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Spindle cell rhabdomyosarcoma

MedGen UID:
224766
Concept ID:
C1266134
Neoplastic Process
Synonyms: SCRMS; Spindle Cell Rhabdomyosarcoma; spindle cell rhabdomyosarcoma; spindle cell rhabdomyosarcoma (morphologic abnormality)
SNOMED CT: Spindle cell rhabdomyosarcoma (404055006); Spindle cell rhabdomyosarcoma (128749008)
 
Monarch Initiative: MONDO:0002581

Definition

An uncommon variant of rhabdomyosarcoma characterized by the presence of whorls of spindle cells forming a storiform pattern. In children it usually arises in the paratesticular region. In adults it usually arises from the deep soft tissues in the head and neck. [from NCI]

Professional guidelines

PubMed

Ahmeti H, Jüttner E, Röcken C, Jansen O, Laudes M, Synowitz M
J Neurol Surg A Cent Eur Neurosurg 2023 May;84(3):295-299. Epub 2021 Nov 15 doi: 10.1055/s-0041-1735857. PMID: 34781400
Parham DM
F1000Res 2018;7 Epub 2018 Dec 20 doi: 10.12688/f1000research.16236.1. PMID: 30613391Free PMC Article
Carroll SJ, Nodit L
Arch Pathol Lab Med 2013 Aug;137(8):1155-8. doi: 10.5858/arpa.2012-0465-RS. PMID: 23899074

Recent clinical studies

Etiology

Han R, Dermawan JK, Demicco EG, Ferguson PC, Griffin AM, Swanson D, Antonescu CR, Dickson BC
Genes Chromosomes Cancer 2022 Nov;61(11):645-652. Epub 2022 May 20 doi: 10.1002/gcc.23052. PMID: 35521817Free PMC Article
Butel T, Karanian M, Pierron G, Orbach D, Ranchere D, Cozic N, Galmiche L, Coulomb A, Corradini N, Lacour B, Proust S, Guerin F, Boutroux H, Rome A, Mansuy L, Vérité C, Defachelles AS, Tirode F, Minard-Colin V
Cancer Med 2020 Apr;9(8):2698-2709. Epub 2020 Feb 22 doi: 10.1002/cam4.2713. PMID: 32087612Free PMC Article
Zhao Z, Yin Y, Zhang J, Qi J, Zhang D, Ma Y, Wang Y, Li S, Zhou J
Int J Clin Exp Pathol 2015;8(11):13814-20. Epub 2015 Nov 1 PMID: 26823695Free PMC Article
Yasui N, Yoshida A, Kawamoto H, Yonemori K, Hosono A, Kawai A
Pediatr Blood Cancer 2015 Jun;62(6):1011-6. Epub 2014 Dec 31 doi: 10.1002/pbc.25367. PMID: 25557260
Medeiros CW, Kondo W, Baptista I Jr, Vizzotto AO Jr, Noronha Ld, Hakim Neto CA
Rev Hosp Clin Fac Med Sao Paulo 2002 Mar-Apr;57(2):67-72. doi: 10.1590/s0041-87812002000200004. PMID: 11981587

Diagnosis

Lethongsavarn V, Vieille P, Kikweta Makhama J, Azmani R, Lafrance W, Khneisser P, Truffaut N, Alame M, Genestie C, Gaspar N, Diedhiou A, Croce S, Le Loarer F
Genes Chromosomes Cancer 2023 Dec;62(12):746-754. Epub 2023 Sep 14 doi: 10.1002/gcc.23201. PMID: 37706559
Ahmeti H, Jüttner E, Röcken C, Jansen O, Laudes M, Synowitz M
J Neurol Surg A Cent Eur Neurosurg 2023 May;84(3):295-299. Epub 2021 Nov 15 doi: 10.1055/s-0041-1735857. PMID: 34781400
Whittle S, Venkatramani R, Schönstein A, Pack SD, Alaggio R, Vokuhl C, Rudzinski ER, Wulf AL, Zin A, Gruver JR, Arnold MA, Merks JHM, Hettmer S, Koscielniak E, Barr FG, Hawkins DS, Bisogno G, Sparber-Sauer M
Eur J Cancer 2022 Jun;168:56-64. Epub 2022 Apr 19 doi: 10.1016/j.ejca.2022.03.022. PMID: 35452896Free PMC Article
Kerr DA, Thompson LDR, Tafe LJ, Jo VY, Neyaz A, Divakar P, Paydarfar JA, Pastel DA, Shirai K, John I, Seethala RR, Salgado CM, Deshpande V, Bridge JA, Kashofer K, Brčić I, Linos K
Am J Surg Pathol 2021 Dec 1;45(12):1707-1719. doi: 10.1097/PAS.0000000000001735. PMID: 34001695
Carroll SJ, Nodit L
Arch Pathol Lab Med 2013 Aug;137(8):1155-8. doi: 10.5858/arpa.2012-0465-RS. PMID: 23899074

Therapy

Whittle SB, Hicks MJ, Roy A, Vasudevan SA, Reddy K, Venkatramani R
Pediatr Blood Cancer 2019 Nov;66(11):e27935. Epub 2019 Jul 24 doi: 10.1002/pbc.27935. PMID: 31339226
Gui H, Lhospital E, Staddon AP, Nagda SN, Zager EL, Zhang PJL, Brooks JS
Int J Surg Pathol 2019 May;27(3):328-335. Epub 2018 Oct 1 doi: 10.1177/1066896918802030. PMID: 30270691
Wu TT, Wang QY, Zhou SH, Zheng ZJ
Int J Clin Exp Pathol 2014;7(8):5254-8. Epub 2014 Jul 15 PMID: 25197406Free PMC Article
Latz S, Ellinger J, Goltz D, Marx C, Leuschner I, Müller SC, Fechner G
Int J Urol 2013 Sep;20(9):935-7. Epub 2013 Jan 16 doi: 10.1111/iju.12082. PMID: 23320845
Goosens V, Van den Berghe I, De Clercq C, Casselman J
Int J Oral Maxillofac Surg 2008 Apr;37(4):395-7. Epub 2007 Nov 26 doi: 10.1016/j.ijom.2007.09.173. PMID: 18023143

Prognosis

Haug L, Doll J, Appenzeller S, Kunzmann V, Rosenwald A, Maurus K, Gerhard-Hartmann E
Pathol Res Pract 2023 Sep;249:154779. Epub 2023 Aug 22 doi: 10.1016/j.prp.2023.154779. PMID: 37633005
Agaimy A, Dermawan JK, Leong I, Stoehr R, Swanson D, Weinreb I, Zhang L, Antonescu CR, Dickson BC
Genes Chromosomes Cancer 2022 Dec;61(12):701-709. Epub 2022 Aug 1 doi: 10.1002/gcc.23083. PMID: 35766997Free PMC Article
Whittle S, Venkatramani R, Schönstein A, Pack SD, Alaggio R, Vokuhl C, Rudzinski ER, Wulf AL, Zin A, Gruver JR, Arnold MA, Merks JHM, Hettmer S, Koscielniak E, Barr FG, Hawkins DS, Bisogno G, Sparber-Sauer M
Eur J Cancer 2022 Jun;168:56-64. Epub 2022 Apr 19 doi: 10.1016/j.ejca.2022.03.022. PMID: 35452896Free PMC Article
Whittle SB, Hicks MJ, Roy A, Vasudevan SA, Reddy K, Venkatramani R
Pediatr Blood Cancer 2019 Nov;66(11):e27935. Epub 2019 Jul 24 doi: 10.1002/pbc.27935. PMID: 31339226
Carroll SJ, Nodit L
Arch Pathol Lab Med 2013 Aug;137(8):1155-8. doi: 10.5858/arpa.2012-0465-RS. PMID: 23899074

Clinical prediction guides

Duan FL, Yang H, Gong X, Zuo Z, Qin S, Ji J, Zhou C, Dai J, Guo P, Liu Y
Histopathology 2023 Feb;82(3):478-484. Epub 2022 Nov 21 doi: 10.1111/his.14833. PMID: 36350070
Kerr DA, Thompson LDR, Tafe LJ, Jo VY, Neyaz A, Divakar P, Paydarfar JA, Pastel DA, Shirai K, John I, Seethala RR, Salgado CM, Deshpande V, Bridge JA, Kashofer K, Brčić I, Linos K
Am J Surg Pathol 2021 Dec 1;45(12):1707-1719. doi: 10.1097/PAS.0000000000001735. PMID: 34001695
Jo VY, Mariño-Enríquez A, Fletcher CDM, Hornick JL
Am J Surg Pathol 2018 Oct;42(10):1275-1285. doi: 10.1097/PAS.0000000000001092. PMID: 29863547
Zhao Z, Yin Y, Zhang J, Qi J, Zhang D, Ma Y, Wang Y, Li S, Zhou J
Int J Clin Exp Pathol 2015;8(11):13814-20. Epub 2015 Nov 1 PMID: 26823695Free PMC Article
de Saint Aubain Somerhausen N, Fletcher CD
Am J Surg Pathol 1999 Jul;23(7):755-63. doi: 10.1097/00000478-199907000-00002. PMID: 10403297

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