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__NOTOC__
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{{Neuroblastoma}}
{{Neuroblastoma}}
{{CMG}}
{{CMG}} {{AE}}{{ZAS}}{{HL}}
==Overview==
Neuroblastoma may progress to develop [[fatigue]], [[loss of appetite]], [[joint pain]] and [[fever]] if left untreated. There is gradual [[development]] of site specific [[Symptom|symptoms]] as the [[tumor]] size gradually increases. [[Complication (medicine)|Complications]] of neuroblastoma include persistent refractory [[diarrhea]], [[horner's syndrome]], [[hypertension]], [[Transverse myelitis|transverse myelopathy]], [[anemia]] and [[Immunodeficiency|suppressed immunity]]. The [[prognosis]] of neuroblastoma is generally regarded as poor, depending on the [[tumor]] extent at the time of [[diagnosis]]. The other [[Prognosis|prognostic]] factors for nuroblastoma include patient's [[age]], [[tumor]] stage and grade, [[genetic mutations]] and response to treatment.
 
==Natural History==
* Neuroblastoma [[Patient|patients]] may initially present with vague [[Symptom|symptoms]] making the [[diagnosis]] of such [[Patient|patients]] difficult.<ref>{{Cite journal
| author = [[L. M. Wilson]] & [[G. J. Draper]]
| title = Neuroblastoma, its natural history and prognosis: a study of 487 cases
| journal = [[British medical journal]]
| volume = 3
| issue = 5926
| pages = 301–307
| year = 1974
| month = August
| pmid = 4414795
}}</ref><ref>{{Cite journal
| author = [[Somruetai Shuangshoti]], [[Shanop Shuangshoti]], [[Issarang Nuchprayoon]], [[Somjai Kanjanapongkul]], [[Paula Marrano]], [[Meredith S. Irwin]] & [[Paul S. Thorner]]
| title = Natural course of low risk neuroblastoma
| journal = [[Pediatric blood & cancer]]
| volume = 58
| issue = 5
| pages = 690–694
| year = 2012
| month = May
| doi = 10.1002/pbc.23325
| pmid = 21922650
}}</ref>
* If left untreated, [[Patient|patients]] with neuroblastoma may progress to develop [[fatigue]], [[loss of appetite]], [[joint pain]], and [[fever]].
* Gradual development of site specific [[Symptom|symptoms]] may occur as the [[tumor]] size gradually increases.<ref>{{Cite journal
| author = [[C. S. Alvarado]], [[W. B. London]], [[A. T. Look]], [[G. M. Brodeur]], [[D. H. Altmiller]], [[P. S. Thorner]], [[V. V. Joshi]], [[S. T. Rowe]], [[M. B. Nash]], [[E. I. Smith]], [[R. P. Castleberry]] & [[S. L. Cohn]]
| title = Natural history and biology of stage A neuroblastoma: a Pediatric Oncology Group Study
| journal = [[Journal of pediatric hematology/oncology]]
| volume = 22
| issue = 3
| pages = 197–205
| year = 2000
| month = May-June
| pmid = 10864050
}}</ref>
 
==Complications==
==Complications==
*Spread (metastasis) of the tumor
* [[Complication (medicine)|Complications]] of neuroblastoma may include:<ref>{{Cite journal
*Damage and loss of function of involved organ(s)
| author = [[Nadja C. Colon]] & [[Dai H. Chung]]
**Kidney failure
| title = Neuroblastoma
**Liver failure
| journal = [[Advances in pediatrics]]
**Loss of blood cells produced by the bone marrow
| volume = 58
**Decreased resistance to infection
| issue = 1
**Other organ system failures
| pages = 297–311
| year = 2011
| month =
| doi = 10.1016/j.yapd.2011.03.011
| pmid = 21736987
}}</ref><ref>{{Cite journal
| author = [[John M. Maris]]
| title = Recent advances in neuroblastoma
| journal = [[The New England journal of medicine]]
| volume = 362
| issue = 23
| pages = 2202–2211
| year = 2010
| month = June
| doi = 10.1056/NEJMra0804577
| pmid = 20558371
}}</ref><ref>{{Cite journal
| author = [[Michal Sieniawski]], [[Jeremy Franklin]], [[Lucia Nogova]], [[Jan-Peter Glossmann]], [[Thomas Schober]], [[Hiltrud Nisters-Backes]], [[Volker Diehl]] & [[Andreas Josting]]
| title = Outcome of patients experiencing progression or relapse after primary treatment with two cycles of chemotherapy and radiotherapy for early-stage favorable Hodgkin's lymphoma
| journal = [[Journal of clinical oncology : official journal of the American Society of Clinical Oncology]]
| volume = 25
| issue = 15
| pages = 2000–2005
| year = 2007
| month = May
| doi = 10.1200/JCO.2006.10.1386
| pmid = 17420510
}}</ref>
:* Persistent refractory [[diarrhea]] (due to [[Vasoactive intestinal peptide|vasoactive intestinal polypeptide]] secretion)
:* [[Horner's syndrome]] (due to [[cervical sympathetic chain]] compression)
:* [[Opsoclonus myoclonus syndrome]] and [[ataxia]] (due to a [[paraneoplastic syndrome]])
:* [[Hypertension]] (due to either [[catecholamine]] secretion or [[renal artery]] compression)
:* [[Transverse myelitis|Transverse myelopathy]] (due to [[tumor]] [[spinal cord]] compression)
:* [[Anemia]] and [[Immunodeficiency|suppressed immunity]] (due to [[bone marrow]] [[metastasis]])
 
==Prognosis==
==Prognosis==
The prognosis of neuroblastoma is poor and it depends on the following:
* 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>{{Cite journal
:*Whether or not the tumor can be removed by surgery.
| author = [[Julie R. Park]], [[Angelika Eggert]] & [[Huib Caron]]
:*The stage of the cancer: the size of the tumor, whether the cancer has spread
| title = Neuroblastoma: biology, prognosis, and treatment
:*The child’s general health
| journal = [[Pediatric clinics of North America]]
=== Post-treatment prognosis ===
| volume = 55
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.
| issue = 1
| pages = 97–120
| year = 2008
| month = February
| doi = 10.1016/j.pcl.2007.10.014
| pmid = 18242317
}}</ref><ref>{{Cite journal
| author = [[L. M. Wilson]] & [[G. J. Draper]]
| title = Neuroblastoma, its natural history and prognosis: a study of 487 cases
| journal = [[British medical journal]]
| volume = 3
| issue = 5926
| pages = 301–307
| year = 1974
| month = August
| pmid = 4414795
}}</ref><ref>{{Cite journal
| author = [[Maya Suzuki]], [[Brian H. Kushner]], [[Kim Kramer]], [[Ellen M. Basu]], [[Stephen S. Roberts]], [[William J. Hammond]], [[Michael P. LaQuaglia]], [[Suzanne L. Wolden]], [[Nai-Kong V. Cheung]] & [[Shakeel Modak]]
| title = Treatment and outcome of adult-onset neuroblastoma
| journal = [[International journal of cancer]]
| volume = 143
| issue = 5
| pages = 1249–1258
| year = 2018
| month = September
| doi = 10.1002/ijc.31399
| pmid = 29574715
}}</ref><ref>{{Cite journal
| author = [[Nermine O. Basta]], [[Gail C. Halliday]], [[Guy Makin]], [[Jillian Birch]], [[Richard Feltbower]], [[Nick Bown]], [[Martin Elliott]], [[Lucas Moreno]], [[Giuseppe Barone]], [[Andrew Dj Pearson]], [[Peter W. James]], [[Deborah A. Tweddle]] & [[Richard Jq McNally]]
| title = Factors associated with recurrence and survival length following relapse in patients with neuroblastoma
| journal = [[British journal of cancer]]
| volume = 115
| issue = 9
| pages = 1048–1057
| year = 2016
| month = October
| doi = 10.1038/bjc.2016.302
| pmid = 27701387
}}</ref>
* INSS '''stage 1''', '''stage 2''', and '''stage 4S''' neuroblastomas  are associated with a 3 year [[survival rate]] of 75-90%.
* INSS '''stage 3''' neuroblastoma is associated with a 3 year [[survival rate]] of 80-90% among patients '''younger''' than 1 year of [[age]].
* INSS '''stage 3''' neuroblastoma is associated with a 3 year [[survival rate]] of 50% among patients '''older''' than 1 year of [[age]].
* INSS '''stage 4''' neuroblastoma is associated with a 3 year [[survival rate]] of 60-75% among patients '''younger''' than 1 year of [[age]].
* INSS '''stage 4''' neuroblastoma is associated with a 3 year [[survival rate]] of 15% among patients '''older''' than 1 year of [[age]].
* The table below lists the [[Prognosis|prognostic]] factors for neuroblastoma [[Patient|patients]]:<ref name="radio">Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/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><ref>{{Cite journal
  | author = [[G. Hale]], [[M. J. Gula]] & [[J. Blatt]]
| title = Impact of gender on the natural history of neuroblastoma
| journal = [[Pediatric hematology and oncology]]
| volume = 11
| issue = 1
| pages = 91–97
| year = 1994
| month = January-February
| pmid = 8155504
}}</ref>
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1100px"
| valign="top" |
|+
! style="background: #4479BA; width: 200px; color: #FFFFFF;" |'''Prognostic Factor'''


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.<ref>[http://www.ajc.com/health/content/shared-auto/healthnews/-bon/602068.html "Childhood Cancer Survivors Face Increased Sarcoma Risk"], HealthDay News, February 21, 2007</ref><ref>Oeffinger et al.,[http://content.nejm.org/cgi/content/abstract/355/15/1572 "Chronic Health Conditions in Adult Survivors of Childhood Cancer"], New England Journal of Medicine, October 12, 2006</ref>
! style="background: #4479BA; width: 600px; color: #FFFFFF;" |'''Description'''


===Criteria===
|-
When the [[lesion]] is localized, it is generally curable. However, long-term survival for children with advanced disease is poor despite aggressive multimodality therapy.


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. 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. 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.
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Patient's Age'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:[[Patient|Patients]] older than 18 months of age are associated with poor [[prognosis]].
 
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Tumor Stage'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:Advanced INSS stages of neuroblastoma are associated with poor [[prognosis]].
 
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Tumor Grade'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:An unfavorable Shimada [[histology]] index is associated with poor [[prognosis]].
 
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Schwannnian Stroma'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:Reduced schwannian [[stroma]] content on [[histological]] analysis is associated with poor [[prognosis]].
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Mitotic-karyorrhectic Index'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:A high mitotic-karyorrhectic index is associated with poor [[prognosis]].
 
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''[[Genetic mutations|Genetic Mutations]]'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:Chromosome 1p deletion, [[chromosome]] 11q deletion, and ''N-MYC'' [[oncogene]] amplification are associated with poor [[prognosis]].
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | '''Response to Treatment'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:[[Patient|Patients]] whose neuroblastoma responds to treatment and goes into complete [[Remission (medicine)|remission]] have a better [[prognosis]] than people whose [[cancer]] does not respond to the initial treatment.
|}


==References==
==References==
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[[Category:Disease]]
[[Category:Disease]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Neurology]]
[[Category:Neurosurgery]]

Latest revision as of 15:40, 7 March 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Zahir Ali Shaikh, MD[2]Haytham Allaham, M.D. [3]

Overview

Neuroblastoma may progress to develop fatigue, loss of appetite, joint pain and fever if left untreated. There is gradual development of site specific symptoms as the tumor size gradually increases. Complications of neuroblastoma include persistent refractory diarrhea, horner's syndrome, hypertension, transverse myelopathy, anemia and suppressed immunity. The prognosis of neuroblastoma is generally regarded as poor, depending on the tumor extent at the time of diagnosis. The other prognostic factors for nuroblastoma include patient's age, tumor stage and grade, genetic mutations and response to treatment.

Natural History

Complications

Prognosis

  • 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.[7][8][9][10]
  • INSS stage 1, stage 2, and stage 4S neuroblastomas are associated with a 3 year survival rate of 75-90%.
  • INSS stage 3 neuroblastoma is associated with a 3 year survival rate of 80-90% among patients younger than 1 year of age.
  • INSS stage 3 neuroblastoma is associated with a 3 year survival rate of 50% among patients older than 1 year of age.
  • INSS stage 4 neuroblastoma is associated with a 3 year survival rate of 60-75% among patients younger than 1 year of age.
  • INSS stage 4 neuroblastoma is associated with a 3 year survival rate of 15% among patients older than 1 year of age.
  • The table below lists the prognostic factors for neuroblastoma patients:[11][12][13][14]
Prognostic Factor Description
Patient's Age
Patients older than 18 months of age are associated with poor prognosis.
Tumor Stage
Advanced INSS stages of neuroblastoma are associated with poor prognosis.
Tumor Grade
An unfavorable Shimada histology index is associated with poor prognosis.
Schwannnian Stroma
Reduced schwannian stroma content on histological analysis is associated with poor prognosis.
Mitotic-karyorrhectic Index
A high mitotic-karyorrhectic index is associated with poor prognosis.
Genetic Mutations
Chromosome 1p deletion, chromosome 11q deletion, and N-MYC oncogene amplification are associated with poor prognosis.
Response to Treatment
Patients whose neuroblastoma responds to treatment and goes into complete remission have a better prognosis than people whose cancer does not respond to the initial treatment.

References

  1. ↑ L. M. Wilson & G. J. Draper (1974). "Neuroblastoma, its natural history and prognosis: a study of 487 cases". British medical journal. 3 (5926): 301–307. PMID 4414795. Unknown parameter |month= ignored (help)
  2. ↑ Somruetai Shuangshoti, Shanop Shuangshoti, Issarang Nuchprayoon, Somjai Kanjanapongkul, Paula Marrano, Meredith S. Irwin & Paul S. Thorner (2012). "Natural course of low risk neuroblastoma". Pediatric blood & cancer. 58 (5): 690–694. doi:10.1002/pbc.23325. PMID 21922650. Unknown parameter |month= ignored (help)
  3. ↑ C. S. Alvarado, W. B. London, A. T. Look, G. M. Brodeur, D. H. Altmiller, P. S. Thorner, V. V. Joshi, S. T. Rowe, M. B. Nash, E. I. Smith, R. P. Castleberry & S. L. Cohn (2000). "Natural history and biology of stage A neuroblastoma: a Pediatric Oncology Group Study". Journal of pediatric hematology/oncology. 22 (3): 197–205. PMID 10864050. Unknown parameter |month= ignored (help)
  4. ↑ Nadja C. Colon & Dai H. Chung (2011). "Neuroblastoma". Advances in pediatrics. 58 (1): 297–311. doi:10.1016/j.yapd.2011.03.011. PMID 21736987.
  5. ↑ John M. Maris (2010). "Recent advances in neuroblastoma". The New England journal of medicine. 362 (23): 2202–2211. doi:10.1056/NEJMra0804577. PMID 20558371. Unknown parameter |month= ignored (help)
  6. ↑ Michal Sieniawski, Jeremy Franklin, Lucia Nogova, Jan-Peter Glossmann, Thomas Schober, Hiltrud Nisters-Backes, Volker Diehl & Andreas Josting (2007). "Outcome of patients experiencing progression or relapse after primary treatment with two cycles of chemotherapy and radiotherapy for early-stage favorable Hodgkin's lymphoma". Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 25 (15): 2000–2005. doi:10.1200/JCO.2006.10.1386. PMID 17420510. Unknown parameter |month= ignored (help)
  7. ↑ Julie R. Park, Angelika Eggert & Huib Caron (2008). "Neuroblastoma: biology, prognosis, and treatment". Pediatric clinics of North America. 55 (1): 97–120. doi:10.1016/j.pcl.2007.10.014. PMID 18242317. Unknown parameter |month= ignored (help)
  8. ↑ L. M. Wilson & G. J. Draper (1974). "Neuroblastoma, its natural history and prognosis: a study of 487 cases". British medical journal. 3 (5926): 301–307. PMID 4414795. Unknown parameter |month= ignored (help)
  9. ↑ Maya Suzuki, Brian H. Kushner, Kim Kramer, Ellen M. Basu, Stephen S. Roberts, William J. Hammond, Michael P. LaQuaglia, Suzanne L. Wolden, Nai-Kong V. Cheung & Shakeel Modak (2018). "Treatment and outcome of adult-onset neuroblastoma". International journal of cancer. 143 (5): 1249–1258. doi:10.1002/ijc.31399. PMID 29574715. Unknown parameter |month= ignored (help)
  10. ↑ Nermine O. Basta, Gail C. Halliday, Guy Makin, Jillian Birch, Richard Feltbower, Nick Bown, Martin Elliott, Lucas Moreno, Giuseppe Barone, Andrew Dj Pearson, Peter W. James, Deborah A. Tweddle & Richard Jq McNally (2016). "Factors associated with recurrence and survival length following relapse in patients with neuroblastoma". British journal of cancer. 115 (9): 1048–1057. doi:10.1038/bjc.2016.302. PMID 27701387. Unknown parameter |month= ignored (help)
  11. ↑ Neuroblastoma. Radiopaedia(2015) http://radiopaedia.org/articles/neuroblastoma Accessed on October, 5 2015
  12. ↑ 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
  13. ↑ Neuroblastoma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Adrenal_gland#Neuroblastoma Accessed on October, 5 2015
  14. ↑ G. Hale, M. J. Gula & J. Blatt (1994). "Impact of gender on the natural history of neuroblastoma". Pediatric hematology and oncology. 11 (1): 91–97. PMID 8155504. Unknown parameter |month= ignored (help)

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