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Spindle Cell/Sclerosing Rhabdomyosarcoma

MedGen UID:
890733
•Concept ID:
C4053999
•
Neoplastic Process

Definition

An uncommon variant of rhabdomyosarcoma with spindle cell or sclerosing morphology. It affects both children and adults and it is more common in males. [from NCI]

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
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
MYOD1-mutant spindle cell and sclerosing rhabdomyosarcoma: an aggressive subtype irrespective of age. A reappraisal for molecular classification and risk stratification.
Agaram NP, LaQuaglia MP, Alaggio R, Zhang L, Fujisawa Y, Ladanyi M, Wexler LH, Antonescu CR
Mod Pathol 2019 Jan;32(1):27-36. Epub 2018 Sep 4 doi: 10.1038/s41379-018-0120-9. PMID: 30181563Free 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
Clinicopathologic analysis of spindle cell/sclerosing rhabdomyosarcoma.
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

Diagnosis

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
Spindle Cell/Sclerosing Rhabdomyosarcoma With PAX8::PPARG Fusion.
Rakheja D, Park JY, Alhasan M, Uddin N
Int J Surg Pathol 2022 Dec;30(8):950-955. Epub 2022 Apr 24 doi: 10.1177/10668969221095170. PMID: 35466752
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

Prognosis

Diagnostic utility of FOXO1 immunohistochemistry for rhabdomyosarcoma classification.
Rutland CD, Gedallovich J, Wang A, Zdravkovic S, Varma S, Hornick JL, Charville GW
Histopathology 2023 Jul;83(1):49-56. Epub 2023 Mar 15 doi: 10.1111/his.14898. PMID: 36860202
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
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
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
Clinicopathologic analysis of spindle cell/sclerosing rhabdomyosarcoma.
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

Clinical prediction guides

Adult NTRK-rearranged spindle cell neoplasms of the viscera: with an emphasis on rare locations and heterologous elements.
Tsai JW, Lee JC, Hsieh TH, Huang SC, Lee PH, Liu TT, Kao YC, Chang CD, Weng TF, Li CF, Lin JC, Liang CW, Su YL, Chang IY, Wang YT, Chang NY, Yu SC, Wang JC, Huang HY
Mod Pathol 2022 Jul;35(7):911-921. Epub 2022 Feb 11 doi: 10.1038/s41379-021-01005-3. PMID: 35149769
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-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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