Subtype
Intermediate Soft Tissue Neoplasm
CI-CAN-00001021Explore in graph →
- NCIt
- C7653
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Subtype
CI-CAN-00001021Explore in graph →
Variants & evidence
68 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 |
|---|---|---|---|---|---|---|---|---|
| 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 Sensitivity Response | 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 |
| 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 |
| FOSB Fusion1 | ||||||||
| (diagnostic) | Pseudomyogenic Hemangioendothelioma | Diagnostic | B | Supports Positive | 3 | accepted | EID11785In this study, the authors analyzed two index cases of pseudomyogenic haemangioendothelioma and 10 additional cases by interphase FISH. Both index cases showed a balanced t(7;19)(q22;q13) translocatio… (full text at CIViC) PMID 24374978 · Walther et al., 2014 · 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 14 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.
| 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 |
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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 |