Mesenchymal Cell Neoplasm

In a patient with RANBP2::ALK-positive inflammatory myofibroblastic tumor, acquisition of the ALK F1174L mutation following crizotinib treatment was associated with acquired resistance. Ba/F3 studies … (full text at CIViC)

PMID 21030459 · Sasaki et al., 2010 · Open in CIViC

In a phase 3 trial (NCT03785964) involving 142 adults with progressing desmoid tumors, the gamma-secretase inhibitor nirogacestat demonstrated a significant progression-free survival benefit compared … (full text at CIViC)

PMID 36884323 · Gounder et al., 2023 · Open in CIViC

Loss of the chromatin-remodeling protein ATRX associates with ALT in cancers. Inhibition of the protein kinase ATR, a critical regulator of recombination recruited by RPA, disrupts ALT and triggers ch… (full text at CIViC)

PMID 25593184 · Flynn et al., 2015 · Open in CIViC

Jadun et al. reported an adolescent girl with a skull-base meningothelial meningioma, WHO grade 1. Initial germline testing of NF2, LZTR1, and SMARCB1 was negative. Subsequent paired tumor-normal geno… (full text at CIViC)

PMID 41442315 · Jadun et al., 2026 · Open in CIViC

This phase II clinical trial of regorafenib (NCT01068769) examined the long term safety and efficacy of regorafenib monotherapy in patients with metastatic and/or unresectable gastrointestinal stromal… (full text at CIViC)

PMID 27371698 · Ben-Ami et al., 2016 · Open in CIViC

This retrospective study of a phase 2 clinical trial (NCT01068769) examined regorafenib safety and efficacy in advanced gastrointestinal stromal tumor (GIST) patients refractory to at least imatinib a… (full text at CIViC)

PMID 22614970 · George et al., 2012 · Open in CIViC

In an in vitro study, a Plat-E cell line expressing a BRAF F595L mutation was associated with resistance to vemurafenib treatment, as compared to Plat-E cells expressing vector, wild-type BRAF or BRAF… (full text at CIViC)

PMID 26582644 · Kordes et al., 2016 · Open in CIViC

A primary central nervous system (CNS)-histiocytic sarcoma patient harboring BRAF V600E mutation was associated with improved response to vemurafenib treatment. The patient was treated with vemurafeni… (full text at CIViC)

PMID 25209580 · Idbaih et al., 2014 · Open in CIViC

In a phase 2 clinical trial, NCT01209598, 30 patients with CDK4-amplified and RB-expressing well differentiated and dedifferentiated liposarcomas (WDLS/DDLS) were treated with a selective CDK 4/6 inhi… (full text at CIViC)

PMID 23569312 · Dickson et al., 2013 · Open in CIViC

A total of 59 patients with well-differentiated or dedifferentiated liposarcomas were enrolled in a phase 2 study to evaluate palbociclib efficacy. Half of the patients had received prior treatment an… (full text at CIViC)

PMID 27124835 · Dickson et al., 2016 · Open in CIViC

Analysis of the antiproliferative effects of pharmacologic CDK4/6 inhibitors provided initial evidence of this notion, as lowest CDK4- expressing Rh28 cells showed the highest sensitivity to both LEE0… (full text at CIViC)

PMID 25810375 · Olanich et al., 2015 · Open in CIViC

In a phase 2 trial, patients with GIST bearing CDKN2A gene loss were treated with Palbociclib. 86.4% of patients had PD at 4 months.

PMID 30979737 · Toulmonde et al., 2019 · Open in CIViC

An imatinib-resistant cell line, DFSP105, was established from a dermatofibrosarcoma protuberans which had progressed to a high-grade fibrosarcomatous DFSP. The disease-associated COL1A1-PDGFB fusion … (full text at CIViC)

PMID 25852058 · Eilers et al., 2015 · Open in CIViC

This study analyzed 27 formalin-fixed-paraffin-embedded samples of patients with known dermatofibrosarcoma protuberans (DFSP) using a newly designed multiplex RT-PCR assay. The assay identified COL1A1… (full text at CIViC)

PMID 17950782 · Patel et al., 2008 · Open in CIViC

The authors studied 27 archival formalin-fixed, paraffin-embedded (FFPE) samples of dermatofibrosarcoma protuberans (DFSP) cases obtained from Texas Children's Hospital and University of Texas MD Ande… (full text at CIViC)

PMID 17950782 · Patel et al., 2008 · Open in CIViC

In the phase II trial (NCT02992743) of letetresgene autoleucel (lete-cel) in human leukocyte antigen ((HLA)-A*02:01–, HLA-A*02:05–, and/or HLA-A*02:06–positive), NY-ESO-1 (CTAG1B)-expressing advanced … (full text at CIViC)

PMID 39836945 · D'Angelo et al., 2025 · Open in CIViC

This study illustrates the prevalence of CTNNB1 mutations in sporadic desmoid tumors, and examines whether the presence of CTNNB1 mutations correlates with the disease outcome. CTNNB1 mutations (inclu… (full text at CIViC)

PMID 23960186 · Mullen et al., 2013 · Open in CIViC

An analysis of 260 typical desmoid tumors and 191 cases of spindle cell lesions (which can be difficult to distinguish morphologically from desmoid tumors) identified CTNNB1 mutations in 88% of desmoi… (full text at CIViC)

PMID 22766794 · Le Guellec et al., 2012 · Open in CIViC

Desmoid fibromatosis is a rare, nonmetastatic neoplasm marked by local invasiveness and relentless recurrence. Beta-Catenin deregulation has been commonly identified in sporadic desmoids. CTNNB1 mutat… (full text at CIViC)

PMID 18832571 · Lazar et al., 2008 · Open in CIViC

In this study a collection of 254 sporadic desmoid tumors were analysed for presence of mutations in CTNNB1. A mutation in exon 3 of the CTNNB1 gene was identified in 223 (88%) of the 254 cases. The C… (full text at CIViC)

PMID 22766794 · Le Guellec et al., 2012 · Open in CIViC

Desmoid fibromatosis is a rare, nonmetastatic neoplasm marked by local invasiveness and relentless recurrence. Beta-Catenin deregulation has been commonly identified in sporadic desmoids. CTNNB1 mutat… (full text at CIViC)

PMID 18832571 · Lazar et al., 2008 · Open in CIViC

In this study a collection of 254 sporadic desmoid tumors were analysed for presence of mutations in CTNNB1. A mutation in exon 3 of the CTNNB1 gene was identified in 223 (88%) of the 254 cases. The C… (full text at CIViC)

PMID 22766794 · Le Guellec et al., 2012 · Open in CIViC

Desmoid fibromatosis is a rare, nonmetastatic neoplasm marked by local invasiveness and relentless recurrence. Beta-Catenin deregulation has been commonly identified in sporadic desmoids. CTNNB1 mutat… (full text at CIViC)

PMID 18832571 · Lazar et al., 2008 · Open in CIViC

In this study a collection of 254 sporadic desmoid tumors were analysed for presence of mutations in CTNNB1. A mutation in exon 3 of the CTNNB1 gene was identified in 223 (88%) of the 254 cases. The C… (full text at CIViC)

PMID 22766794 · Le Guellec et al., 2012 · Open in CIViC

In this retrospective circulating biomarker study, in two cohorts of osteosarcoma patients, the cytokine in blood samples was measured by Luminex assay. The cutoff values for cytokine was determined b… (full text at CIViC)

PMID 27529817 · Flores et al., 2017 · Open in CIViC

A patient with a recurrent, metastatic uterine myxoid neoplasm and a DCTN1-ALK fusion experienced both clinical and radiographic improvement with crizotinib and pazopanib. The patient was initially di… (full text at CIViC)

PMID 26062823 · Subbiah et al., 2015 · Open in CIViC

In 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

This study, involving 20 pediatric patients, looked at molecular alterations in Lipofibromatosis. RNA sequencing detected EGFR::BRAF fusion in Patient 19 (6-year-old male). The fusion was picked up by… (full text at CIViC)

PMID 30310176 · Al-Ibraheemi et al., 2019 · Open in CIViC

In a cohort of 20 patients with dermatofibrosarcoma protuberans that were previously negative for COL1A1::PDGFB fusion with fluorescence in situ hybridization, RNA-sequencing was applied. novel and re… (full text at CIViC)

PMID 29955147 · Dadone-Montaudié et al., 2018 · Open in CIViC

To determine if EML4::NTRK3 fusions were recurrent in tumors types known to harbor ETV6::NTRK3 fusions, the authors screened 63 cases with varying diagnoses (infantile fibrosarcoma, congenital mesobla… (full text at CIViC)

PMID 29099503 · Church et al., 2018 · Open in CIViC

This case study detailed a 9-month-old male with congenital fibrosarcoma with subsequent metastasis to the lungs. The patient had a history of hemimelia in the same limb as the congenital fibrosarcoma… (full text at CIViC)

PMID 27148571 · Tannenbaum-Dvir et al., 2015 · Open in CIViC