Cancer Family
Fibroblastic Neoplasm
CI-CAN-00000181Explore in graph →
- NCIt
- C7075
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Cancer Family
CI-CAN-00000181Explore in graph →
Variants & evidence
81 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 |
|---|---|---|---|---|---|---|---|---|
| CREB3L1 Fusion3 | ||||||||
| (diagnostic) | Sclerosing Epithelioid Fibrosarcoma | Diagnostic | B | Supports Positive | 3 | accepted | EID12058In this 2017 study, the authors utilized SNP arrays, expression profiling, transcriptome, and whole-exome sequencing to further characterize sclerosing epithelioid fibrosarcoma (SEF) and low-grade fi… (full text at CIViC) PMID 28939748 · Arbajian et al., 2017 · Open in CIViC | civic |
| 〃 | Sclerosing Epithelioid Fibrosarcoma | Diagnostic | C | Supports Positive | 3 | accepted | EID12057In this 2014 study, the authors compared 10 primary sclerosing epithelioid fibrosarcomas (SEF), 4 cases of low-grade fibromyxoid sarcoma (LGFMS), and one primary hybrid SEF/LGFMS. Patients with SEF ra… (full text at CIViC) PMID 24441665 · Arbajian et al., 2014 · Open in CIViC | civic |
| 〃 | Sclerosing Epithelioid Fibrosarcoma | Diagnostic | C | Supports Positive | 3 | accepted | EID12059In this 2015 study, the authors aimed to further characterize the molecular characteristics of pure sclerosing epithelioid fibrosarcoma (SEF) and hybrid SEF/low-grade fibromyxoid sarcoma (LGFMS) lesio… (full text at CIViC) PMID 25231134 · Prieto-Granada et al., 2015 · Open in CIViC | civic |
| ALK Fusion1 | ||||||||
| Lorlatinib | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID11292This case study was on a 43-year-old female patient with an FN1-ALK gene fusion. In several case reports and phase 1b/phase 2 studies, responses of ALK-positive IMTs to crizotinib have been shown. Cri… (full text at CIViC) PMID 33007314 · Reinhart et al., 2020 · Open in CIViC | civic |
| ROS1 Fusion1 | ||||||||
| (oncogenic) | Inflammatory Myofibroblastic Tumor | Oncogenic | C | Supports Oncogenicity | 2 | submitted | EID12611In the STARTRK-NG Phase 1/2 trial of entrectinib in pediatric patients, with extracranial solid tumors or primary CNS tumors, aged <22 years with relapsed or refractory disease, tumors with fusions… (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| CREB3L2 Fusion3 | ||||||||
| (diagnostic) | Low Grade Fibromyxoid Sarcoma | Diagnostic | B | Supports Positive | 4 | accepted | EID12061RT-PCR analysis of 59 low-grade fibromyxoid sarcomas (LGFMS) and 52 non-LGFMS tumors selected for their resemblance to LGFMS (LGFMS-like tumors), identified a FUS::CREB3L2 fusion in 45 cases, a FUS::C… (full text at CIViC) PMID 17721195 · Guillou et al., 2007 · Open in CIViC | civic |
| 〃 | Low Grade Fibromyxoid Sarcoma | Diagnostic | B | Supports Positive | 3 | accepted | EID12062In this study, an RT-PCR assay detected a FUS::CREB3L2 transcript in 14 of 16 (88%) low-grade fibromyxoid sarcoma (LGFMS) cases, whereas the fusion was not detected in any of the 123 other soft-tissue… (full text at CIViC) PMID 16931951 · Matsuyama et al., 2006 · Open in CIViC | civic |
| 〃 | Low Grade Fibromyxoid Sarcoma | Diagnostic | C | Supports Positive | ||||
| ALK Fusion1 | ||||||||
| Entrectinib | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID12610In the STARTRK-NG Phase 1/2 trial of entrectinib in pediatric patients, with extracranial solid tumors or primary CNS tumors, aged <22 years with relapsed or refractory disease, tumors with fusions… (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| NTRK1 Fusion1 | ||||||||
| Crizotinib | Infantile FibrosarcomaALIAS | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID8878A mass found at birth in a patient’s left arm became enlarged at age 2 and was resected and characterized as a high-grade spindle cell sarcoma. FISH and RT-PCR did not detect any common sarcoma transl… (full text at CIViC) PMID 30709876 · Bender et al., 2019 · Open in CIViC | civic |
| NTRK3 R248C1 | ||||||||
| (diagnostic) | Infantile FibrosarcomaALIAS | Diagnostic | A | Supports Positive | 4 | rejected | EID8331This is the first study showing the ETV6-NTRK3 gene fusion in congenital fibrosarcoma (CFS). Using cytogenetic and FISH analyses, a novel t(12;15)(p13;q25) rearrangement was identified in CFS cases (3… (full text at CIViC) PMID 9462753 · Knezevich et al., 1998 · Open in CIViC | civic |
| PDGFB Rearrangement3 | ||||||||
| Imatinib Mesylate | Dermatofibrosarcoma Protuberans | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID10312An 18-month-old female patient presented with a history of an enlarging, subcutaneous mass in her right lower extremity since birth. A biopsy was performed and the findings were typical of Dermatofibr… (full text at CIViC) PMID 15503291 · Price et al., 2005 · Open in CIViC | civic |
| 〃 | Dermatofibrosarcoma Protuberans | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID10330In this retrospective study, two patients with locally advanced Dermatofibrosarcoma Protuberans (DFSP) and two patients with recurrent DFSP received imatinib treatment prior to undergoing Mohs surgery… (full text at CIViC) PMID 19620561 · Han et al., 2009 · Open in CIViC | civic |
| Sorafenib Tosylate | Dermatofibrosarcoma Protuberans | Predictive | C | Supports | ||||
| PDGFRB Overexpression1 | ||||||||
| Sunitinib | Dermatofibrosarcoma Protuberans | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID7992A 37-year-old man presented with dermatofibrosarcoma protuberans (DFSP). Post resection, multiple nodular metastases were found in both lungs and the disease progressed following treatments with cytot… (full text at CIViC) PMID 29760553 · Xiao et al., 2018 · Open in CIViC | civic |
| ALK Fusion1 | ||||||||
| Crizotinib | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | 4 | accepted | EID1244ALK-rearrangements are seen in 50% of inflammatory myofibroblastic tumor (IMT) cases. A 44 year old man was diagnosed with IMT. He was treated with surgery and catheter placement for administration o… (full text at CIViC) PMID 20979472 · Butrynski et al., 2010 · Open in CIViC | civic |
| MET Fusion1 | ||||||||
| Cabozantinib | Infantile FibrosarcomaALIAS | Predictive | C | Supports Sensitivity Response | 4 | submitted | EID8892We describe a novel RBPMS-MET driver fusion in association with a locally aggressive IFS-like tumor. MET functions as an oncogene and, when associated with the RNA binding protein RBPMS, forms an in-f… (full text at CIViC) PMID 33028644 · Gupta et al., 2020 · Open in CIViC | civic |
| NTRK1 Fusion2 | ||||||||
| Larotrectinib | Infantile FibrosarcomaALIAS | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID6103One patient with infantile fibrosarcoma and a NTRK1-SQSTM1 fusion was treated with larotrectinib and exhibited a partial response. Treatment was done within a multicentre, open-label, phase 1/2 study … (full text at CIViC) PMID 29606586 · Laetsch et al., 2018 · Open in CIViC | civic |
| 〃 | Infantile FibrosarcomaALIAS | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID8865In a pediatric phase 1 trial (NCT02637687), a 2 year old with infantile fibrosarcoma of the shoulder harbored the SQSTM1-NTRK1 fusion. After two surgical resections and multiple rounds of chemotherapy… (full text at CIViC) PMID 30204247 · DuBois et al., 2018 · Open in CIViC | civic |
| ROS1 Fusion3 | ||||||||
| Crizotinib | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID1444Case report of a pediatric patient with treatment-refactory inflammatory myofibroblastic tumor negative for ALK expression and rearrangement. Next generation sequencing of the patient revealed a TFG-R… (full text at CIViC) PMID 24875859 · Lovly et al., 2014 · Open in CIViC | civic |
| Entrectinib | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID11860In 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 inflammat… (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| 〃 | Inflammatory Myofibroblastic Tumor | Predictive | C | Supports Sensitivity Response | ||||
| 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 |
| 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 | ||
Data updated 13 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.
| 3 |
| accepted |
EID12060In this 2014 study, the authors compared 10 primary sclerosing epithelioid fibrosarcomas (SEF), 4 cases of low-grade fibromyxoid sarcoma (LGFMS), and one primary hybrid SEF/LGFMS. Patients with LGFMS … (full text at CIViC) PMID 24441665 · Arbajian et al., 2014 · Open in CIViC |
| civic |
| 1 |
| submitted |
EID10333In this case report, a 36-year-old male is presented with an advanced and recurrent Dermatofibrosarcoma Protuberans (DFSP) lesion. Following the third relapse and a round of radiotherapy, he was put o… (full text at CIViC) PMID 23557478 · Kamar et al., 2013 · Open in CIViC |
| civic |
| 1 |
| accepted |
EID11862In 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 myofibrob… (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC |
| civic |
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 |
| civic |
| 〃 | 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 |