= 1 | pages= 297-311 | pmid=21736987 | doi=10.1016/j.yapd.2011.03.011 | pmc=PMC3668791 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21736987 }} </ref> | |||
===Laboratory Findings=== | |||
An elevated levels of blood and urinary [[catecholamine]]s, [[vanillylmandelic acid]] (VMA), and [[homovanillic acid]] (HVA) are suggestive of neuroblastoma.<ref name="gov"> 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</ref><ref name="wiki">Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 5 2015</ref><ref name="pmid21736987">{{cite journal| author=Colon NC, Chung DH| title=Neuroblastoma. | journal=Adv Pediatr | year= 2011 | volume= 58 | issue= 1 | pages= 297-311 | pmid=21736987 | doi=10.1016/j.yapd.2011.03.011 | pmc=PMC3668791 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21736987 }} </ref> | |||
===X Ray=== | |||
On plain radiography neuroblastoma is characterized by an intraabdominal or intrathoracic soft tissue mass. Other findings of neuroblastoma on plain radiography include [[calcification]] and remodelling of surrounding bony structures.<ref name="radio">Neuroblastoma. Radiopaedia (2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 8 2015</ref> | |||
===CT=== | |||
CT scan is the investigation of choice for the diagnosis of neuroblastoma.<ref name="pmid21736987">{{cite journal| author=Colon NC, Chung DH| title=Neuroblastoma. | journal=Adv Pediatr | year= 2011 | volume= 58 | issue= 1 | pages= 297-311 | pmid=21736987 | doi=10.1016/j.yapd.2011.03.011 | pmc=PMC3668791 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21736987 }} </ref> On CT scan, neuroblastoma is characterized by a heterogeneous, calcified, and [[necrotic]] mass, that may encase the surrounding [[vessel]]s or invade the surrounding tissues.<ref name="radio">Neuroblastoma. Radiopaedia (2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 8 2015</ref> | |||
===MRI=== | |||
On a T1 weighted brain [[MRI]] image, neurblastoma is characterized by a hyperintense, heterogeneous, and necrotic mass.<ref name="radio">Neuroblastoma. Radiopaedia (2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 4 2015</ref> | |||
===Echocardiography or Ultrasound=== | |||
On ultrasound, neuroblastoma is characterized by a heterogeneous , vascular, and necrotic mass with localized areas of calcification.<ref name="radio">Neuroblastoma. Radiopaedia (2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 8 2015</ref> | |||
===Other Imaging Findings=== | |||
Other imaging studies for neuroblastoma include nuclear medicine studies such as fludeoxyglucose-18F positron emission tomography scan (18F-FDG PET) and metaiodobenzylguanidine (123I-MIBG) scintigraphy.<ref name="pmid19617326">{{cite journal| author=Sharp SE, Shulkin BL, Gelfand MJ, Salisbury S, Furman WL| title=123I-MIBG scintigraphy and 18F-FDG PET in neuroblastoma. | journal=J Nucl Med | year= 2009 | volume= 50 | issue= 8 | pages= 1237-43 | pmid=19617326 | doi=10.2967/jnumed.108.060467 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19617326 }} </ref> | |||
===Other Diagnostic Studies=== | |||
The definitive diagnosis of nueroblastoma is confirmed by a biopsy. Charecterstic findings for neuroblastoma on microscopic histopathological analysis can be found [[Neuroblastoma pathophysiology|'''here''']].<ref name="patho"> Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015</ref> | |||
==Treatment== | |||
==References== | ==References== | ||
Revision as of 17:43, 11 October 2015
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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]
Diagnosis
Staging
The staging of neuroblastoma is based on the International Neuroblastoma Staging System (INSS). According to a newly proposed International Neuroblastoma Risk Group Staging System (INRGSS), there are four stages of neuroblastoma based on the extent of dissemination and image findings. According to the Children Oncology Group (COG) risk stratification system neuroblastoma patients are risk stratified into a low risk group, an intermediate risk group, and a high risk group based on the tumor INSS stage, the patient's age, tumor grade, and the amplification of MYCN gene.[1][9][10][6]
History and Symptoms
Symptoms of neuroblastoma include fever, abdominal distension, constipation, cough and weakness.[1][11][10]
Physical Examination
A positive history of palpable abdominal mass, fever and purple skin patches is suggestive of neuroblastoma.[1][11][6][10]
Laboratory Findings
An elevated levels of blood and urinary catecholamines, vanillylmandelic acid (VMA), and homovanillic acid (HVA) are suggestive of neuroblastoma.[6][1][10]
X Ray
On plain radiography neuroblastoma is characterized by an intraabdominal or intrathoracic soft tissue mass. Other findings of neuroblastoma on plain radiography include calcification and remodelling of surrounding bony structures.[5]
CT
CT scan is the investigation of choice for the diagnosis of neuroblastoma.[10] On CT scan, neuroblastoma is characterized by a heterogeneous, calcified, and necrotic mass, that may encase the surrounding vessels or invade the surrounding tissues.[5]
MRI
On a T1 weighted brain MRI image, neurblastoma is characterized by a hyperintense, heterogeneous, and necrotic mass.[5]
Echocardiography or Ultrasound
On ultrasound, neuroblastoma is characterized by a heterogeneous , vascular, and necrotic mass with localized areas of calcification.[5]
Other Imaging Findings
Other imaging studies for neuroblastoma include nuclear medicine studies such as fludeoxyglucose-18F positron emission tomography scan (18F-FDG PET) and metaiodobenzylguanidine (123I-MIBG) scintigraphy.[12]
Other Diagnostic Studies
The definitive diagnosis of nueroblastoma is confirmed by a biopsy. Charecterstic findings for neuroblastoma on microscopic histopathological analysis can be found here.[2]
Treatment
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 Neuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Neuroblastoma Accessed on October, 4 2015
- ↑ 2.0 2.1 2.2 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 5.3 5.4 5.5 5.6 Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015
- ↑ 6.0 6.1 6.2 6.3 6.4 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
- ↑ Davidoff AM (2012). "Neuroblastoma". Semin Pediatr Surg. 21 (1): 2–14. doi:10.1053/j.sempedsurg.2011.10.009. PMC 3261589. PMID 22248965.
- ↑ 10.0 10.1 10.2 10.3 10.4 Colon NC, Chung DH (2011). "Neuroblastoma". Adv Pediatr. 58 (1): 297–311. doi:10.1016/j.yapd.2011.03.011. PMC 3668791. PMID 21736987.
- ↑ 11.0 11.1 Neuroblastoma Childhood: Symptoms and Signs. Cancer.net http://www.cancer.net/cancer-types/neuroblastoma-childhood/symptoms-and-signs Accessed on October, 7 2015
- ↑ Sharp SE, Shulkin BL, Gelfand MJ, Salisbury S, Furman WL (2009). "123I-MIBG scintigraphy and 18F-FDG PET in neuroblastoma". J Nucl Med. 50 (8): 1237–43. doi:10.2967/jnumed.108.060467. PMID 19617326.