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Profile Name RET fusion
Gene Variant Detail

RET fusion (unknown)

Relevant Treatment Approaches RET Inhibitor

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Vandetanib Phase II Actionable In a Phase II trial, Caprelsa (vandetanib) treatment resulted in a disease control rate of 88% (15/17), median progression-free survival (PFS) of 4.7 months, and partial response in 53% (9/17) of non-small cell lung adenocarcinoma patients harboring RET fusions (J Clin Oncol 34, 2016 (suppl; abstr 9012)). detail...
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Vandetanib Phase II Actionable In a Phase II trial, Caprelsa (vandetanib) treatment resulted in partial remission in 17% (3/18) and stable disease in 44% (8/18) of non-small cell lung adenocarcinoma patients harboring RET fusions (J Clin Oncol 34, 2016 (suppl; abstr 9013)). detail...
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Selpercatinib FDA approved - On Companion Diagnostic Actionable In a Phase I/II trial (LIBRETTO-001) that supported FDA approval, Retevmo (selpercatinib) treatment resulted in an objective response rate (ORR) of 64% (64/105) in patients with RET fusion-positive non-small cell lung cancer, with a median duration of response of 17.5 months, ORR was 85% (33/39) and 91% (10/11) in previously treated patients and patients with measurable CNS metastasis, respectively (PMID: 32846060; NCT03157128). detail... 32846060 detail...
RET fusion papillary thyroid carcinoma sensitive RET Inhibitor Selpercatinib Phase I Actionable In a Phase I trial, Retevmo (selpercatinib) treatment resulted in an objective response rate of 83% (5/6) in RET fusion-positive papillary thyroid cancer patients (J Clin Oncol 36, 2018 (suppl; abstr 102); NCT03157128). detail...
RET fusion thyroid cancer sensitive RET Inhibitor Selpercatinib FDA approved - On Companion Diagnostic Actionable In a Phase I/II trial (LIBRETTO-001) that supported FDA approval, Retevmo (selpercatinib) treatment resulted in an objective response rate of 79% (15/19), including one complete and 14 partial responses, with a median response duration of 18.4 months, and median progression-free survival of 20.1 months in adult and pediatric patients 12 years and older with RET fusion-positive thyroid cancer who were previously treated (PMID: 32846061; NCT03157128). detail... detail... 32846061
RET fusion papillary thyroid carcinoma sensitive RET Inhibitor Pralsetinib Phase Ib/II Actionable In a Phase I/II trial (ARROW), Gavreto (pralsetinib) treatment was well-tolerated, resulted in an objective response rate of 75% (9/12) and a median duration of response of 14.5 months in patients with RET fusion positive papillary thyroid cancer (J Clin Oncol 38, no. 15_suppl (May 20, 2020) 109-109; NCT03037385). detail...
RET fusion Advanced Solid Tumor predicted - sensitive RET Inhibitor Pralsetinib Phase Ib/II Actionable In a Phase I/II trial (ARROW), Gavreto (pralsetinib) treatment was well tolerated in patients with advanced solid tumors harboring RET fusions, and led to an overall response rate of 57% (13/23, 3 complete and 10 partial responses), a clinical benefit rate of 70% (16/23), a disease control rate of 83% (19/23), a median duration of response of 11.7 months, a median progression-free survival of 7.4 months, and a median overall survival of 13.6 months (PMID: 35962206; NCT03037385). 35962206
RET fusion pancreatic cancer predicted - sensitive RET Inhibitor Pralsetinib Case Reports/Case Series Actionable In a Phase I/II trial (ARROW), Gavreto (pralsetinib) treatment was well-tolerated, resulted in a partial response in 2 of 2 patients with RET fusion positive pancreatic cancer, with a duration of response of 5.5 and 7.4 months, respectively (J Clin Oncol 38, no. 15_suppl (May 20, 2020) 109-109; NCT03037385). detail...
RET fusion intrahepatic cholangiocarcinoma sensitive RET Inhibitor Pralsetinib Case Reports/Case Series Actionable In a Phase I/II trial (ARROW), Gavreto (pralsetinib) treatment was well-tolerated, resulted in a partial response in a patient with RET fusion-positive intrahepatic cholangiocarcinoma, with a duration of response of 7.5 months (J Clin Oncol 38, no. 15_suppl (May 20, 2020) 109-109; NCT03037385). detail...
RET fusion colon cancer predicted - sensitive RET Inhibitor Pralsetinib Case Reports/Case Series Actionable In a Phase I/II trial (ARROW), Gavreto (pralsetinib) treatment was well-tolerated, resulted in stable disease in 2 patients with RET fusion-positive colon cancer, with a duration of response of 7.3 and 9.3 months, respectively (J Clin Oncol 38, no. 15_suppl (May 20, 2020) 109-109; NCT03037385). detail...
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Pralsetinib FDA approved - On Companion Diagnostic Actionable In a Phase I/II trial (ARROW) that supported FDA approval, Gavreto (pralsetinib) treatment was well-tolerated, resulting in an objective response rate of 61% (53/87) and 70% (19/27) in previously treated and treatment-naive patients with RET fusion-positive non-small cell lung cancer, respectively (PMID: 34118197; NCT03037385). detail... 34118197 detail...
RET fusion papillary thyroid carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with papillary thyroid carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion follicular thyroid carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with follicular thyroid carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion oncocytic carcinoma of the thyroid sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with thyroid Hurthle cell carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion medullary thyroid carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with medullary thyroid carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion anaplastic thyroid carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with anaplastic thyroid carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion thyroid cancer sensitive RET Inhibitor Pralsetinib FDA approved Actionable In a Phase I/II trial (ARROW) that supported FDA approval, Gavreto (pralsetinib) treatment was well-tolerated, and resulted in an overall response rate (ORR) of 89% (8/9) in patients with advanced or metastatic RET fusion-positive thyroid cancer, with a median duration of response of 14.5 months, and 67% of responding patients continuing treatment (PMID: 34118198; NCT03037385). 34118198 detail...
RET fusion lung non-small cell carcinoma unknown unspecified immune checkpoint inhibitor Clinical Study - Cohort Actionable In a retrospective clinical study, patients with non-small cell lung cancer harboring rare targetable drivers (RTD) (BRAF, ERBB2/3, RET, MET, ROS1, NTRK) who received immune checkpoint inhibitors (ICI) achieved longer median overall survival (mOS) (32 vs 13 mo, p=0.01) compared to those who did not receive ICI, mOS was 25.7 mo in patients harboring RET fusions (n=4), although RTD type was not associated with OS in a univariate analysis (PMID: 30268448). 30268448
RET fusion papillary thyroid carcinoma sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines as systemic therapy for patients with papillary thyroid carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion medullary thyroid carcinoma sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines as systemic therapy for patients with medullary thyroid carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion anaplastic thyroid carcinoma sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines as systemic therapy for patients with anaplastic thyroid carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion follicular thyroid carcinoma sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines for patients with follicular thyroid carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion oncocytic carcinoma of the thyroid sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines for patients with thyroid Hurthle cell carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion Erdheim-Chester disease sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as first-line or subsequent-line therapy for patients with Erdheim-Chester disease harboring RET fusions (NCCN.org). detail...
RET fusion gallbladder cancer sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines as primary (category 2B) and subsequent-line therapy (category 2B) for patients with unresectable or metastatic biliary tract cancer harboring RET fusions, including gallbladder cancer (NCCN.org). detail...
RET fusion intrahepatic cholangiocarcinoma sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines as primary (category 2B) and subsequent-line therapy (category 2B) for patients with unresectable or metastatic biliary tract cancer harboring RET fusions, including intrahepatic cholangiocarcinoma (NCCN.org). detail...
RET fusion extrahepatic bile duct carcinoma sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines as primary (category 2B) and subsequent-line therapy (category 2B) for patients with unresectable or metastatic biliary tract cancer harboring RET fusions, including extrahepatic cholangiocarcinoma (NCCN.org). detail...
RET fusion differentiated high-grade thyroid carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with advanced or metastatic differentiated thyroid gland carcinoma harboring RET fusions (PMID: 31549998, PMID: 35491008; ESMO.org). 35491008 31549998 detail...
RET fusion differentiated high-grade thyroid carcinoma sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines for adult or pediatric patients 12 years or older with radioactive iodine-refractory, advanced or metastatic differentiated thyroid gland carcinoma harboring RET fusions (PMID: 31549998, PMID: 35491008; ESMO.org). 35491008 31549998 detail...
RET fusion Advanced Solid Tumor sensitive RET Inhibitor Selpercatinib FDA approved - On Companion Diagnostic Actionable In a Phase I/II trial (LIBRETTO-001) that supported FDA approval, Retevmo (selpercatinib) treatment resulted in an objective response rate of 44% (18/41, 2 complete responses, 16 partial responses), a clinical benefit rate of 63% (26/41), a median duration of response of 24.5 months, and a median progression-free survival of 13.2 months in patients with RET fusion-positive advanced solid tumors (PMID: 36108661; NCT03157128). 36108661 detail...
RET fusion pancreatic cancer predicted - sensitive RET Inhibitor Selpercatinib Case Reports/Case Series Actionable In a Phase I/II trial (LIBRETTO-001), Retevmo (selpercatinib) treatment resulted in an objective response rate (ORR) of 43.9% (18/41, 2 complete responses, 16 partial responses) with a median duration of response (mDOR) of 24.5 months in patients with RET fusion-positive advanced or metastatic solid tumors, and ORR and mDOR in patients with pancreatic cancer were 54.5% (6/11) and not reached, respectively (PMID: 36108661; NCT03157128). 36108661
RET fusion colon cancer sensitive RET Inhibitor Selpercatinib Case Reports/Case Series Actionable In a Phase I/II trial (LIBRETTO-001), Retevmo (selpercatinib) treatment resulted in an objective response rate (ORR) of 43.9% (18/41, 2 complete responses, 16 partial responses) with a median duration of response (mDOR) of 24.5 months in patients with RET fusion-positive advanced or metastatic solid tumors, and ORR and mDOR in patients with colon cancer were 20.0% (2/10) and 9.4 months, respectively (PMID: 36108661; NCT03157128). 36108661
RET fusion hepatocellular carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as subsequent-line therapy (category 2B) for patients with hepatocellular carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion cholangiocarcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as primary (category 2B) or subsequent-line therapy (category 2A) for patients with unresectable or metastatic cholangiocarcinoma harboring RET fusions (NCCN.org). detail...
RET fusion small intestine adenocarcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with metastatic small bowel adenocarcinoma with RET fusion (NCCN.org). detail...
RET fusion rectum cancer sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with metastatic rectum cancer harboring RET fusion (NCCN.org). detail...
RET fusion Adenocarcinoma of Unknown Primary sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with adenocarcinoma of unknown primary harboring RET fusions (NCCN.org). detail...
RET fusion Squamous Cell Carcinoma of Unknown Primary sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with squamous cell carcinoma of unknown primary harboring RET fusions (NCCN.org). detail...
RET fusion colon cancer sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with metastatic colon cancer harboring RET fusions (NCCN.org). detail...
RET fusion pancreatic adenocarcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as first-line or subsequent therapy for patients with locally advanced, metastatic, or recurrent pancreatic adenocarcinoma harboring RET fusions (NCCN.org). detail...
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Pralsetinib Clinical Study Actionable In a retrospective study, Gavreto (pralsetinib) treatment resulted in an objective response rate (ORR) of 61% (39/59, 2 complete responses (CR), 37 partial responses (PR)) and a disease control rate (DCR) of 79.7% in non-small cell lung cancer patients harboring RET fusions, and an intracranial ORR of 66.7% (4/6, 3 CR and 1 PR), an intracranial DCR of 100% in patients with measurable brain metastases (PMID: 36379124). 36379124
RET fusion cervical cancer sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as second-line or subsequent therapy for patients with cervical cancer harboring RET fusions (NCCN.org). detail...
RET fusion salivary gland cancer sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with recurrent, unresectable, or metastatic salivary gland tumors harboring RET fusions (NCCN.org). detail...
RET fusion neuroendocrine tumor sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines for patients with unresectable or metastatic extrapulmonary poorly differentiated neuroendocrine carcinoma, large or small cell carcinoma, or mixed neuroendocrine/non-neuroendocrine neoplasms harboring RET fusions who have progressed on or following prior treatment (NCCN.org). detail...
RET fusion ovary epithelial cancer sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as systemic therapy for patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer harboring a RET fusion (NCCN.org). detail...
RET fusion sarcoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as first-line therapy for patients with advanced or metastatic soft tissue sarcoma harboring RET fusions (NCCN.org). detail...
RET fusion esophagus squamous cell carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as second-line or subsequent therapy for patients with locally advanced, recurrent, or metastatic esophageal squamous cell carcinoma harboring RET fusions (NCCN.org). detail...
RET fusion esophagus adenocarcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as second-line or subsequent therapy for patients with locally advanced, recurrent, or metastatic esophageal adenocarcinoma harboring RET fusions (NCCN.org). detail...
RET fusion gastroesophageal junction adenocarcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as second-line or subsequent therapy for patients with locally advanced, recurrent, or metastatic gastroesophageal junction adenocarcinoma harboring RET fusions (NCCN.org). detail...
RET fusion stomach cancer sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as second-line or subsequent therapy for patients with unresectable locally advanced, recurrent, or metastatic gastric cancer harboring RET fusion (NCCN.org). (NCCN.org). detail...
RET fusion ampulla of Vater adenocarcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as first-line or subsequent therapy for patients with metastatic ampullary adenocarcinoma harboring RET fusions (NCCN.org). detail...
RET fusion lung non-small cell carcinoma predicted - sensitive RET Inhibitor HS-10365 Phase I Actionable In a Phase I trial, HS-10365 treatment demonstrated safety and resulted in a response rate of 70% (21/30) and a disease control rate of 96.7% (29/30) in patients with non-small cell lung cancer harboring RET fusions (Cancer Res (2023) 83 (8_Supplement): CT201; NCT05207787). detail...
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as a first-line therapy for patients with metastatic non-small cell lung cancer harboring RET fusions (PMID: 36872130; ESMO.org). 36872130 detail...
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Pralsetinib Guideline Actionable Gavreto (pralsetinib) is included in guidelines as a first-line therapy for patients with metastatic non-small cell lung cancer harboring RET fusions (PMID: 36872130; ESMO.org). detail... 36872130
RET fusion gallbladder cancer sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as primary (category 2B) and subsequent-line therapy for patients with unresectable or metastatic biliary tract cancer harboring RET fusions, including gallbladder cancer (NCCN.org). detail...
RET fusion intrahepatic cholangiocarcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as primary (category 2B) and subsequent-line therapy for patients with unresectable or metastatic biliary tract cancer harboring RET fusions, including intrahepatic cholangiocarcinoma (NCCN.org). detail...
RET fusion extrahepatic bile duct carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as primary (category 2B) and subsequent-line therapy for patients with unresectable or metastatic biliary tract cancer harboring RET fusions, including extrahepatic cholangiocarcinoma (NCCN.org). detail...
RET fusion vaginal carcinoma sensitive RET Inhibitor Selpercatinib Guideline Actionable Retevmo (selpercatinib) is included in guidelines as second-line or subsequent therapy for patients with recurrent or metastatic vaginal squamous cell carcinoma or adenocarcinoma harboring an RET fusion (NCCN.org). detail...
RET fusion Advanced Solid Tumor sensitive RET Inhibitor Selpercatinib FDA approved - On Companion Diagnostic Actionable In a Phase I/II trial (LIBRETTO-121) that supported FDA approval, Retevmo (selpercatinib) treatment was safe in pediatric patients of 2 years or older with solid tumors harboring RET fusion (n=2) or mutations (n=9), and resulted in unconfirmed partial responses in 4 patients, stable disease in 6 patients (two lasting >/=16 weeks) (JCO 39, 10009-10009(2021); NCT03899792). detail... detail... detail...
RET fusion thyroid cancer sensitive RET Inhibitor Selpercatinib FDA approved - On Companion Diagnostic Actionable In a Phase I/II trial (LIBRETTO-121) that supported FDA approval, Retevmo (selpercatinib) treatment was safe in pediatric patients of 2 years or older with solid tumors (8 medullary thyroid cancer, 2 papillary thyroid cancer, 1 osteosarcoma) harboring RET fusion (n=2) or mutations (n=9), and resulted in unconfirmed partial responses in 4 patients, stable disease in 6 patients (two lasting >/=16 weeks) (JCO 39, 10009-10009(2021); NCT03899792). detail... detail... detail...
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Selpercatinib Phase III Actionable In a Phase III trial (LIBRETTO-431), first-line Retevmo (selpercatinib) treatment resulted in superior median progression-free survival (PFS) compared to platinum-based chemotherapy with (24.8 vs 11.2 mo, HR=0.46, p<0.001) or without ((24.8 vs 11.2 mo, HR=0.48, p<0.001) Keytruda (pembrolizumab) in advanced non-small cell lung cancer patients harboring a RET fusion, and improved intracranial (IC) response rate (82.4% vs 58.3%) and median IC PFS (16.1 vs 10.4 mo) (PMID: 38807655; NCT04194944). 38807655
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor Pralsetinib Clinical Study Actionable In a retrospective study, Gavreto (pralsetinib) treatment resulted in an overall response rate of 55.6%, a disease control rate of 72.2%, a median progression-free survival of 10.7 months, and overall survival of 21.2 months in patients with metastatic non-small cell lung cancer harboring a RET fusion (n=36) including KIF5B-RET (n=13) and CCDC6-RET (n=2) (PMID: 39241296). 39241296
RET fusion lung non-small cell carcinoma sensitive RET Inhibitor SY-5007 Phase I Actionable In a Phase I trial, SY-5007 therapy was tolerable and led to an objective response rate (ORR) of 57.8% (67/116, 67 partial responses), disease control rate (DCR) of 95.7% (111/116), and median progression-free survival of 21.1 mo in advanced solid tumor patients with RET fusions or alterations, and an ORR of 64.1% (41/64) and DCR of 96.9% (62/64) at the phase II dose in non-small cell lung cancer patients with KIF5B (n=51), CCDC6 (n=12), NCOA4 (n=1), or other RET fusions (n=4) (PMID: 39489747; NCT05278364). 39489747