Cancer Family
Epithelial Neoplasm
CI-CAN-00000016Explore in graph →
Non-malignant7,531 active trials118 approved drugs1,152 evidence items
- NCIt
- C3709
Loading cancer entity…
Loading cancer entity…
Cancer Family
CI-CAN-00000016Explore in graph →
Variants & evidence
2,364 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 |
|---|---|---|---|---|---|---|---|---|
| VHL W88L (c.263G>T)1 | ||||||||
| (predisposing) | Renal Cell CarcinomaCURATED_BROADER | Predisposing | C | Supports Uncertain Significance | 3 | submitted | EID6009Tissue analysis from 61 VHL patients, 30 sporadic renal cell carcinoma (RCC) tumor samples, and 6 sporadic RCC cell lines revealed 22 variants within VHL patients, 10 variants in tumor samples and var… (full text at CIViC) PMID 7977367 · Whaley et al., 1994 · Open in CIViC | civic |
| VHL W8* (c.24G>A)1 | ||||||||
| (oncogenic) | Renal Cell CarcinomaCURATED_BROADER | Oncogenic | C | Supports Oncogenicity | 1 | accepted | EID2986A single case (70 year-old named "3GT" in this study) with sporadic grade 3 renal cell carcinoma was observed with both W8* and G44D somatic variants. PMID 11536052 · Ma et al., 2001 · Open in CIViC | civic |
| VHL Y175* (c.525C>A)1 | ||||||||
| (oncogenic) | Clear Cell Renal Cell Carcinoma | Oncogenic | C | Supports Oncogenicity | 1 | submitted | EID6166This study analyzed tumor and non-tumor kidney tissue from 195 unrelated patients with sporadic clear cell renal cell carcinoma (CCRCC) for a relationship between VHL variants in sporadic CCRCC and 'g… (full text at CIViC) PMID 11505222 · Gallou et al., 2001 · Open in CIViC | civic |
| VHL Y185* (c.555C>G)1 | ||||||||
| (predisposing) | Renal Cell CarcinomaCURATED_BROADER | Predisposing | C | Supports Uncertain Significance | 3 | submitted | EID6015Tissue analysis from 61 VHL patients, 30 sporadic renal cell carcinoma (RCC) tumor samples, and 6 sporadic RCC cell lines revealed 22 variants within VHL patients, 10 variants in tumor samples and var… (full text at CIViC) PMID 7977367 · Whaley et al., 1994 · Open in CIViC | civic |
| VHL Y98FS (c.291_310del20)1 | ||||||||
| (oncogenic) | Clear Cell Renal Cell Carcinoma | Oncogenic | C | Supports Oncogenicity | 1 | accepted | EID1951This variant was detected in a single case with clear-cell renal cell carcinoma. The tumor had two somatic variants (c.1-1_20del21 and c.291_310del20). Either of these variants is predicted to be quit… (full text at CIViC) PMID 15932632 · van Houwelingen et al., 2005 · Open in CIViC | civic |
| VHL Y98N (c.292T>A)1 | ||||||||
| (predisposing) | Kidney CarcinomaALIAS | Predisposing | C | Supports Uncertain Significance | 3 | submitted | EID6898Tumors from 110 patients with sporadic renal carcinoma were analyzed for VHL mutations and loss of heterozygosity. 56 of the 98 samples from sporadic, clear cell renal carcinoma patients were identifi… (full text at CIViC) PMID 7915601 · Gnarra et al., 1994 · Open in CIViC | civic |
| MAML2 Fusion3 | ||||||||
| (diagnostic) | Salivary Gland Mucoepidermoid Carcinoma | Diagnostic | B | Supports Positive | 4 | accepted | EID11591In this 2010 study, the authors included 55 salivary gland mucoepidermoid carcinomas (MEC) and a control group of 36 potential MEC mimics, including Warthin tumors, oncocytomas, squamous cell carcinom… (full text at CIViC) PMID 20588178 · Seethala et al., 2010 · Open in CIViC | civic |
| 〃 | Salivary Gland Mucoepidermoid Carcinoma | Diagnostic | B | Supports Positive | 3 | accepted | EID11592In this 2013 study, the authors investigated 95 salivary gland mucoepidermoid carcinomas (MEC) using FFPE material. RT-PCR and FISH were utilized to detect CRTC1::MAML2 and CRTC3::MAML2 fusions, as we… (full text at CIViC) PMID 23035786 · Noda et al., 2013 · Open in CIViC | civic |
| 〃 | Salivary Gland Mucoepidermoid Carcinoma | Diagnostic | B | Supports | ||||
| MITF Fusion1 | ||||||||
| (diagnostic) | Renal Cell Carcinoma with MiT Translocations | Diagnostic | C | Supports Positive | 1 | accepted | EID11702Bakouny, et al. (2022) performed a comprehensive study of the molecular and clinical features of 90 translocation renal cell carcinomas (tRCCs), defined as subtype of kidney cancer driven by MiT/TFE g… (full text at CIViC) PMID 34986355 · Bakouny et al., 2022 · Open in CIViC | civic |
| NR4A3 Fusion4 | ||||||||
| (diagnostic) | Acinar Cell CarcinomaALIAS | Diagnostic | B | Supports Positive | 4 | accepted | EID12816In this 2023 study, the authors evaluated the diagnostic utility of NR4A3 immunohistochemistry in acinic cell carcinoma (AciCC) of the salivary gland. The cohort included 128 AciCCs, comprising 85 cla… (full text at CIViC) PMID 36469101 · Klubíčková et al., 2023 · Open in CIViC | civic |
| 〃 | Acinar Cell CarcinomaALIAS | Diagnostic | B | Supports Positive | 4 | accepted | EID12828In this 2019 study, the authors aimed to evaluate the usefulness of NR4A3 immunostaining and compare it with NR4A3 Fluorescence in situ hybridization (FISH) for the differential diagnosis of acinic ce… (full text at CIViC) PMID 31094928 · Haller et al., 2019 · Open in CIViC | civic |
| 〃 | Acinar Cell CarcinomaALIAS | |||||||
| NRG1 Fusion4 | ||||||||
| Afatinib | Pancreatic Adenocarcinoma | Predictive | C | Supports Sensitivity Response | 4 | accepted | EID6273In patients with KRAS wild-type (KRASwt) tumors druggable gene fusions have been identified. These alterations included recurrent NRG1 rearrangements predicted to drive PDAC development through aberra… (full text at CIViC) PMID 29802158 · Heining et al., 2018 · Open in CIViC | civic |
| Erlotinib + PertuzumabCombination | Pancreatic Adenocarcinoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID7534In patients with KRAS wild-type (KRASwt) tumors druggable gene fusions have been identified. These alterations included recurrent NRG1 rearrangements predicted to drive PDAC development through aberra… (full text at CIViC) PMID 29802158 · Heining et al., 2018 · Open in CIViC | civic |
| Zenocutuzumab-zbco | Lung Non-Small Cell CarcinomaCURATED_BROADER | |||||||
| NTRK1 Fusion3 | ||||||||
| Entrectinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 3 | rejected | EID29471378 cases of NSCLC were screened. One patient (frequency 0.1%) with a NTRK1 gene rearrangement (SQSTM1-NTRK1 Fusion) was treated with entrectinib in a phase 1 study. The patient had resolution of pri… (full text at CIViC) PMID 26565381 · Farago et al., 2015 · Open in CIViC | civic |
| Larotrectinib | Cholangiocarcinoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID6052In a Phase I/II study of Larotrectinib, highly selective TRK inhibitor, two patient with cholangiocarcinoma with NTRK fusion was enrolled. One of the two patient showed PR. PMID 29466156 · Drilon et al., 2018 · Open in CIViC | civic |
| Crizotinib + Larotrectinib + LestaurtinibSubstitutes | Lung Adenocarcinoma | Predictive | ||||||
| NUTM1 Fusion4 | ||||||||
| (diagnostic) | NUT CarcinomaALIAS | Diagnostic | B | Supports Positive | 4 | accepted | EID12050In this 2020 study, the authors included a total of 141 NUT carcinomas defined by rearrangement of NUTM1 detected by IHC, cytogenetics, FISH or sequencing. The median age at diagnosis was 24 years (ra… (full text at CIViC) PMID 32328562 · Chau et al., 2020 · Open in CIViC | civic |
| 〃 | NUT CarcinomaALIAS | Diagnostic | B | Supports Positive | 4 | accepted | EID12051In this 2004 study, the authors sought to identify carcinomas with translocation t(15;19)(q13, p13.1), which results in the novel oncogenic fusion BRD4::NUTM1 (described as NUT). The authors screened … (full text at CIViC) PMID 15483023 · French et al., 2004 · Open in CIViC | civic |
| 〃 | NUT CarcinomaALIAS | |||||||
| RET Fusion10 | ||||||||
| Alectinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID4850Alectinib is an anaplastic lymphoma kinase tyrosine kinase inhibitor (TKI) that also has anti-RET activity in vitro. 4 patients with metastatic RET-rearranged NSCLC were identified. 3 of 4 had receive… (full text at CIViC) PMID 27544060 · Lin et al., 2016 · Open in CIViC | civic |
| Cabozantinib | Lung Adenocarcinoma | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID4847RET rearrangements are found in 1-2% of non-small-cell lung cancers. Cabozantinib is a multikinase inhibitor with activity against RET that produced a 10% overall response in unselected patients with … (full text at CIViC) PMID 27825636 · Drilon et al., 2016 · Open in CIViC | civic |
| Pralsetinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | ||||||
| ROS1 Fusion13 | ||||||||
| Ceritinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 4 | submitted | EID11334In this phase II trial, 404 patients with advanced NSCLC underwent ROS1 pre-screening, and 32 patients with ROS1 rearrangement confirmed with FISH were enrolled. All patients except two were crizotini… (full text at CIViC) PMID 28520527 · Lim et al., 2017 · Open in CIViC | civic |
| Crizotinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 5 | accepted | EID7282In the ongoing phase 1 study PROFILE 1001, NSCLC patients with ROS1 rearrangement confirmed by FISH or RT-PCR received crizotinib 250mg twice daily. Among 53 patients, the ORR was 72% (95% CI, 58% to … (full text at CIViC) PMID 30980071 · Shaw et al., 2019 · Open in CIViC | civic |
| 〃 | Lung Non-Small Cell Carcinoma | |||||||
| TFE3 Fusion2 | ||||||||
| (diagnostic) | Renal Cell Carcinoma with MiT Translocations | Diagnostic | B | Supports Positive | 4 | accepted | EID10150This study screened 983 Renal Cell Carcinoma (RCC) cases and identified 65 tumors with morphologic features suspicious of RCC with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC). TFE3 immunohistoc… (full text at CIViC) PMID 26169997 · He et al., 2015 · Open in CIViC | civic |
| 〃 | TFE3-Rearranged Renal Cell Carcinoma | Diagnostic | B | Supports Positive | 4 | accepted | EID11533Argani. et al,. (2016) included a total of 60 cases with known TFE3 gene rearrangements in this study of Xp11 translocation positive neoplasms (47 RCC, 8 PEComa, and 5 melanotic renal cancers). The ag… (full text at CIViC) PMID 26975036 · Argani et al., 2016 · Open in CIViC | civic |
Data updated 4 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 |
EID11593In this 2009 study, the authors investigated 101 mucoepidermoid carcinomas (MECs) and 89 non-MEC salivary gland tumors using RT-PCR to detect CRTC1::MAML2, CRTC2::MAML2, and CRTC3::MAML2 fusions. The … (full text at CIViC) PMID 19749740 · Nakayama et al., 2009 · Open in CIViC |
| civic |
| Diagnostic |
| B |
| Supports Positive |
| 3 |
| accepted |
EID12336In this 2022 study the authors included 4 cases of sinonasal acinic cell carcinoma and 10 acinic cell carcinoma cases of the parotid gland. All four sinonasal patients were male, aged from 27 to 70 ye… (full text at CIViC) PMID 34873305 · Wang et al., 2022 · Open in CIViC |
| civic |
| 〃 | Acinar Cell CarcinomaALIAS | Diagnostic | C | Supports Positive | 3 | accepted | EID12337In this 2023 study the authors included 4 cases of acinic cell carcinoma of the salivary gland. The age of the patients ranged from 13 to 16 years, and included 2 males and 2 females. Immunohistochemi… (full text at CIViC) PMID 36169793 · Cole et al., 2023 · Open in CIViC | civic |
| Predictive |
| A |
| Supports Sensitivity Response |
| 4 |
| accepted |
EID12246On December 4, 2024, the Food and Drug Administration granted accelerated approval to zenocutuzumab-zbco (Bizengri, Merus N.V.) for adults with advanced, unresectable, or metastatic non-small cell lun… (full text at CIViC) PMID 39908431 · Schram et al., 2025 · Open in CIViC |
| civic |
| 〃 | Pancreatic Adenocarcinoma | Predictive | A | Supports Sensitivity Response | 4 | accepted | EID12247On December 4, 2024, the Food and Drug Administration granted accelerated approval to zenocutuzumab-zbco (Bizengri, Merus N.V.) for adults with advanced, unresectable, or metastatic non-small cell lun… (full text at CIViC) PMID 39908431 · Schram et al., 2025 · Open in CIViC | civic |
| D |
| Supports Sensitivity Response |
| 3 |
| accepted |
EID1019Two patients were found to harbor gene fusions maintaining the kinase domain of NTRK1 (3' partner), specifically: MPRIP-NTRK1 and CD74-NTRK1. Both of these fusions lead to constitutive kinase activit… (full text at CIViC) PMID 24162815 · Vaishnavi et al., 2013 · Open in CIViC |
| civic |
| C |
| Supports Positive |
| 2 |
| accepted |
EID12009RNA sequencing identified NUTM1 fusions in six patients with primary undifferentiated soft tissue and visceral tumors. Fusions included BRD4::NUTM1 (three cases), BRD3::NUTM1, MXD1::NUTM1, and BCORL1… (full text at CIViC) PMID 29356724 · Dickson et al., 2018 · Open in CIViC |
| civic |
| Molibresib | NUT CarcinomaALIAS | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID12055NUT carcinoma is known to be driven by BET fusion proteins, including BRD4::NUTM1 and BRD3::NUTM1, raising the possibility of response to BET inhibitors. Of 19 patients with NUT carcinoma treated wit… (full text at CIViC) PMID 32328561 · Piha-Paul et al., 2020 · Open in CIViC | civic |
| A |
| Supports Sensitivity Response |
| 4 |
| accepted |
EID11233In an open-label, phase 1/2 trial on 87 patients with NSCLC including RET-fusion were given 400 mg once-daily oral pralsetinib, with the outcome measured as overall response rate. Overall responses we… (full text at CIViC) PMID 34118197 · Gainor et al., 2021 · Open in CIViC |
| civic |
| Selpercatinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 4 | submitted | EID11318In a follow up publication on the NCT03157128 clinical trial, results of 316 RET fusion positive NSCLC patients treated with the RET inhibitor selpercatinib were reported. Of note, this includes 144 … (full text at CIViC) PMID 36122315 · Drilon et al., 2023 · Open in CIViC | civic |
| 〃 | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID7066In this phase 1 trial, 57 patients with RET fusion- or mutation positive cancers were treated in 7 dose levels. Among patients with RET-Fusion positive NSCLC, objective response rates were 65% (n = 17… (full text at CIViC) | civic |
| 〃 | Thyroid Gland Papillary CarcinomaALIAS | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID7069In this phase 1 trial, patients with RET-fusion or mutation positive cancers were treated with the selective RET-inhibitor selpercatinib (LOXO-292). Among the 6 patients with RET-fusion positive papil… (full text at CIViC) | civic |
| 〃 | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID8850Patients with RET-fusion positive non-small cell lung cancer were enrolled in this phase 1-2 trial of RET inhibitor selpercatinib. Among 105 consecutively enrolled patients who had previously received… (full text at CIViC) PMID 32846060 · Drilon et al., 2020 · Open in CIViC | civic |
| 〃 | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID12135In this arm of the trial (NCT03157128), the intracranial objective response rate (iORR) was assessed in 80 patients with RET fusion-positive non-small cell lung cancer (NSCLC) treated with selpercatin… (full text at CIViC) PMID 34088726 · Subbiah et al., 2021 · Open in CIViC | civic |
| Vandetanib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID4848RET rearrangements involving RET are found in about 1% of NSCLC and define a unique molecular subset. This study was a multi-center, open-label, phase II clinical trial to examine the safety and effi… (full text at CIViC) PMID 27803005 · Lee et al., 2017 · Open in CIViC | civic |
| 〃 | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID4849We aimed to assess the efficacy and safety of vandetanib in patients with advanced RET-rearranged NSCLC. In this open-label, multicentre, phase 2 trial (LURET), patients with advanced RET-rearranged N… (full text at CIViC) PMID 27825616 · Yoh et al., 2017 · Open in CIViC | civic |
| Predictive |
| A |
| Supports Sensitivity Response |
| 4 |
| accepted |
EID688An expansion cohort of a phase-1 Crizotinib study found a 72% objective response rate among 50 patients with ROS1 rearrangement. The study did not compare to patients without rearrangement that were t… (full text at CIViC) PMID 25264305 · Shaw et al., 2014 · Open in CIViC |
| civic |
| 〃 | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 4 | accepted | EID7933A phase 2 clinical trial studied the efficacy of Crizotinib in East Asian NSCLC patients positive for ROS1 rearrangements (detected by RT-PCR AmoyDx assay) and negative for ALK1 rearrangements. Of the… (full text at CIViC) PMID 29596029 · Wu et al., 2018 · Open in CIViC | civic |
| 〃 | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 3 | accepted | EID1030In a retrospective study, 30 patients were evaluated after crizotinib treatment for stage IV NSCLC confirmed for ROS1 rearrangement. On crizotinib, 24 of the patients achieved objective responses incl… (full text at CIViC) PMID 25667280 · Mazières et al., 2015 · Open in CIViC | civic |
| 〃 | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 3 | submitted | EID8916The phase 2 EUCROSS trial (NCT02183870) examined the safety and efficacy of 250mg crizotinib twice daily in European ROS1-rearranged NSCLC patients. ROS1 status was assessed by break-apart FISH or bot… (full text at CIViC) PMID 30978502 · Michels et al., 2019 · Open in CIViC | civic |
| 〃 | Minimally Invasive Lung AdenocarcinomaALIAS | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID1443Study describes a 31-year old male, never-smoker patient with advanced NSCLC. Testing did not indicate tumor had EGFR mutation or ALK rearrangement. First line erlotininb did not yield a response. Aft… (full text at CIViC) PMID 22215748 · Bergethon et al., 2012 · Open in CIViC | civic |
| 〃 | Colorectal Adenocarcinoma | Predictive | E | Supports Sensitivity Response | 2 | accepted | EID1301A tissue microarray of 268 patients with histologically confirmed colorectal adenocarcinoma was performed to search for ROS1 gene fusion events to guide potential targeted therapy in future cases. Bre… (full text at CIViC) PMID 24296758 · Aisner et al., 2014 · Open in CIViC | civic |
| Entrectinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 5 | accepted | EID11123Describes a meta-analysis of three separate phase I or II trials: ALKA-372-001, STARTRK-1, and STARTRK-2. All of these evaluate entrectinib in ROS-1 fusion-positive NSCLC (locally advanced or metastat… (full text at CIViC) PMID 31838015 · Drilon et al., 2020 · Open in CIViC | civic |
| 〃 | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 3 | accepted | EID2951Two phase I studies included 119 patients with advanced or metastatic solid tumors, which included 28 patients with ROS1 rearrangements (n=28) where the overall safety and antitumor activity of entrec… (full text at CIViC) PMID 28183697 · Drilon et al., 2017 · Open in CIViC | civic |
| Lorlatinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 4 | submitted | EID11335In this open-label, single-arm, phase 1-2 trial, 364 patients with histologically or cytologically confirmed advanced ROS1-positive NSCLC, with or without CNS metastases were assessed. Of them, 69 wit… (full text at CIViC) PMID 31669155 · Shaw et al., 2019 · Open in CIViC | civic |
| Repotrectinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 4 | submitted | EID12021In a phase 1-2 trial (NCT03093116), the ROS1/ALK/NTRK inhibitor repotrectinib demonstrated efficacy in NSCLC with ROS1 fusions. Among the 71 ROS1-inhibitor-naive patients, 56 (79%; 95% CI, 68 to 88) h… (full text at CIViC) PMID 38197815 · Drilon et al., 2024 · Open in CIViC | civic |
| Taletrectinib | Lung Non-Small Cell CarcinomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID12240The TRUST-I study (NCT04395677) was a phase II, multicenter, single-arm, open-label trial conducted in China to evaluate taletrectinib for ROS1 fusion-positive, locally advanced, or metastatic non-sma… (full text at CIViC) PMID 38822758 · Li et al., 2024 · Open in CIViC | civic |