Subtype
Spindle Cell Rhabdomyosarcoma
CI-CAN-00008472Explore in graph →
2 active trials25 evidence items
Loading cancer entity…
Loading cancer entity…
Subtype
CI-CAN-00008472Explore in graph →
Variants & evidence
27 evidence items mapped to this entity or its descendants, grouped by molecular profile, then therapy. 50 items per page.
| Therapy | Type | Level | Direction · significance | Rating (1–5) | Status | Evidence | Source |
|---|---|---|---|---|---|---|---|
| TFCP2 Fusion3 | |||||||
| (diagnostic) | Diagnostic | C | Supports Positive | 4 | accepted | EID12825This study presented clinicopathological and molecular findings of TFCP2-rearranged rhabdomyosarcomas with epithelioid and/or spindle cell morphology. The cohort comprised 14 cases, including 3 index … (full text at CIViC) PMID 31383960 · Le Loarer et al., 2020 · Open in CIViC | civic |
| 〃 | Diagnostic | C | Supports Positive | 4 | accepted | EID12827This study reported clinicopathologic and molecular features of 25 cases of fusion-driven spindle cell/sclerosing rhabdomyosarcoma (SCSRMS). The tumors occurred in bone (n=19) and soft tissue (n=6). T… (full text at CIViC) PMID 37422156 · Dehner et al., 2023 · Open in CIViC | civic |
| 〃 | Diagnostic | C | Supports Positive | 3 | accepted | EID12826This study presented clinicopathological and molecular features of 11 cases of head and neck rhabdomyosarcomas (HNRMSs). The median age of the cohort was 29 years, ranging 16-74 years with no sex bias… (full text at CIViC) PMID 33382123 · Xu et al., 2021 · Open in CIViC | civic |
| TFCP2 Fusion1 | |||||||
| (diagnostic) | Diagnostic | C | Supports Positive | 4 | accepted | EID12326This 2018 study highlighted the diagnostic role of the FUS::TFCP2 fusion in spindle cell/sclerosing rhabdomyosarcoma (sRMS) through a single-case study of a primary spindle cell rhabdomyosarcoma of bo… (full text at CIViC) PMID 29758589 · Dashti et al., 2018 · Open in CIViC | civic |
| ALK Exon 2-18 Deletion + TFCP2 FusionTFCP2ALK2 | |||||||
| (diagnostic) | Diagnostic | C | Supports Positive | 4 | accepted | EID12405A 31 year old male initially thought to have a mandibular osteosarcoma that was resected and treated with adjuvant radiation and cisplatin-doxorubicin. He had a mandibular recurrence with multiple pul… (full text at CIViC) PMID 39302072 · Csizmok et al., 2024 · Open in CIViC | civic |
| Alectinib | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID12406A 31 year old male initially thought to have a mandibular osteosarcoma that was resected and treated with adjuvant radiation and cisplatin-doxorubicin. He had a mandibular recurrence with multiple pul… (full text at CIViC) PMID 39302072 · Csizmok et al., 2024 · Open in CIViC | civic |
| MYOD1 L122R10 | |||||||
| (diagnostic) | Diagnostic | B | Supports Positive | 4 | accepted | EID8193This study evaluated forty-nine cases of rhabdomyosarcoma (RMS) (17 cases of alveolar RMS, 10 of embryonal RMS, 21 of spindle cell and sclerosing RMS, and a single case of a pleomorphic RMS) for MYOD1… (full text at CIViC) PMID 27562493 · Rekhi et al., 2016 · Open in CIViC | civic |
| 〃 | Diagnostic | B | Supports Positive | 4 | accepted | EID11582This study assessed MYOD1 exon 1 mutations in pediatric and adult sclerosing and spindle cell rhabdomyosarcoma. 5 sclerosing (2 pediatric and 3 adults) and 11 spindle cell rhabdomyosarcomas (3 pediatr… (full text at CIViC) PMID 24824843 · Agaram et al., 2014 · Open in CIViC | civic |
| (prognostic) | Prognostic | B | Supports Poor Outcome | 4 | accepted | EID11583This study assessed MYOD1 exon 1 mutations in pediatric and adult sclerosing and spindle cell rhabdomyosarcoma. 5 sclerosing (2 pediatric and 3 adults) and 11 spindle cell rhabdomyosarcomas (3 pediatr… | |
| VGLL2 Fusion4 | |||||||
| (prognostic) | Prognostic | B | Supports Better Outcome | 4 | accepted | EID11568In this 2017 study, the authors analyzed molecular features of 26 pediatric spindle cell and sclerosing rhabdomyosarcoma (SRMS) cases. The cohort included 11 congenital/infantile patients (age 0–1 yea… (full text at CIViC) PMID 26501226 · Alaggio et al., 2016 · Open in CIViC | civic |
| (diagnostic) | Diagnostic | B | Supports Positive | 3 | accepted | EID11565In this 2015 study, the authors analyzed 26 pediatric spindle cell and sclerosing rhabdomyosarcoma (SRMS) cases, including 11 congenital/infantile cases, using a combination of RNA sequencing, FISH, t… (full text at CIViC) PMID 26501226 · Alaggio et al., 2016 · Open in CIViC | civic |
| 〃 | Diagnostic | C | Supports Positive | 4 | accepted | EID12870In this 2020 study, the authors analyzed clinical, histopathologic, and RNA sequencing data from 37 infants with rhabdomyosarcoma (RMS) diagnosed before six months of age. The median age of the cohort… | |
| NCOA2 Fusion5 | |||||||
| (diagnostic) | Diagnostic | B | Supports Positive | 4 | accepted | EID12699In this 2025 retrospective study, 14 spindle cell rhabdomyosarcoma (SCRMS) cases previously identified as possessing ZFP64::NCOA2 or ZFP64::NCOA3 fusions were pulled from clinical archives to assess c… (full text at CIViC) PMID 41075874 · Dehner et al., 2025 · Open in CIViC | civic |
| 〃 | Diagnostic | B | Supports Positive | 3 | accepted | EID11566In this 2016 study, the authors analyzed 26 pediatric spindle and sclerosing rhabdomyosarcomas (SRMS) using FISH, targeted DNA, and whole transcriptome sequencing to define their molecular landscape. … (full text at CIViC) PMID 26501226 · Alaggio et al., 2016 · Open in CIViC | civic |
| (prognostic) | Prognostic | B | Supports Better Outcome | 3 | accepted | EID11567This study looked at 26 spindle cell and sclerosing rhabdomyosarcomas. 11 were congenital/infantile type diagnosed at birth or within one year of age, 10 of which harbored recurrent fusions involving … | |
| TFCP2 Fusion2 | |||||||
| (diagnostic) | Diagnostic | B | Supports Positive | 3 | accepted | EID12335In this study, the authors investigated the diagnostic role of TFCP2 fusions in spindle cell/sclerosing rhabdomyosarcoma (SC/S-RMS), a rare and aggressive subtype of rhabdomyosarcoma. While SC/S-RMS e… (full text at CIViC) PMID 31383960 · Le Loarer et al., 2020 · Open in CIViC | civic |
| 〃 | Diagnostic | C | Supports Positive | 4 | accepted | EID12325This study investigated the role of TFCP2-related gene fusions in primary intraosseous rhabdomyosarcomas (PIOS), particularly within spindle cell and sclerosing rhabdomyosarcoma subtypes. The analysis… (full text at CIViC) PMID 30720533 · Agaram et al., 2019 · Open in CIViC | civic |
Data updated 23 hours agoSource updated unknownsource: civic (CC0)
Evidence levels, directions and ratings are those assigned by CIViC curators. "Submitted" items have not completed curation review. This is not treatment guidance.
PMID 24824843 · Agaram et al., 2014 · Open in CIViC
| civic |
| 〃 | Prognostic | B | Supports Poor Outcome | 4 | submitted | EID11584This study assessed the clinicopathologic and prognostic characteristics of MYOD1-mutant rhabdomyosarcoma in a total of 30 cases of which 15 are children. Cases were analyzed for the known MYOD1 exon … (full text at CIViC) PMID 30181563 · Agaram et al., 2019 · Open in CIViC | civic |
| (diagnostic) | Diagnostic | B | Supports Positive | 4 | accepted | EID11585This study assessed the clinicopathologic and molecular features of 13 spindle cell (SRMS) and 7 spindle cell/sclerosing rhabdomyosarcomas (ScRMS) arising in the head and neck region. Age ranged from … (full text at CIViC) PMID 31949721 · Wang et al., 2018 · Open in CIViC | civic |
| (prognostic) | Prognostic | B | Supports Poor Outcome | 4 | submitted | EID11586This study assessed the clinicopathologic and molecular features of 13 spindle cell (SRMS) and 7 spindle cell/sclerosing rhabdomyosarcomas (ScRMS) arising in the head and neck region. Age ranged from … (full text at CIViC) PMID 31949721 · Wang et al., 2018 · Open in CIViC | civic |
| 〃 | Prognostic | B | Supports Poor Outcome | 4 | accepted | EID11587This study looked at 26 spindle cell and sclerosing rhabdomyosarcomas. 11 were congenital/infantile type diagnosed at birth or within one year of age, 10 of which harbored recurrent fusions involving … (full text at CIViC) PMID 26501226 · Alaggio et al., 2016 · Open in CIViC | civic |
| (diagnostic) | Diagnostic | B | Supports Positive | 4 | accepted | EID11588In this study, FISH, targeted DNA, and whole transcriptome sequencing were used to define a better molecular classification of sclerosing and spindle cell rhabdomyosarcoma. 11 of 26 were congenital/i… (full text at CIViC) PMID 26501226 · Alaggio et al., 2016 · Open in CIViC | civic |
| (prognostic) | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID8313This study examined 17 cases of spindle cell/sclerosing rhabdomyosarcoma (SC/SRMS) for the presence of MYOD1 variants. 12 cases harbored a MYOD-1 mutation, of which 8 cases were homozygous for p.L122R… (full text at CIViC) PMID 30604891 · Tsai et al., 2019 · Open in CIViC | civic |
| 〃 | Prognostic | B | Supports Poor Outcome | 2 | accepted | EID8194This study evaluated forty-nine cases of rhabdomyosarcoma (RMS) (17 cases of alveolar RMS, 10 of embryonal RMS, 21 of spindle cell and sclerosing RMS, and a single case of a pleomorphic RMS) for MYOD1… (full text at CIViC) PMID 27562493 · Rekhi et al., 2016 · Open in CIViC | civic |
PMID 32087612 · Butel et al., 2020 · Open in CIViC
| civic |
| 〃 | Diagnostic | C | Supports Positive | 3 | accepted | EID12871In this 2021 study, the authors analyzed clinical, pathologic, molecular, and radiologic features of 4 pediatric patients with VGLL2-rearranged infantile sclerosing and spindle-cell rhabdomyosarcoma (… (full text at CIViC) PMID 33949344 · Cyrta et al., 2021 · Open in CIViC | civic |
PMID 26501226 · Alaggio et al., 2016 · Open in CIViC
| civic |
| (diagnostic) | Diagnostic | B | Supports Positive | 3 | accepted | EID12727In this 2022 study, the authors included 40 patients aged ≤12 months diagnosed with spindle cell rhabdomyosarcoma (SCRMS) enrolled in international trials and registries, of whom 39 presented with loc… (full text at CIViC) PMID 35452896 · Whittle et al., 2022 · Open in CIViC | civic |
| 〃 | Diagnostic | C | Supports Positive | 3 | accepted | EID12731In this 2013 study, the authors included 21 spindle cell and sclerosing rhabdomyosarcomas (RMS), and a control group of 4 embryonal RMS and 3 ectomesenchymomas. RNA sequencing and FISH were utilized t… (full text at CIViC) PMID 23463663 · Mosquera et al., 2013 · Open in CIViC | civic |