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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

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
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
Berthold F, Hero B
Drugs 2000 Jun;59(6):1261-77. doi: 10.2165/00003495-200059060-00006. PMID: 10882162

Recent clinical studies

Etiology

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
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
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
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
Koka VN, Julieron M, Bourhis J, Janot F, Le Ridant AM, Marandas P, Luboinski B, Schwaab G
J Laryngol Otol 1998 Jul;112(7):628-33. doi: 10.1017/s0022215100141295. PMID: 9775291

Diagnosis

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
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
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
Koka VN, Julieron M, Bourhis J, Janot F, Le Ridant AM, Marandas P, Luboinski B, Schwaab G
J Laryngol Otol 1998 Jul;112(7):628-33. doi: 10.1017/s0022215100141295. PMID: 9775291
Humpl T
World J Urol 1995;13(4):233-9. doi: 10.1007/BF00182969. PMID: 8528298

Therapy

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
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
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
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
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

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
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
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
Koka VN, Julieron M, Bourhis J, Janot F, Le Ridant AM, Marandas P, Luboinski B, Schwaab G
J Laryngol Otol 1998 Jul;112(7):628-33. doi: 10.1017/s0022215100141295. PMID: 9775291
Humpl T
World J Urol 1995;13(4):233-9. doi: 10.1007/BF00182969. PMID: 8528298

Clinical prediction guides

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
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
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
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
Kuroda T
Pediatr Surg Int 2011 Sep;27(9):913-7. Epub 2011 Jul 19 doi: 10.1007/s00383-011-2949-6. PMID: 21769682

Recent systematic reviews

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
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
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
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
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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