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Refractory Peripheral Primitive Neuroectodermal Tumor

MedGen UID:
1639124
Concept ID:
C4684863
Neoplastic Process

Definition

Peripheral primitive neuroectodermal tumor that does not respond to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Peripheral Primitive Neuroectodermal Tumor

Professional guidelines

PubMed

Nysom K, Morad AG, Rafael MS, Zier J, Marachelian A, Watt T, Morgenstern DA
Pediatr Blood Cancer 2023 May;70(5):e30217. Epub 2023 Feb 11 doi: 10.1002/pbc.30217. PMID: 36772891
Cabral J, Fernandez EI, Toy B, Secola R
Paediatr Drugs 2023 Jan;25(1):13-25. Epub 2022 Nov 25 doi: 10.1007/s40272-022-00544-9. PMID: 36434427Free PMC Article
Charron M
Q J Nucl Med Mol Imaging 2013 Mar;57(1):40-52. PMID: 23474634

Recent clinical studies

Etiology

Del Bufalo F, De Angelis B, Caruana I, Del Baldo G, De Ioris MA, Serra A, Mastronuzzi A, Cefalo MG, Pagliara D, Amicucci M, Li Pira G, Leone G, Bertaina V, Sinibaldi M, Di Cecca S, Guercio M, Abbaszadeh Z, Iaffaldano L, Gunetti M, Iacovelli S, Bugianesi R, Macchia S, Algeri M, Merli P, Galaverna F, Abbas R, Garganese MC, Villani MF, Colafati GS, Bonetti F, Rabusin M, Perruccio K, Folsi V, Quintarelli C, Locatelli F; Precision Medicine Team–IRCCS Ospedale Pediatrico Bambino Gesù
N Engl J Med 2023 Apr 6;388(14):1284-1295. doi: 10.1056/NEJMoa2210859. PMID: 37018492
Goldsmith KC, Park JR, Kayser K, Malvar J, Chi YY, Groshen SG, Villablanca JG, Krytska K, Lai LM, Acharya PT, Goodarzian F, Pawel B, Shimada H, Ghazarian S, States L, Marshall L, Chesler L, Granger M, Desai AV, Mody R, Morgenstern DA, Shusterman S, Macy ME, Pinto N, Schleiermacher G, Vo K, Thurm HC, Chen J, Liyanage M, Peltz G, Matthay KK, Berko ER, Maris JM, Marachelian A, Mossé YP
Nat Med 2023 May;29(5):1092-1102. Epub 2023 Apr 3 doi: 10.1038/s41591-023-02297-5. PMID: 37012551Free PMC Article
Qiu B, Matthay KK
Nat Rev Clin Oncol 2022 Aug;19(8):515-533. Epub 2022 May 25 doi: 10.1038/s41571-022-00643-z. PMID: 35614230
DuBois SG, Macy ME, Henderson TO
Am Soc Clin Oncol Educ Book 2022 Apr;42:1-13. doi: 10.1200/EDBK_349783. PMID: 35522915
Ponzoni M, Bachetti T, Corrias MV, Brignole C, Pastorino F, Calarco E, Bensa V, Giusto E, Ceccherini I, Perri P
J Exp Clin Cancer Res 2022 Mar 11;41(1):92. doi: 10.1186/s13046-022-02281-w. PMID: 35277192Free PMC Article

Diagnosis

Oesterheld J, Ferguson W, Kraveka JM, Bergendahl G, Clinch T, Lorenzi E, Berry D, Wada RK, Isakoff MS, Eslin DE, Brown VI, Roberts W, Zage P, Harrod VL, Mitchell DS, Hanson D, Saulnier Sholler GL
J Clin Oncol 2024 Jan 1;42(1):90-102. Epub 2023 Oct 26 doi: 10.1200/JCO.22.02875. PMID: 37883734Free PMC Article
Goldsmith KC, Park JR, Kayser K, Malvar J, Chi YY, Groshen SG, Villablanca JG, Krytska K, Lai LM, Acharya PT, Goodarzian F, Pawel B, Shimada H, Ghazarian S, States L, Marshall L, Chesler L, Granger M, Desai AV, Mody R, Morgenstern DA, Shusterman S, Macy ME, Pinto N, Schleiermacher G, Vo K, Thurm HC, Chen J, Liyanage M, Peltz G, Matthay KK, Berko ER, Maris JM, Marachelian A, Mossé YP
Nat Med 2023 May;29(5):1092-1102. Epub 2023 Apr 3 doi: 10.1038/s41591-023-02297-5. PMID: 37012551Free PMC Article
DuBois SG, Macy ME, Henderson TO
Am Soc Clin Oncol Educ Book 2022 Apr;42:1-13. doi: 10.1200/EDBK_349783. PMID: 35522915
Rafael MS, Cohen-Gogo S, Irwin MS, Vali R, Shammas A, Morgenstern DA
PET Clin 2021 Jul;16(3):419-427. doi: 10.1016/j.cpet.2021.03.006. PMID: 34053585
Schengrund CL
Int J Mol Sci 2020 Jul 27;21(15) doi: 10.3390/ijms21155313. PMID: 32726962Free PMC Article

Therapy

Del Bufalo F, De Angelis B, Caruana I, Del Baldo G, De Ioris MA, Serra A, Mastronuzzi A, Cefalo MG, Pagliara D, Amicucci M, Li Pira G, Leone G, Bertaina V, Sinibaldi M, Di Cecca S, Guercio M, Abbaszadeh Z, Iaffaldano L, Gunetti M, Iacovelli S, Bugianesi R, Macchia S, Algeri M, Merli P, Galaverna F, Abbas R, Garganese MC, Villani MF, Colafati GS, Bonetti F, Rabusin M, Perruccio K, Folsi V, Quintarelli C, Locatelli F; Precision Medicine Team–IRCCS Ospedale Pediatrico Bambino Gesù
N Engl J Med 2023 Apr 6;388(14):1284-1295. doi: 10.1056/NEJMoa2210859. PMID: 37018492
Goldsmith KC, Park JR, Kayser K, Malvar J, Chi YY, Groshen SG, Villablanca JG, Krytska K, Lai LM, Acharya PT, Goodarzian F, Pawel B, Shimada H, Ghazarian S, States L, Marshall L, Chesler L, Granger M, Desai AV, Mody R, Morgenstern DA, Shusterman S, Macy ME, Pinto N, Schleiermacher G, Vo K, Thurm HC, Chen J, Liyanage M, Peltz G, Matthay KK, Berko ER, Maris JM, Marachelian A, Mossé YP
Nat Med 2023 May;29(5):1092-1102. Epub 2023 Apr 3 doi: 10.1038/s41591-023-02297-5. PMID: 37012551Free PMC Article
Qiu B, Matthay KK
Nat Rev Clin Oncol 2022 Aug;19(8):515-533. Epub 2022 May 25 doi: 10.1038/s41571-022-00643-z. PMID: 35614230
DuBois SG, Macy ME, Henderson TO
Am Soc Clin Oncol Educ Book 2022 Apr;42:1-13. doi: 10.1200/EDBK_349783. PMID: 35522915
Mody R, Yu AL, Naranjo A, Zhang FF, London WB, Shulkin BL, Parisi MT, Servaes SE, Diccianni MB, Hank JA, Felder M, Birstler J, Sondel PM, Asgharzadeh S, Glade-Bender J, Katzenstein H, Maris JM, Park JR, Bagatell R
J Clin Oncol 2020 Jul 1;38(19):2160-2169. Epub 2020 Apr 28 doi: 10.1200/JCO.20.00203. PMID: 32343642Free PMC Article

Prognosis

Qiu B, Matthay KK
Nat Rev Clin Oncol 2022 Aug;19(8):515-533. Epub 2022 May 25 doi: 10.1038/s41571-022-00643-z. PMID: 35614230
Tang XX, Shimada H, Ikegaki N
Genes Immun 2022 Jun;23(3-4):129-140. Epub 2022 May 7 doi: 10.1038/s41435-022-00172-w. PMID: 35525858Free PMC Article
DuBois SG, Macy ME, Henderson TO
Am Soc Clin Oncol Educ Book 2022 Apr;42:1-13. doi: 10.1200/EDBK_349783. PMID: 35522915
Schengrund CL
Int J Mol Sci 2020 Jul 27;21(15) doi: 10.3390/ijms21155313. PMID: 32726962Free PMC Article
Mody R, Yu AL, Naranjo A, Zhang FF, London WB, Shulkin BL, Parisi MT, Servaes SE, Diccianni MB, Hank JA, Felder M, Birstler J, Sondel PM, Asgharzadeh S, Glade-Bender J, Katzenstein H, Maris JM, Park JR, Bagatell R
J Clin Oncol 2020 Jul 1;38(19):2160-2169. Epub 2020 Apr 28 doi: 10.1200/JCO.20.00203. PMID: 32343642Free PMC Article

Clinical prediction guides

Guo X, Chang M, Wang Y, Xing B, Ma W
Int J Biol Sci 2023;19(12):3762-3780. Epub 2023 Jul 24 doi: 10.7150/ijbs.85813. PMID: 37564196Free PMC Article
Del Bufalo F, De Angelis B, Caruana I, Del Baldo G, De Ioris MA, Serra A, Mastronuzzi A, Cefalo MG, Pagliara D, Amicucci M, Li Pira G, Leone G, Bertaina V, Sinibaldi M, Di Cecca S, Guercio M, Abbaszadeh Z, Iaffaldano L, Gunetti M, Iacovelli S, Bugianesi R, Macchia S, Algeri M, Merli P, Galaverna F, Abbas R, Garganese MC, Villani MF, Colafati GS, Bonetti F, Rabusin M, Perruccio K, Folsi V, Quintarelli C, Locatelli F; Precision Medicine Team–IRCCS Ospedale Pediatrico Bambino Gesù
N Engl J Med 2023 Apr 6;388(14):1284-1295. doi: 10.1056/NEJMoa2210859. PMID: 37018492
Su Y, Luo B, Lu Y, Wang D, Yan J, Zheng J, Xiao J, Wang Y, Xue Z, Yin J, Chen P, Li L, Zhao Q
Clin Cancer Res 2022 Feb 15;28(4):793-809. doi: 10.1158/1078-0432.CCR-21-2241. PMID: 34844980Free 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, Yu AL, Naranjo A, Zhang FF, London WB, Shulkin BL, Parisi MT, Servaes SE, Diccianni MB, Hank JA, Felder M, Birstler J, Sondel PM, Asgharzadeh S, Glade-Bender J, Katzenstein H, Maris JM, Park JR, Bagatell R
J Clin Oncol 2020 Jul 1;38(19):2160-2169. Epub 2020 Apr 28 doi: 10.1200/JCO.20.00203. PMID: 32343642Free PMC Article

Recent systematic reviews

Herd F, Basta NO, McNally RJQ, Tweddle DA
Eur J Cancer 2019 Apr;111:50-58. Epub 2019 Feb 26 doi: 10.1016/j.ejca.2018.12.032. PMID: 30822684Free PMC Article
Wilson JS, Gains JE, Moroz V, Wheatley K, Gaze MN
Eur J Cancer 2014 Mar;50(4):801-15. Epub 2013 Dec 12 doi: 10.1016/j.ejca.2013.11.016. PMID: 24333097

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