Cancer Family
Mesenchymal Cell Neoplasm
CI-CAN-00000027Explore in graph →
Non-malignant413 active trials10 approved drugs208 evidence items
- NCIt
- C7059
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Cancer Family
CI-CAN-00000027Explore in graph →
Variants & evidence
427 evidence items mapped to this entity or its descendants, grouped by molecular profile, then therapy. 50 items per page.
| Therapy | Cancer | Type | Level | Direction · significance | Rating (1–5) | Status | Evidence | Source |
|---|---|---|---|---|---|---|---|---|
| TP53 R273H1 | ||||||||
| Doxorubicin + MethotrexateSubstitutes | Osteosarcoma | Predictive | D | Supports Resistance | 3 | accepted | EID7430Several preclinical studies were testing drug resistance mechanisms of TP53-R273H variant. Saos-2 cells were transfected with TP53-R273H mutation and these cells were used in the study. Using a wester… (full text at CIViC) PMID 17363498 · Wong et al., 2007 · Open in CIViC | civic |
| TP53 Rearrangement2 | ||||||||
| (diagnostic) | Osteosarcoma | Diagnostic | B | Supports Positive | 5 | submitted | EID9247Intron 1 rearrangements of TP53 were identified in 46 of 288 osteosarcoma cases (16%). No fusions were detected in 1090 other tumors screened. Fusions were associated with loss of TP53 expression. PMID 25762628 · Ribi et al., 2015 · Open in CIViC | civic |
| 〃 | Osteosarcoma | Diagnostic | B | Supports Positive | 4 | submitted | EID9248TP53 fusions were identified in 10 of 25 osteosarcoma cases. The resulting inactivation of p53 was demonstrated by a deficiency of the radiation-induced DNA damage response. PMID 26672768 · Lorenz et al., 2016 · Open in CIViC | civic |
| NTRK1 Fusion1 | ||||||||
| Entrectinib | Myofibroma | Predictive | C | Supports Sensitivity Response | 1 | accepted | EID11861In a phase 1/2 trial of entrectinib in pediatric patients, tumors with fusions in NTRK, ROS1, or ALK had an overall response (ORR) of 57.7% (95% CI; 36.9-76.7). This included a patient with infantile… (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| ALK Fusion1 | ||||||||
| Lorlatinib | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID11293This case study was on a 40-year-old male with dyspnea and a productive cough. He was found to have a right upper lobe opacity on a chest X-ray. Subsequent investigations, including CT and PET, showed… (full text at CIViC) PMID 32868646 · Wong et al., 2020 · Open in CIViC | civic |
| NTRK2 e6::e71 | ||||||||
| (oncogenic) | Osteosarcoma | Oncogenic | C | Does Not Support Oncogenicity | 3 | accepted | EID11697Using next-generation DNA and RNA sequencing, three cases harboring NTRK fusions were identified in a cohort of 113 osteosarcomas (including both primary tumors and metastases). Each of the three case… (full text at CIViC) PMID 32022484 · Ameline et al., 2020 · Open in CIViC | civic |
| ALK Fusion8 | ||||||||
| (diagnostic) | Inflammatory Myofibroblastic Tumor | Diagnostic | C | Supports Positive | 4 | accepted | EID1783Fluorescence in situ hybridization with a probe flanking the ALK gene at 2p23 and immunohistochemistry revealed ALK expression, suggesting ALK fusions in three cases of IMT. PMID 10383129 · Griffin et al., 1999 · Open in CIViC | civic |
| Brigatinib | Inflammatory Myofibroblastic Tumor | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID11290In this single-arm, open-label, phase 1/2 trial 137 patients who had advanced malignancies, including ALK-rearranged NSCLC and two patients with inflammatory myofibroblastic tumour (IMT) were enrolled… (full text at CIViC) PMID 27836716 · Gettinger et al., 2016 · Open in CIViC | civic |
| Ceritinib | Inflammatory Myofibroblastic Tumor | Predictive | B | Supports Sensitivity Response | 3 | submitted | ||
| VGLL2 Fusion4 | ||||||||
| (prognostic) | Spindle Cell Rhabdomyosarcoma | 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) | Spindle Cell Rhabdomyosarcoma | 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 |
| 〃 | Spindle Cell Rhabdomyosarcoma | Diagnostic | C | Supports Positive | 4 | |||
| NCOA2 Fusion5 | ||||||||
| (diagnostic) | Spindle Cell Rhabdomyosarcoma | 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 |
| 〃 | Spindle Cell Rhabdomyosarcoma | 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) | Spindle Cell Rhabdomyosarcoma | Prognostic | B | Supports Better Outcome | 3 | |||
| NTRK3 e4::e11 | ||||||||
| (oncogenic) | Osteosarcoma | Oncogenic | C | Does Not Support Oncogenicity | 3 | accepted | EID11698Using next-generation DNA and RNA sequencing, three cases harboring NTRK fusions were identified in a cohort of 113 osteosarcomas (including primary tumors and metastases). Each of the three cases had… (full text at CIViC) PMID 32022484 · Ameline et al., 2020 · Open in CIViC | civic |
| TFCP2 Fusion2 | ||||||||
| (diagnostic) | Spindle Cell Rhabdomyosarcoma | 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 |
| 〃 | Spindle Cell Rhabdomyosarcoma | 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 |
| ZNF217 Overexpression1 | ||||||||
| Triciribine | Osteosarcoma | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID12210In an orthotopic mouse model of osteosarcoma (OSA), triciribine (TCN) treatment significantly reduces tumour growth and weight in both SJSA-1 and HOS cell line injections, with 5.4- and 3.0-fold reduc… (full text at CIViC) PMID 32999043 · Smeester et al., 2020 · Open in CIViC | civic |
Data updated 21 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.
EID11291In this phase 1 study, oral ceritinib was administered to 59 patients in doses of 50 - 750 mg once daily to patients with advanced cancers harbouring genetic alterations in ALK. A total of 130 patient… (full text at CIViC) PMID 24670165 · Shaw et al., 2014 · Open in CIViC |
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| 〃 | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID7547In a phase I study of the efficacy of ceritinib in ALK fusion positive cancer, a single patient with inflammatory myofibroblastic tumor was treated with 750mg daily ceritinb and showed a partial respo… (full text at CIViC) PMID 24670165 · Shaw et al., 2014 · Open in CIViC | civic |
| Crizotinib | Inflammatory Myofibroblastic Tumor | Predictive | A | Supports Sensitivity Response | 3 | accepted | EID111229 patients with ALK-positive inflammatory myofibroblastic tumour (IMT) enrolled in the PROFILE 1013 (NCT01121588) phase 1b single-arm, open-label, multi-centre study received crizotinib. ORR was 67% f… (full text at CIViC) PMID 29352732 · Gambacorti-Passerini et al., 2018 · Open in CIViC | civic |
| 〃 | Inflammatory Myofibroblastic Tumor | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID1243As part of a phase 1 dose-escalation trial (NCT00939770), seven patients between ages 12 months and 22 years with relapsed or refractory inflammatory myofibroblastic tumors with ALK-rearrangement were… (full text at CIViC) PMID 23598171 · Mossé et al., 2013 · Open in CIViC | civic |
| 〃 | Inflammatory Myofibroblastic Tumor | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID7362In this phase I/II study, 14 patients with metastatic or inoperable ALK-positive IMT were administered crizotinib orally twice daily in 28-day cycles as a single agent for an indefinite duration. The … (full text at CIViC) PMID 28787259 · Mossé et al., 2017 · Open in CIViC | civic |
| Lorlatinib | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | 4 | submitted | EID11294This case study was on an 18-year-old Caucasian female presenting with severe headaches. Brain MRI revealed multiple brain masses with the largest (5 cm) found in the anterior left frontal lobe. A met… (full text at CIViC) PMID 28713152 · Yuan et al., 2017 · Open in CIViC | civic |
| 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… (full text at CIViC) PMID 32087612 · Butel et al., 2020 · Open in CIViC |
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| 〃 | Spindle Cell Rhabdomyosarcoma | 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 |
| 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 … (full text at CIViC) PMID 26501226 · Alaggio et al., 2016 · Open in CIViC |
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| (diagnostic) | Spindle Cell Rhabdomyosarcoma | 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 |
| 〃 | Spindle Cell Rhabdomyosarcoma | 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 |