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Sclerosing Rhabdomyosarcoma

MedGen UID:
881907
•Concept ID:
C4048700
•
Neoplastic Process
Synonym: Sclerosing rhabdomyosarcoma
SNOMED CT: Sclerosing rhabdomyosarcoma (128749008)

Definition

An uncommon variant of rhabdomyosarcoma with sclerosing morphology. It usually arises from the limbs. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSclerosing Rhabdomyosarcoma

Professional guidelines

PubMed

Rhabdomyosarcoma: From Obscurity to Clarity in Diagnosis … But With Ongoing Challenges in Management: The Farber-Landing Lecture of 2020.
Parham DM, Giannikopoulos P
Pediatr Dev Pathol 2021 Mar-Apr;24(2):87-95. Epub 2021 Jan 13 doi: 10.1177/1093526620977720. PMID: 33439112

Recent clinical studies

Etiology

An Integrative Morphologic and Molecular Approach for Diagnosis and Subclassification of Rhabdomyosarcoma.
Fan R, Parham DM, Wang LL
Arch Pathol Lab Med 2022 Aug 1;146(8):953-959. doi: 10.5858/arpa.2021-0183-RA. PMID: 35051261
Methylation profiling reveals novel molecular classes of rhabdomyosarcoma.
Clay MR, Patel A, Tran Q, Hedges DJ, Chang TC, Stewart E, Charville G, Cline C, Dyer MA, Orr BA
Sci Rep 2021 Nov 15;11(1):22213. doi: 10.1038/s41598-021-01649-w. PMID: 34782706Free PMC Article
Establishment and characterization of NCC-ssRMS2-C1: a novel patient-derived cell line of spindle cell/sclerosing rhabdomyosarcoma.
Tsuchiya R, Yoshimatsu Y, Noguchi R, Sin Y, Ono T, Sei A, Takeshita F, Sugaya J, Nakatani F, Yoshida A, Ohtori S, Kawai A, Kondo T
Hum Cell 2021 Sep;34(5):1569-1578. Epub 2021 Jun 23 doi: 10.1007/s13577-021-00569-1. PMID: 34164773
Spindle cell/sclerosing rhabdomyosarcoma: case series from a single institution emphasizing morphology, immunohistochemistry and follow-up.
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
Rhabdomyosarcomas in adults and children: an update.
Parham DM, Ellison DA
Arch Pathol Lab Med 2006 Oct;130(10):1454-65. doi: 10.5858/2006-130-1454-RIAACA. PMID: 17090187

Diagnosis

Conjunctival Spindle Cell/Sclerosing Rhabdomyosarcoma.
Su YW, Wang LC, Fan NW
Ophthalmology 2024 Aug;131(8):926. Epub 2023 Oct 24 doi: 10.1016/j.ophtha.2023.09.027. PMID: 37877920
An Integrative Morphologic and Molecular Approach for Diagnosis and Subclassification of Rhabdomyosarcoma.
Fan R, Parham DM, Wang LL
Arch Pathol Lab Med 2022 Aug 1;146(8):953-959. doi: 10.5858/arpa.2021-0183-RA. PMID: 35051261
Challenges in the Diagnosis of Pediatric Spindle Cell/Sclerosing Rhabdomyosarcoma.
Chen S, Rudzinski ER, Arnold MA
Surg Pathol Clin 2020 Dec;13(4):729-738. Epub 2020 Oct 9 doi: 10.1016/j.path.2020.08.010. PMID: 33183730
The current landscape of rhabdomyosarcomas: an update.
Leiner J, Le Loarer F
Virchows Arch 2020 Jan;476(1):97-108. Epub 2019 Nov 6 doi: 10.1007/s00428-019-02676-9. PMID: 31696361
Ear and Temporal Bone Pathology: Neural, Sclerosing and Myofibroblastic Lesions.
Flaman AN, Wasserman JK, Gravel DH, Purgina BM
Head Neck Pathol 2018 Sep;12(3):392-406. Epub 2018 Aug 1 doi: 10.1007/s12105-018-0891-9. PMID: 30069839Free PMC Article

Therapy

Congenital spindle cell rhabdomyosarcoma.
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
Combined Sclerosing and Spindle Cell Rhabdomyosarcoma in Previous Craniotomy Site: A Case Report and a Review of the Literature.
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
Sclerosing rhabdomyosarcoma of a chest wall in an adult: a case report and review of the literature.
Mikubo M, Ikeda S, Hoshino T, Yokota T, Fujii A, Mori M
Ann Thorac Cardiovasc Surg 2014;20 Suppl:642-5. Epub 2013 Oct 3 doi: 10.5761/atcs.cr.13-00030. PMID: 24088914
A complete remission of sclerosing rhabdomyosarcoma with multiple lung and bone metastases treated with multi-agent chemotherapy and peripheral blood stem cell transplantation (PBSCT): a case report.
Sakayama K, Tauchi H, Sugawara Y, Kidani T, Tokuda K, Miyazaki T, Watanabe Y, Yamamoto H
Anticancer Res 2008 Jul-Aug;28(4C):2361-7. PMID: 18751419

Prognosis

Spindle Cell/Sclerosing Rhabdomyosarcoma of the Orbit.
Proia AD
Ophthalmic Plast Reconstr Surg 2023 Jan-Feb 01;39(1):e17-e20. Epub 2022 Jul 13 doi: 10.1097/IOP.0000000000002252. PMID: 35829629
Methylation profiling reveals novel molecular classes of rhabdomyosarcoma.
Clay MR, Patel A, Tran Q, Hedges DJ, Chang TC, Stewart E, Charville G, Cline C, Dyer MA, Orr BA
Sci Rep 2021 Nov 15;11(1):22213. doi: 10.1038/s41598-021-01649-w. PMID: 34782706Free PMC Article
Challenges in the Diagnosis of Pediatric Spindle Cell/Sclerosing Rhabdomyosarcoma.
Chen S, Rudzinski ER, Arnold MA
Surg Pathol Clin 2020 Dec;13(4):729-738. Epub 2020 Oct 9 doi: 10.1016/j.path.2020.08.010. PMID: 33183730
Congenital spindle cell rhabdomyosarcoma.
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
Recently characterized soft tissue tumors that bring biologic insight.
Fletcher CD
Mod Pathol 2014 Jan;27 Suppl 1:S98-112. doi: 10.1038/modpathol.2013.172. PMID: 24384856

Clinical prediction guides

Methylation profiling reveals novel molecular classes of rhabdomyosarcoma.
Clay MR, Patel A, Tran Q, Hedges DJ, Chang TC, Stewart E, Charville G, Cline C, Dyer MA, Orr BA
Sci Rep 2021 Nov 15;11(1):22213. doi: 10.1038/s41598-021-01649-w. PMID: 34782706Free PMC Article
MUC4 is expressed in alveolar rhabdomyosarcoma.
Forgó E, Hornick JL, Charville GW
Histopathology 2021 May;78(6):905-908. Epub 2021 Mar 16 doi: 10.1111/his.14321. PMID: 33368602Free PMC Article
Establishment and characterization of NCC-ssRMS1-C1: a novel patient-derived spindle-cell/sclerosing rhabdomyosarcoma cell line.
Yoshimatsu Y, Noguchi R, Tsuchiya R, Sei A, Sugaya J, Iwata S, Sugiyama M, Yoshida A, Kawai A, Kondo T
Hum Cell 2020 Jul;33(3):886-893. Epub 2020 Apr 16 doi: 10.1007/s13577-020-00359-1. PMID: 32300959
Soft Tissue Special Issue: Skeletal Muscle Tumors: A Clinicopathological Review.
Kohashi K, Kinoshita I, Oda Y
Head Neck Pathol 2020 Mar;14(1):12-20. Epub 2020 Jan 16 doi: 10.1007/s12105-019-01113-2. PMID: 31950473Free PMC Article
Spindle cell/sclerosing rhabdomyosarcoma: case series from a single institution emphasizing morphology, immunohistochemistry and follow-up.
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

Recent systematic reviews

Epithelioid and spindle rhabdomyosarcoma with TFCP2 rearrangement in abdominal wall: a distinctive entity with poor prognosis.
Li Y, Li D, Wang J, Tang J
Diagn Pathol 2023 Mar 30;18(1):41. doi: 10.1186/s13000-023-01330-y. PMID: 36998041Free PMC Article

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