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Localized Peripheral Primitive Neuroectodermal Tumor of Bone

MedGen UID:
208939
•Concept ID:
C0855007
•
Neoplastic Process
Synonyms: Localized Neuroepithelioma of Bone; Localized PNET of Bone

Definition

A small round cell tumor with neural differentiation that is confined to a specific area of the bone and has not spread to other anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLocalized Peripheral Primitive Neuroectodermal Tumor of Bone

Professional guidelines

PubMed

Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469
Management of Orbital Involvement in Sinonasal and Ventral Skull Base Malignancies.
Neel GS, Nagel TH, Hoxworth JM, Lal D
Otolaryngol Clin North Am 2017 Apr;50(2):347-364. doi: 10.1016/j.otc.2016.12.010. PMID: 28314402
Neuroblastoma: current drug therapy recommendations as part of the total treatment approach.
Berthold F, Hero B
Drugs 2000 Jun;59(6):1261-77. doi: 10.2165/00003495-200059060-00006. PMID: 10882162

Recent clinical studies

Etiology

Stage 4N neuroblastoma before and during the era of anti-G(D2) immunotherapy.
Kushner BH, LaQuaglia MP, Cardenas FI, Basu EM, Gerstle JT, Kramer K, Roberts SS, Wolden SL, Cheung NV, Modak S
Int J Cancer 2023 Dec 15;153(12):2019-2031. Epub 2023 Aug 21 doi: 10.1002/ijc.34693. PMID: 37602920
Children's Oncology Group's 2023 blueprint for research: Bone tumors.
Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30583. Epub 2023 Jul 27 doi: 10.1002/pbc.30583. PMID: 37501549Free PMC Article
Clinical outcomes, Kadish-INSICA staging and therapeutic targeting of somatostatin receptor 2 in olfactory neuroblastoma.
Lechner M, Takahashi Y, Turri-Zanoni M, Liu J, Counsell N, Hermsen M, Kaur RP, Zhao T, Ramanathan M Jr, Schartinger VH, Emanuel O, Helman S, Varghese J, Dudas J, Riechelmann H, Sprung S, Haybaeck J, Howard D, Engel NW, Stewart S, Brooks L, Pickles JC, Jacques TS, Fenton TR, Williams L, Vaz FM, O'Flynn P, Stimpson P, Wang S, Hannan SA, Unadkat S, Hughes J, Dwivedi R, Forde CT, Randhawa P, Gane S, Joseph J, Andrews PJ, Royle G, Franchi A, Maragliano R, Battocchio S, Bewicke-Copley H, Pipinikas C, Webster A, Thirlwell C, Ho D, Teschendorff A, Zhu T, Steele CD, Pillay N, Vanhaesebroeck B, Mohyeldin A, Fernandez-Miranda J, Park KW, Le QT, West RB, Saade R, Manes RP, Omay SB, Vining EM, Judson BL, Yarbrough WG, Sansovini M, Silvia N, Grassi I, Bongiovanni A, Capper D, Schüller U, Thavaraj S, Sandison A, Surda P, Hopkins C, Ferrari M, Mattavelli D, Rampinelli V, Facchetti F, Nicolai P, Bossi P, Henriquez OA, Magliocca K, Solares CA, Wise SK, Llorente JL, Patel ZM, Nayak JV, Hwang PH, Lacy PD, Woods R, O'Neill JP, Jay A, Carnell D, Forster MD, Ishii M, London NR Jr, Bell DM, Gallia GL, Castelnuovo P, Severi S, Lund VJ, Hanna EY
Eur J Cancer 2022 Feb;162:221-236. Epub 2021 Dec 31 doi: 10.1016/j.ejca.2021.09.046. PMID: 34980502Free PMC Article
Irinotecan-temozolomide with temsirolimus or dinutuximab in children with refractory or relapsed neuroblastoma (COG ANBL1221): an open-label, randomised, phase 2 trial.
Mody R, Naranjo A, Van Ryn C, Yu AL, London WB, Shulkin BL, Parisi MT, Servaes SE, Diccianni MB, Sondel PM, Bender JG, Maris JM, Park JR, Bagatell R
Lancet Oncol 2017 Jul;18(7):946-957. Epub 2017 May 23 doi: 10.1016/S1470-2045(17)30355-8. PMID: 28549783Free PMC Article
Pediatrics: diagnosis of neuroblastoma.
Sharp SE, Gelfand MJ, Shulkin BL
Semin Nucl Med 2011 Sep;41(5):345-53. doi: 10.1053/j.semnuclmed.2011.05.001. PMID: 21803184

Diagnosis

Children's Oncology Group's 2023 blueprint for research: Bone tumors.
Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30583. Epub 2023 Jul 27 doi: 10.1002/pbc.30583. PMID: 37501549Free PMC Article
Irinotecan-temozolomide with temsirolimus or dinutuximab in children with refractory or relapsed neuroblastoma (COG ANBL1221): an open-label, randomised, phase 2 trial.
Mody R, Naranjo A, Van Ryn C, Yu AL, London WB, Shulkin BL, Parisi MT, Servaes SE, Diccianni MB, Sondel PM, Bender JG, Maris JM, Park JR, Bagatell R
Lancet Oncol 2017 Jul;18(7):946-957. Epub 2017 May 23 doi: 10.1016/S1470-2045(17)30355-8. PMID: 28549783Free PMC Article
Neuroblastoma (Peripheral neuroblastic tumours).
Luksch R, Castellani MR, Collini P, De Bernardi B, Conte M, Gambini C, Gandola L, Garaventa A, Biasoni D, Podda M, Sementa AR, Gatta G, Tonini GP
Crit Rev Oncol Hematol 2016 Nov;107:163-181. Epub 2016 Oct 6 doi: 10.1016/j.critrevonc.2016.10.001. PMID: 27823645
Pediatrics: diagnosis of neuroblastoma.
Sharp SE, Gelfand MJ, Shulkin BL
Semin Nucl Med 2011 Sep;41(5):345-53. doi: 10.1053/j.semnuclmed.2011.05.001. PMID: 21803184
Neuroblastoma.
Humpl T
World J Urol 1995;13(4):233-9. doi: 10.1007/BF00182969. PMID: 8528298

Therapy

Children's Oncology Group's 2023 blueprint for research: Bone tumors.
Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30583. Epub 2023 Jul 27 doi: 10.1002/pbc.30583. PMID: 37501549Free PMC Article
Immunotherapy for neuroblastoma by hematopoietic cell transplantation and post-transplant immunomodulation.
Ash S, Askenasy N
Crit Rev Oncol Hematol 2023 May;185:103956. Epub 2023 Mar 7 doi: 10.1016/j.critrevonc.2023.103956. PMID: 36893946
Clinical outcomes, Kadish-INSICA staging and therapeutic targeting of somatostatin receptor 2 in olfactory neuroblastoma.
Lechner M, Takahashi Y, Turri-Zanoni M, Liu J, Counsell N, Hermsen M, Kaur RP, Zhao T, Ramanathan M Jr, Schartinger VH, Emanuel O, Helman S, Varghese J, Dudas J, Riechelmann H, Sprung S, Haybaeck J, Howard D, Engel NW, Stewart S, Brooks L, Pickles JC, Jacques TS, Fenton TR, Williams L, Vaz FM, O'Flynn P, Stimpson P, Wang S, Hannan SA, Unadkat S, Hughes J, Dwivedi R, Forde CT, Randhawa P, Gane S, Joseph J, Andrews PJ, Royle G, Franchi A, Maragliano R, Battocchio S, Bewicke-Copley H, Pipinikas C, Webster A, Thirlwell C, Ho D, Teschendorff A, Zhu T, Steele CD, Pillay N, Vanhaesebroeck B, Mohyeldin A, Fernandez-Miranda J, Park KW, Le QT, West RB, Saade R, Manes RP, Omay SB, Vining EM, Judson BL, Yarbrough WG, Sansovini M, Silvia N, Grassi I, Bongiovanni A, Capper D, Schüller U, Thavaraj S, Sandison A, Surda P, Hopkins C, Ferrari M, Mattavelli D, Rampinelli V, Facchetti F, Nicolai P, Bossi P, Henriquez OA, Magliocca K, Solares CA, Wise SK, Llorente JL, Patel ZM, Nayak JV, Hwang PH, Lacy PD, Woods R, O'Neill JP, Jay A, Carnell D, Forster MD, Ishii M, London NR Jr, Bell DM, Gallia GL, Castelnuovo P, Severi S, Lund VJ, Hanna EY
Eur J Cancer 2022 Feb;162:221-236. Epub 2021 Dec 31 doi: 10.1016/j.ejca.2021.09.046. PMID: 34980502Free PMC Article
Anti-GD2 CAR-NKT cells in patients with relapsed or refractory neuroblastoma: an interim analysis.
Heczey A, Courtney AN, Montalbano A, Robinson S, Liu K, Li M, Ghatwai N, Dakhova O, Liu B, Raveh-Sadka T, Chauvin-Fleurence CN, Xu X, Ngai H, Di Pierro EJ, Savoldo B, Dotti G, Metelitsa LS
Nat Med 2020 Nov;26(11):1686-1690. Epub 2020 Oct 12 doi: 10.1038/s41591-020-1074-2. PMID: 33046868
Irinotecan-temozolomide with temsirolimus or dinutuximab in children with refractory or relapsed neuroblastoma (COG ANBL1221): an open-label, randomised, phase 2 trial.
Mody R, Naranjo A, Van Ryn C, Yu AL, London WB, Shulkin BL, Parisi MT, Servaes SE, Diccianni MB, Sondel PM, Bender JG, Maris JM, Park JR, Bagatell R
Lancet Oncol 2017 Jul;18(7):946-957. Epub 2017 May 23 doi: 10.1016/S1470-2045(17)30355-8. PMID: 28549783Free PMC Article

Prognosis

Stage 4N neuroblastoma before and during the era of anti-G(D2) immunotherapy.
Kushner BH, LaQuaglia MP, Cardenas FI, Basu EM, Gerstle JT, Kramer K, Roberts SS, Wolden SL, Cheung NV, Modak S
Int J Cancer 2023 Dec 15;153(12):2019-2031. Epub 2023 Aug 21 doi: 10.1002/ijc.34693. PMID: 37602920
Irinotecan-temozolomide with temsirolimus or dinutuximab in children with refractory or relapsed neuroblastoma (COG ANBL1221): an open-label, randomised, phase 2 trial.
Mody R, Naranjo A, Van Ryn C, Yu AL, London WB, Shulkin BL, Parisi MT, Servaes SE, Diccianni MB, Sondel PM, Bender JG, Maris JM, Park JR, Bagatell R
Lancet Oncol 2017 Jul;18(7):946-957. Epub 2017 May 23 doi: 10.1016/S1470-2045(17)30355-8. PMID: 28549783Free PMC Article
Neuroblastoma (Peripheral neuroblastic tumours).
Luksch R, Castellani MR, Collini P, De Bernardi B, Conte M, Gambini C, Gandola L, Garaventa A, Biasoni D, Podda M, Sementa AR, Gatta G, Tonini GP
Crit Rev Oncol Hematol 2016 Nov;107:163-181. Epub 2016 Oct 6 doi: 10.1016/j.critrevonc.2016.10.001. PMID: 27823645
Pediatrics: diagnosis of neuroblastoma.
Sharp SE, Gelfand MJ, Shulkin BL
Semin Nucl Med 2011 Sep;41(5):345-53. doi: 10.1053/j.semnuclmed.2011.05.001. PMID: 21803184
Neuroblastoma.
Humpl T
World J Urol 1995;13(4):233-9. doi: 10.1007/BF00182969. PMID: 8528298

Clinical prediction guides

Sequential Analysis of cfDNA Reveals Clonal Evolution in Patients with Neuroblastoma Receiving ALK-Targeted Therapy.
Bobin C, Iddir Y, Butterworth C, Masliah-Planchon J, Saint-Charles A, Bellini A, Bhalshankar J, Pierron G, Combaret V, Attignon V, André N, Corradini N, Dumont B, Mansuy L, Khanfar C, Klein S, Briandet C, Plantaz D, Millot F, Thouvenin S, Aerts I, Ndounga-Diakou LA, Laghouati S, Abbou S, Jehanno N, Tissot H, Renault S, Baulande S, Raynal V, Bozec L, Bieche I, Delattre O, Berlanga P, Schleiermacher G
Clin Cancer Res 2024 Aug 1;30(15):3316-3328. doi: 10.1158/1078-0432.CCR-24-0753. PMID: 38787533Free PMC Article
Report and literature review of four cases of EWSR1::NFATC2 round cell sarcoma.
Liu L, Li L, Ding Y, Kong F, Mo W, Ye H, Shen D
Diagn Pathol 2024 Jan 22;19(1):19. doi: 10.1186/s13000-024-01443-y. PMID: 38254207Free PMC Article
A phase II trial of nifurtimox combined with topotecan and cyclophosphamide for refractory or relapsed neuroblastoma and medulloblastoma.
Eslin D, Zage PE, Bergendahl G, Lewis E, Roberts W, Kraveka J, Mitchell D, Isakoff MS, Rawwas J, Wada RK, Fluchel M, Brown VI, Ginn K, Higgins T, BeeravallyNagulapally A, Dykema K, Hanna G, Ferguson W, Saulnier Sholler GL
Int J Cancer 2023 Sep 1;153(5):1026-1034. Epub 2023 May 29 doi: 10.1002/ijc.34569. PMID: 37246577
Clinical outcomes, Kadish-INSICA staging and therapeutic targeting of somatostatin receptor 2 in olfactory neuroblastoma.
Lechner M, Takahashi Y, Turri-Zanoni M, Liu J, Counsell N, Hermsen M, Kaur RP, Zhao T, Ramanathan M Jr, Schartinger VH, Emanuel O, Helman S, Varghese J, Dudas J, Riechelmann H, Sprung S, Haybaeck J, Howard D, Engel NW, Stewart S, Brooks L, Pickles JC, Jacques TS, Fenton TR, Williams L, Vaz FM, O'Flynn P, Stimpson P, Wang S, Hannan SA, Unadkat S, Hughes J, Dwivedi R, Forde CT, Randhawa P, Gane S, Joseph J, Andrews PJ, Royle G, Franchi A, Maragliano R, Battocchio S, Bewicke-Copley H, Pipinikas C, Webster A, Thirlwell C, Ho D, Teschendorff A, Zhu T, Steele CD, Pillay N, Vanhaesebroeck B, Mohyeldin A, Fernandez-Miranda J, Park KW, Le QT, West RB, Saade R, Manes RP, Omay SB, Vining EM, Judson BL, Yarbrough WG, Sansovini M, Silvia N, Grassi I, Bongiovanni A, Capper D, Schüller U, Thavaraj S, Sandison A, Surda P, Hopkins C, Ferrari M, Mattavelli D, Rampinelli V, Facchetti F, Nicolai P, Bossi P, Henriquez OA, Magliocca K, Solares CA, Wise SK, Llorente JL, Patel ZM, Nayak JV, Hwang PH, Lacy PD, Woods R, O'Neill JP, Jay A, Carnell D, Forster MD, Ishii M, London NR Jr, Bell DM, Gallia GL, Castelnuovo P, Severi S, Lund VJ, Hanna EY
Eur J Cancer 2022 Feb;162:221-236. Epub 2021 Dec 31 doi: 10.1016/j.ejca.2021.09.046. PMID: 34980502Free PMC Article
Anti-GD2 CAR-NKT cells in patients with relapsed or refractory neuroblastoma: an interim analysis.
Heczey A, Courtney AN, Montalbano A, Robinson S, Liu K, Li M, Ghatwai N, Dakhova O, Liu B, Raveh-Sadka T, Chauvin-Fleurence CN, Xu X, Ngai H, Di Pierro EJ, Savoldo B, Dotti G, Metelitsa LS
Nat Med 2020 Nov;26(11):1686-1690. Epub 2020 Oct 12 doi: 10.1038/s41591-020-1074-2. PMID: 33046868

Recent systematic reviews

(18) F-FDG PET and PET/CT as a diagnostic method for Ewing sarcoma: A systematic review and meta-analysis.
Seth N, Seth I, Bulloch G, Siu AHY, Guo A, Chatterjee R, MacManus M, Donnan L
Pediatr Blood Cancer 2022 Mar;69(3):e29415. Epub 2021 Oct 28 doi: 10.1002/pbc.29415. PMID: 34709700
Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469
High-dose chemotherapy and autologous haematopoietic stem cell rescue for children with high-risk neuroblastoma.
Yalçin B, Kremer LC, van Dalen EC
Cochrane Database Syst Rev 2015 Oct 5;2015(10):CD006301. doi: 10.1002/14651858.CD006301.pub4. PMID: 26436598Free PMC Article
High-dose chemotherapy and autologous haematopoietic stem cell rescue for children with high-risk neuroblastoma.
Yalçin B, Kremer LC, Caron HN, van Dalen EC
Cochrane Database Syst Rev 2013 Aug 22;(8):CD006301. doi: 10.1002/14651858.CD006301.pub3. PMID: 23970444
High-dose chemotherapy and autologous haematopoietic stem cell rescue for children with high-risk neuroblastoma.
Yalçin B, Kremer LC, Caron HN, van Dalen EC
Cochrane Database Syst Rev 2010 May 12;(5):CD006301. doi: 10.1002/14651858.CD006301.pub2. PMID: 20464740

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