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Stage 4S Neuroblastoma

MedGen UID:
210046
Concept ID:
C1135161
Neoplastic Process
Synonyms: Neuroblastoma Stage IVS; Neuroblastoma, metastatic; Pepper's Syndrome; Stage IVS Neuroblastoma
SNOMED CT: Neuroblastoma, metastatic (704147007)

Definition

Localized primary tumor, as defined for stage 1, 2A, or 2B, with dissemination limited to skin, liver, and/or bone marrow (limited to infants younger than 1 year). Marrow involvement should be minimal (i.e., <10% of total nucleated cells identified as malignant by bone biopsy or by bone marrow aspirate). More extensive bone marrow involvement would be considered stage 4 disease. The results of the MIBG scan, if performed, should be negative for disease in the bone marrow. (cancer.gov) [from NCI]

Term Hierarchy

Professional guidelines

PubMed

Peyam S, Bansal D
Indian J Pediatr 2023 Mar;90(3):213-214. Epub 2023 Jan 25 doi: 10.1007/s12098-023-04477-0. PMID: 36696002
Anand S, Agarwala S, Jain V, Dhua A, Bakhshi S, Jana M, Kandasamy D, Biswas A
Indian J Pediatr 2023 Mar;90(3):220-226. Epub 2022 Sep 16 doi: 10.1007/s12098-022-04341-7. PMID: 36112265
Weintraub M, Waldman E, Koplewitz B, Bloom AI, Gross E, Freeman AI, Revel-Vilk S
Pediatr Blood Cancer 2012 Jul 15;59(1):182-4. Epub 2011 May 16 doi: 10.1002/pbc.23186. PMID: 22605456

Recent clinical studies

Etiology

Montalto S, Sertorio F, Podda M, Sorrentino S, Di Cataldo A, Provenzi M, Nonnis A, D'Ippolito C, Corrias MV, De Bernardi B
Pediatr Hematol Oncol 2022 Aug;39(5):441-452. Epub 2022 Feb 10 doi: 10.1080/08880018.2021.2013368. PMID: 35139733
Kawano A, Hazard FK, Chiu B, Naranjo A, LaBarre B, London WB, Hogarty MD, Cohn SL, Maris JM, Park JR, Gastier-Foster JM, Ikegaki N, Shimada H
Am J Surg Pathol 2021 Aug 1;45(8):1075-1081. doi: 10.1097/PAS.0000000000001647. PMID: 33739795Free PMC Article
Tas ML, Nagtegaal M, Kraal KCJM, Tytgat GAM, Abeling NGGM, Koster J, Pluijm SMF, Zwaan CM, de Keizer B, Molenaar JJ, van Noesel MM
Pediatr Blood Cancer 2020 Apr;67(4):e28061. Epub 2019 Nov 17 doi: 10.1002/pbc.28061. PMID: 31736229
Ozguven AA, Anak S, Unuvar A, Akcay A, Karakas Z, Ozturk G, Devecioglu O, Agaoğlu L
Indian J Pediatr 2015 May;82(5):450-7. Epub 2014 Oct 2 doi: 10.1007/s12098-014-1578-1. PMID: 25274444
Halperin EC
Int J Radiat Oncol Biol Phys 2000 Apr 1;47(1):171-8. doi: 10.1016/s0360-3016(00)00424-7. PMID: 10758320

Diagnosis

Montalto S, Sertorio F, Podda M, Sorrentino S, Di Cataldo A, Provenzi M, Nonnis A, D'Ippolito C, Corrias MV, De Bernardi B
Pediatr Hematol Oncol 2022 Aug;39(5):441-452. Epub 2022 Feb 10 doi: 10.1080/08880018.2021.2013368. PMID: 35139733
Tas ML, Nagtegaal M, Kraal KCJM, Tytgat GAM, Abeling NGGM, Koster J, Pluijm SMF, Zwaan CM, de Keizer B, Molenaar JJ, van Noesel MM
Pediatr Blood Cancer 2020 Apr;67(4):e28061. Epub 2019 Nov 17 doi: 10.1002/pbc.28061. PMID: 31736229
Ozguven AA, Anak S, Unuvar A, Akcay A, Karakas Z, Ozturk G, Devecioglu O, Agaoğlu L
Indian J Pediatr 2015 May;82(5):450-7. Epub 2014 Oct 2 doi: 10.1007/s12098-014-1578-1. PMID: 25274444
Noguera R, Cañete A, Pellín A, Ruiz A, Tasso M, Navarro S, Castel V, Llombart-Bosch A
Cancer Genet Cytogenet 2003 Jan 15;140(2):157-61. doi: 10.1016/s0165-4608(02)00677-5. PMID: 12645655
Halperin EC
Int J Radiat Oncol Biol Phys 2000 Apr 1;47(1):171-8. doi: 10.1016/s0360-3016(00)00424-7. PMID: 10758320

Therapy

Mubarak M, Singal AK, Gawdi A
Gulf J Oncolog 2019 May;1(30):81-84. PMID: 31242987
Twist CJ, Naranjo A, Schmidt ML, Tenney SC, Cohn SL, Meany HJ, Mattei P, Adkins ES, Shimada H, London WB, Park JR, Matthay KK, Maris JM
J Clin Oncol 2019 Jan 10;37(2):115-124. Epub 2018 Nov 16 doi: 10.1200/JCO.18.00419. PMID: 30444686Free PMC Article
Veal GJ, Errington J, Sastry J, Chisholm J, Brock P, Morgenstern D, Pritchard-Jones K, Chowdhury T
Cancer Chemother Pharmacol 2016 Apr;77(4):685-92. Epub 2016 Feb 13 doi: 10.1007/s00280-016-2975-0. PMID: 26875154Free PMC Article
Kushner BH, Kramer K, Modak S, Akhurst TJ, Cheung NK
Pediatr Blood Cancer 2009 Dec 15;53(7):1340-2. doi: 10.1002/pbc.22243. PMID: 19711437Free PMC Article
Elimam NA, Atra AA, Fayea NY, Al-Asaad TG, Khattab TM, Al-Sulami GA, Felimban SK
Saudi Med J 2006 Nov;27(11):1734-6. PMID: 17106552

Prognosis

Kawano A, Hazard FK, Chiu B, Naranjo A, LaBarre B, London WB, Hogarty MD, Cohn SL, Maris JM, Park JR, Gastier-Foster JM, Ikegaki N, Shimada H
Am J Surg Pathol 2021 Aug 1;45(8):1075-1081. doi: 10.1097/PAS.0000000000001647. PMID: 33739795Free PMC Article
Tas ML, Nagtegaal M, Kraal KCJM, Tytgat GAM, Abeling NGGM, Koster J, Pluijm SMF, Zwaan CM, de Keizer B, Molenaar JJ, van Noesel MM
Pediatr Blood Cancer 2020 Apr;67(4):e28061. Epub 2019 Nov 17 doi: 10.1002/pbc.28061. PMID: 31736229
Ozguven AA, Anak S, Unuvar A, Akcay A, Karakas Z, Ozturk G, Devecioglu O, Agaoğlu L
Indian J Pediatr 2015 May;82(5):450-7. Epub 2014 Oct 2 doi: 10.1007/s12098-014-1578-1. PMID: 25274444
Noguera R, Cañete A, Pellín A, Ruiz A, Tasso M, Navarro S, Castel V, Llombart-Bosch A
Cancer Genet Cytogenet 2003 Jan 15;140(2):157-61. doi: 10.1016/s0165-4608(02)00677-5. PMID: 12645655
Halperin EC
Int J Radiat Oncol Biol Phys 2000 Apr 1;47(1):171-8. doi: 10.1016/s0360-3016(00)00424-7. PMID: 10758320

Clinical prediction guides

Anand S, Agarwala S, Jain V, Dhua A, Bakhshi S, Jana M, Kandasamy D, Biswas A
Indian J Pediatr 2023 Mar;90(3):220-226. Epub 2022 Sep 16 doi: 10.1007/s12098-022-04341-7. PMID: 36112265
Montalto S, Sertorio F, Podda M, Sorrentino S, Di Cataldo A, Provenzi M, Nonnis A, D'Ippolito C, Corrias MV, De Bernardi B
Pediatr Hematol Oncol 2022 Aug;39(5):441-452. Epub 2022 Feb 10 doi: 10.1080/08880018.2021.2013368. PMID: 35139733
Parodi S, Sorrentino S, Cataldo AD, Tondo A, Fagnani AM, Perri P, Gigliotti AR, Erminio G, Corrias MV, De Bernardi B
Pediatr Blood Cancer 2021 May;68(5):e28904. Epub 2021 Jan 18 doi: 10.1002/pbc.28904. PMID: 33459514
Raitio A, Rice MJ, Mullassery D, Losty PD
Eur J Pediatr Surg 2021 Oct;31(5):385-389. Epub 2020 Sep 15 doi: 10.1055/s-0040-1716836. PMID: 32932540
Tsubota S, Kadomatsu K
Cell Tissue Res 2018 May;372(2):211-221. Epub 2018 Feb 14 doi: 10.1007/s00441-018-2796-z. PMID: 29445860

Recent systematic reviews

Raitio A, Rice MJ, Mullassery D, Losty PD
Eur J Pediatr Surg 2021 Oct;31(5):385-389. Epub 2020 Sep 15 doi: 10.1055/s-0040-1716836. PMID: 32932540

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