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Refractory Alveolar Rhabdomyosarcoma

MedGen UID:
1640719
•Concept ID:
C4684888
•
Neoplastic Process

Definition

Alveolar rhabdomyosarcoma that does not respond to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Alveolar Rhabdomyosarcoma

Professional guidelines

PubMed

Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment - Experience from a Major Pediatric Cancer Center in Europe.
Barnbrock AE, Luckowitsch M, Schmidt S, Buus-Gehrig C, Koscher L, Becker M, Vokuhl C, Klingebiel T, Lehrnbecher T, Bochennek K
Klin Padiatr 2023 Nov;235(6):360-365. Epub 2023 Jul 11 doi: 10.1055/a-2103-2781. PMID: 37494132
Vinorelbine and low-dose cyclophosphamide in the treatment of pediatric sarcomas: pilot study for the upcoming European Rhabdomyosarcoma Protocol.
Casanova M, Ferrari A, Bisogno G, Merks JH, De Salvo GL, Meazza C, Tettoni K, Provenzi M, Mazzarino I, Carli M
Cancer 2004 Oct 1;101(7):1664-71. doi: 10.1002/cncr.20544. PMID: 15378498

Recent clinical studies

Etiology

Alveolar rhabdomyosarcoma has superior response rates to vinorelbine compared to embryonal rhabdomyosarcoma in patients with relapsed/refractory disease: A meta-analysis.
Allen-Rhoades W, Lupo PJ, Scheurer ME, Chi YY, Kuttesch JF, Venkatramani R, Meyer WH, Mascarenhas L
Cancer Med 2023 May;12(9):10222-10229. Epub 2023 Apr 4 doi: 10.1002/cam4.5749. PMID: 37016270Free PMC Article
Protocol for the 2ND-STEP study, Japan Clinical Oncology Group study JCOG1802: a randomized phase II trial of second-line treatment for advanced soft tissue sarcoma comparing trabectedin, eribulin and pazopanib.
Endo M, Kataoka T, Fujiwara T, Tsukushi S, Takahashi M, Kobayashi E, Yamada Y, Tanaka T, Nezu Y, Hiraga H, Wasa J, Nagano A, Nakano K, Nakayama R, Hamada T, Kawano M, Torigoe T, Sakamoto A, Asanuma K, Morii T, Machida R, Sekino Y, Fukuda H, Oda Y, Ozaki T, Tanaka K
BMC Cancer 2023 Mar 8;23(1):219. doi: 10.1186/s12885-023-10693-w. PMID: 36890471Free PMC Article
Immunoreactivity against fibroblast growth factor 8 in alveolar rhabdomyosarcoma patients and its involvement in tumor aggressiveness.
Poli E, Barbon V, Lucchetta S, Cattelan M, Santoro L, Zin A, Milano GM, Zanetti I, Bisogno G, Bonvini P
Oncoimmunology 2022;11(1):2096349. Epub 2022 Jul 6 doi: 10.1080/2162402X.2022.2096349. PMID: 35813575Free PMC Article
Second-line therapy in young patients with relapsed or refractory orbital rhabdomyosarcoma.
Zloto O, Minard-Colin V, Boutroux H, Brisse HJ, Levy C, Kolb F, Bolle S, Carton M, Helfre S, Orbach D
Acta Ophthalmol 2021 May;99(3):334-341. Epub 2020 Aug 24 doi: 10.1111/aos.14596. PMID: 32833335
Future directions in risk stratification and therapy for advanced pediatric genitourinary rhabdomyosarcoma.
Harel M, Ferrer FA, Shapiro LH, Makari JH
Urol Oncol 2016 Feb;34(2):103-15. Epub 2015 Oct 28 doi: 10.1016/j.urolonc.2015.09.013. PMID: 26519985

Diagnosis

Alveolar rhabdomyosarcoma has superior response rates to vinorelbine compared to embryonal rhabdomyosarcoma in patients with relapsed/refractory disease: A meta-analysis.
Allen-Rhoades W, Lupo PJ, Scheurer ME, Chi YY, Kuttesch JF, Venkatramani R, Meyer WH, Mascarenhas L
Cancer Med 2023 May;12(9):10222-10229. Epub 2023 Apr 4 doi: 10.1002/cam4.5749. PMID: 37016270Free PMC Article
A phase I trial of metformin in combination with vincristine, irinotecan, and temozolomide in children with relapsed or refractory solid and central nervous system tumors: A report from the national pediatric cancer foundation.
Metts JL, Trucco M, Weiser DA, Thompson P, Sandler E, Smith T, Crimella J, Sansil S, Thapa R, Fridley BL, Llosa N, Badgett T, Gorlick R, Reed D, Gill J
Cancer Med 2023 Feb;12(4):4270-4281. Epub 2022 Sep 23 doi: 10.1002/cam4.5297. PMID: 36151773Free PMC Article
Immunoreactivity against fibroblast growth factor 8 in alveolar rhabdomyosarcoma patients and its involvement in tumor aggressiveness.
Poli E, Barbon V, Lucchetta S, Cattelan M, Santoro L, Zin A, Milano GM, Zanetti I, Bisogno G, Bonvini P
Oncoimmunology 2022;11(1):2096349. Epub 2022 Jul 6 doi: 10.1080/2162402X.2022.2096349. PMID: 35813575Free PMC Article
Refractory alveolar rhabdomyosarcoma in an 11-year-old male.
Ricker CA, Woods AD, Simonson W, Lathara M, Srinivasa G, Rudzinski ER, Mansoor A, Irwin RG, Keller C, Berlow NE
Cold Spring Harb Mol Case Stud 2021 Feb;7(1) Epub 2021 Feb 19 doi: 10.1101/mcs.a005983. PMID: 33436392Free PMC Article
FGF receptors: cancer biology and therapeutics.
Katoh M, Nakagama H
Med Res Rev 2014 Mar;34(2):280-300. Epub 2013 May 21 doi: 10.1002/med.21288. PMID: 23696246

Therapy

A phase I trial of metformin in combination with vincristine, irinotecan, and temozolomide in children with relapsed or refractory solid and central nervous system tumors: A report from the national pediatric cancer foundation.
Metts JL, Trucco M, Weiser DA, Thompson P, Sandler E, Smith T, Crimella J, Sansil S, Thapa R, Fridley BL, Llosa N, Badgett T, Gorlick R, Reed D, Gill J
Cancer Med 2023 Feb;12(4):4270-4281. Epub 2022 Sep 23 doi: 10.1002/cam4.5297. PMID: 36151773Free PMC Article
Immunoreactivity against fibroblast growth factor 8 in alveolar rhabdomyosarcoma patients and its involvement in tumor aggressiveness.
Poli E, Barbon V, Lucchetta S, Cattelan M, Santoro L, Zin A, Milano GM, Zanetti I, Bisogno G, Bonvini P
Oncoimmunology 2022;11(1):2096349. Epub 2022 Jul 6 doi: 10.1080/2162402X.2022.2096349. PMID: 35813575Free PMC Article
Refractory alveolar rhabdomyosarcoma in an 11-year-old male.
Ricker CA, Woods AD, Simonson W, Lathara M, Srinivasa G, Rudzinski ER, Mansoor A, Irwin RG, Keller C, Berlow NE
Cold Spring Harb Mol Case Stud 2021 Feb;7(1) Epub 2021 Feb 19 doi: 10.1101/mcs.a005983. PMID: 33436392Free PMC Article
Second-line therapy in young patients with relapsed or refractory orbital rhabdomyosarcoma.
Zloto O, Minard-Colin V, Boutroux H, Brisse HJ, Levy C, Kolb F, Bolle S, Carton M, Helfre S, Orbach D
Acta Ophthalmol 2021 May;99(3):334-341. Epub 2020 Aug 24 doi: 10.1111/aos.14596. PMID: 32833335
FGF receptors: cancer biology and therapeutics.
Katoh M, Nakagama H
Med Res Rev 2014 Mar;34(2):280-300. Epub 2013 May 21 doi: 10.1002/med.21288. PMID: 23696246

Prognosis

Immunoreactivity against fibroblast growth factor 8 in alveolar rhabdomyosarcoma patients and its involvement in tumor aggressiveness.
Poli E, Barbon V, Lucchetta S, Cattelan M, Santoro L, Zin A, Milano GM, Zanetti I, Bisogno G, Bonvini P
Oncoimmunology 2022;11(1):2096349. Epub 2022 Jul 6 doi: 10.1080/2162402X.2022.2096349. PMID: 35813575Free PMC Article
ERBB2-CAR-Engineered Cytokine-Induced Killer Cells Exhibit Both CAR-Mediated and Innate Immunity Against High-Risk Rhabdomyosarcoma.
Merker M, Wagner J, Kreyenberg H, Heim C, Moser LM, Wels WS, Bonig H, Ivics Z, Ullrich E, Klingebiel T, Bader P, Rettinger E
Front Immunol 2020;11:581468. Epub 2020 Oct 19 doi: 10.3389/fimmu.2020.581468. PMID: 33193388Free PMC Article
Topotecan/carboplatin regimen for refractory/recurrent rhabdomyosarcoma in children: Report from the AIEOP Soft Tissue Sarcoma Committee.
Compostella A, Affinita MC, Casanova M, Milano GM, Scagnellato A, Dall'Igna P, Chiaravalli S, Pierobon M, Manzitti C, Zanetti I, Schiavetti A, Sorbara S, Mura RM, Ruggiero A, Ferrari A, Bisogno G
Tumori 2019 Apr;105(2):138-143. Epub 2018 Aug 22 doi: 10.1177/0300891618792479. PMID: 30131003
Serum and Glucocorticoid Inducible Kinase 1-Sensitive Survival, Proliferation and Migration of Rhabdomyosarcoma Cells.
Schmid E, Stagno MJ, Yan J, Schleicher S, Yu W, Honisch S, Lang F, Fuchs J, Seitz G
Cell Physiol Biochem 2017;43(3):1301-1308. Epub 2017 Oct 9 doi: 10.1159/000481842. PMID: 28992614
Future directions in risk stratification and therapy for advanced pediatric genitourinary rhabdomyosarcoma.
Harel M, Ferrer FA, Shapiro LH, Makari JH
Urol Oncol 2016 Feb;34(2):103-15. Epub 2015 Oct 28 doi: 10.1016/j.urolonc.2015.09.013. PMID: 26519985

Clinical prediction guides

Alveolar rhabdomyosarcoma has superior response rates to vinorelbine compared to embryonal rhabdomyosarcoma in patients with relapsed/refractory disease: A meta-analysis.
Allen-Rhoades W, Lupo PJ, Scheurer ME, Chi YY, Kuttesch JF, Venkatramani R, Meyer WH, Mascarenhas L
Cancer Med 2023 May;12(9):10222-10229. Epub 2023 Apr 4 doi: 10.1002/cam4.5749. PMID: 37016270Free PMC Article
A phase I trial of metformin in combination with vincristine, irinotecan, and temozolomide in children with relapsed or refractory solid and central nervous system tumors: A report from the national pediatric cancer foundation.
Metts JL, Trucco M, Weiser DA, Thompson P, Sandler E, Smith T, Crimella J, Sansil S, Thapa R, Fridley BL, Llosa N, Badgett T, Gorlick R, Reed D, Gill J
Cancer Med 2023 Feb;12(4):4270-4281. Epub 2022 Sep 23 doi: 10.1002/cam4.5297. PMID: 36151773Free PMC Article
A Phase I Trial of TB-403 in Relapsed Medulloblastoma, Neuroblastoma, Ewing Sarcoma, and Alveolar Rhabdomyosarcoma.
Saulnier-Sholler G, Duda DG, Bergendahl G, Ebb D, Snuderl M, Laetsch TW, Michlitsch J, Hanson D, Isakoff MS, Bielamowicz K, Kraveka JM, Ferguson W, Carmeliet P, De Deene A, Gijsen L, Jain RK
Clin Cancer Res 2022 Sep 15;28(18):3950-3957. doi: 10.1158/1078-0432.CCR-22-1169. PMID: 35833850Free PMC Article
ERBB2-CAR-Engineered Cytokine-Induced Killer Cells Exhibit Both CAR-Mediated and Innate Immunity Against High-Risk Rhabdomyosarcoma.
Merker M, Wagner J, Kreyenberg H, Heim C, Moser LM, Wels WS, Bonig H, Ivics Z, Ullrich E, Klingebiel T, Bader P, Rettinger E
Front Immunol 2020;11:581468. Epub 2020 Oct 19 doi: 10.3389/fimmu.2020.581468. PMID: 33193388Free PMC Article
The tyrosine kinase inhibitor crizotinib does not have clinically meaningful activity in heavily pre-treated patients with advanced alveolar rhabdomyosarcoma with FOXO rearrangement: European Organisation for Research and Treatment of Cancer phase 2 trial 90101 'CREATE'.
Schöffski P, Wozniak A, Leahy MG, Aamdal S, Rutkowski P, Bauer S, Richter S, Grünwald V, Debiec-Rychter M, Sciot R, Geoerger B, Marréaud S, Collette S, Nzokirantevye A, Strauss SJ
Eur J Cancer 2018 May;94:156-167. Epub 2018 Mar 20 doi: 10.1016/j.ejca.2018.02.011. PMID: 29567632

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