[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"fibrolamellar-carcinoma\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:fibrolamellar-carcinoma":32},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,4,0,[8,46,131,160],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":4,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":4,"maxAge":17,"enrollmentInfo":18,"targetDuration":4,"studyType":21,"phases":22,"briefSummary":25,"conditions":26,"keywords":4,"overallStatus":33,"whyStopped":4,"lastUpdateSubmitDate":34,"lastUpdatePostDateStruct":35,"startDateStruct":38,"completionDateStruct":40,"leadSponsor":42,"locationsCount":45},"100475241","phase-1-safety-and-efficacy-of-cyclophosphamide-sorafenib-bevacizumab-and-atezolizumab-in-pediatric-solid-tumor-patients-100475241",false,"NCT05468359","Safety and Efficacy of Cyclophosphamide, Sorafenib, Bevacizumab, and Atezolizumab in Pediatric Solid Tumor Patients","ANGIO-A: Safety and Tolerability of Oral Cyclophosphamide and Sorafenib With Intravenous Bevacizumab With the Addition of Atezolizumab in Pediatric Solid Tumor Patients","Inclusion Criteria:\n\n* Age: Patients must be \\\u003C 30 years at the time of enrollment on study.\n* Willingness to enroll on the St. Jude Molecular Analysis of Solid Tumors (MAST) study.\n* Diagnosis\n* Part 1: Patients with refractory or recurrent (relapsed) solid tumors accessible by biopsy for which there is no standard therapy are eligible.\n* Part 2: Patients with one of the following diagnoses:\n* Biopsy accessible refractory or recurrent (relapsed) hepatocellular carcinoma\n* Biopsy accessible refractory or recurrent (relapsed)or FL-HCC, DSRCT or non-CNS MRT.\n* Performance level: Karnofsky \\> 50 for patients \\> 16 years of age and Lansky \\> 50 for patients \\\u003C 16 years of age (See Appendix III). Note: Patients who are unable to walk because of paralysis, but who are up in a wheelchair, will be considered ambulatory for the purpose of assessing the performance score.\n* Disease status: Patients must tumors that are unresectable and have either measurable or evaluable disease that is accessible by biopsy\n* Organ function: Must have adequate organ and bone marrow function as defined by the following parameters:\n* Patients with solid tumor not metastatic to bone marrow:\n\n  * Peripheral absolute neutrophil count (ANC) \\>1,000\u002Fmm3\n  * Platelet count \\> 75,000\u002Fmm3 (no transfusion within 7 days of enrollment)\n  * Hemoglobin \\> 8 g\u002FdL (with or without support)\n* Patients with solid tumor metastatic to bone marrow will be eligible for study but not evaluable for hematologic toxicity. These patients must not be known to be refractory to red cell or platelet transfusions. At least 2 of every cohort of 3 patients must be evaluable for hematologic toxicity. If dose limiting hematologic toxicity is observed at any dose level, all subsequent patients enrolled must be evaluable for hematologic toxicity.\n* Adequate renal function defined as serum creatinine based on age as shown in Table 1, or creatinine clearance or radioisotope GFR 50 ml\u002Fmin\u002F1.73m2 (GFR 40 ml\u002Fmin\u002F1.73m2 if \\\u003C 2 years of age).\n* Adequate hepatic function defined as total bilirubin \\\u003C 5x upper limit of normal (ULN) and AST\u002FALT \\\u003C 3 x ULN for age.\n* Adequate cardiac function defined as shortening fraction \\> 28% OR ejection fraction of ≥ 47% by echocardiogram.\n* Adequate blood clotting defined as PT\u002FPTT \\\u003C 1.2 x ULN without factor replacement products for 7 days\n* Females of childbearing potential and males able to father a child must be willing to practice acceptable methods of birth control to prevent pregnancy during the study and for at least 5 months after last dose of therapy.\n* Patients must have fully recovered from the acute toxic effects of chemotherapy, immunotherapy, surgery, or radiotherapy prior to entering this study:\n* Myelosuppressive chemotherapy: Patient has not received myelosuppressive chemotherapy within 1 weeks of enrollment onto this study (within 2 weeks of estimated therapy start date) (4 weeks if prior nitrosourea).\n* Hematopoietic growth factors: At least 7 days must have elapsed since the completion of therapy with a growth factor. At least 14 days must have elapsed after receiving pegfilgrastim.\n* Biologic (anti-neoplastic agent): At least 7 days must have elapsed since completion of therapy with a biologic agent. For agents that have known adverse events occurring beyond 7 days after administration, this period prior to enrollment must be extended beyond the time during which adverse events are known to occur.\n* Monoclonal antibodies: At least 14 days (at least 21 days from therapy start date) must have elapsed since the completion of therapy with a monoclonal antibody.\n* Radiotherapy: At least 1 week (2 weeks from estimated therapy start date) must have elapsed since any irradiation; at least 5 weeks (at least 6 weeks from estimated therapy start date) must have elapsed since craniospinal RT or substantial bone marrow irradiation.\n* Chemoembolization: at least 21 days (28 days from estimated therapy start date) must have elapsed since the completion of chemoembolization\n* Radioembolization: at least 21 days (28 days from estimated therapy start date) must have elapsed since the completion of radioembolization\n* Cardiac disease or hypertension: Patients must not have a history of myocardial - infarction, severe or unstable angina, or severe peripheral vascular disease. Hypertension must be well controlled on stable doses of medication for at least two weeks.\n* Female participant who is post-monarchal must have a negative urine or serum pregnancy test.\n* Life expectancy of at least 8 weeks\n\nExclusion Criteria:\n\n* Pregnant or breastfeeding.\n* Currently receiving other investigational drugs.\n* Unwilling or unable to comply with the safety monitoring requirements of this protocol.\n* Tumor not safely accessible by biopsy\n* Inability or unwillingness of research participant or legal guardian \u002F representative to give written informed consent.\n* Surgical procedures and serious or non-healing wounds: patients with a documented, chronic non-healing wound, ulcer, or bone fracture or history of a major surgical procedure or significant traumatic injury within 28 days prior to beginning therapy are excluded due to preclinical evidence supporting the potential for delayed wound healing.\n* Minor surgical procedures for minimally invasive biopsies will be allowed. For minor surgeries, the wound must be healed, and 7 days elapsed since surgery. For procedures such as the placement of an indwelling IV catheter, it is recommended that bevacizumab be postponed for at least 24 hours after the procedure.\n* Thrombosis: Patients must not have a deep venous or arterial thrombosis (including pulmonary embolism) within the last three months prior to study entry and must not have a known thrombophilic condition (i.e., protein S, protein C or antithrombin III deficiency, Factor V Leiden, Factor II G20210A mutation, homocysteinemia or antiphospholipid antibody syndrome).","ALL","30 Years",{"count":19,"type":20},64,"ESTIMATED","INTERVENTIONAL",[23,24],"PHASE1","PHASE2","This is a phase I\u002FII study to evaluate the safety of combining intravenous (IV) atezolizumab and bevacizumab every three weeks, with daily oral cyclophosphamide and pharmacokinetic (PK)-guided sorafenib in children and adolescent and young adults (AYA) with relapsed or refractory solid malignancies (Part 1), and then evaluate the response rate of this combination in children, AYA with relapsed or refractory fibrolamellar carcinoma (FLC) and other rare solid malignancies (Part 2).\n\nPrimary Objectives Part 1\n\n* To establish the safety associated with the administration of the combination of cyclophosphamide, PK-guided sorafenib, bevacizumab and atezolizumab in children and AYA with relapsed or refractory solid tumors\n* To determine if sorafenib systemic exposure can be successfully targeted to an AUC between 20 and 55 hr·µg\u002FmL by Day 21 of cycle 1 in 60% of evaluable patients, when given in combination with cyclophosphamide, bevacizumab, and atezolizumab in children and AYA with relapsed or refractory solid tumors\n\nPart 2\n\n* To evaluate the response rate (CR+PR) of the combination of cyclophosphamide, PK-guided sorafenib, bevacizumab and atezolizumab in children and AYA with relapsed or refractory FLC following two cycles of therapy\n* To determine if the use of PK-guided sorafenib dosing to maintain a systemic exposure between 20 and 55 reduces the interpatient pharmacokinetic variability of sorafenib and the incidence of sorafenib- induced skin toxicities in children and AYA with relapsed or refractory FLC and other rare solid tumors\n\nParts 1 \\& 2\n\n* To determine if the combination of cyclophosphamide, PK-guided sorafenib and atezolizumab will result in increased intratumoral T-cell infiltration of CD8+C45RO+ cells between baseline and following two courses of therapy in pediatric children and AYA with relapsed or refractory solid tumors following two cycles of therapy\n* To characterize the pharmacokinetics of atezolizumab in combination with cyclophosphamide, PK-guided sorafenib and bevacizumab in children and AYA with relapsed or refractory solid tumors\n* To assess the feasibility of performing contrast enhanced ultrasound and explore the correlation between quantitative CEUS parameters and clinical response.\n\nSecondary Objectives\n\nPart 1\n\n• To describe the response rate (CR+PR) of the combination of cyclophosphamide, PK-guided sorafenib, bevacizumab and atezolizumab in children and AYA with relapsed or refractory solid tumors following two cycles of therapy\n\nPart 2\n\n• To describe the response rate (CR+PR) of the combination of cyclophosphamide, PK-guided sorafenib, bevacizumab and atezolizumab in children and AYA with relapsed or refractory FLC, HCC, desmoplastic small round cell tumor, malignant rhabdoid tumor, and other rare solid tumors following two cycles of therapy\n\nParts 1\\&2\n\n* To describe the number of children with liver tumors, initially judged unresectable at diagnosis, that can have their primary tumor resected after treatment with oral cyclophosphamide and sorafenib with intravenous bevacizumab and atezolizumab\n* To describe changes in immune cells in the peripheral blood at periodic times before and after treatment with this combination chemoimmunotherapy\n* To describe the PFS, EFS, and OS in patients treated with the combination of cyclophosphamide, PK-guided sorafenib, bevacizumab, and atezolizumab in patients with relapsed or refractory FLC, DSRCT, MRT, HCC and other rare solid tumors",[27,28,29,30,31,32],"Refractory Solid Tumor","Hepatocellular Carcinoma","Malignant Solid Tumor","Pediatric Cancer","Pediatric Solid Tumor","Fibrolamellar Carcinoma","RECRUITING","2026-05-18",{"date":36,"type":37},"2026-05-19","ACTUAL",{"date":39,"type":37},"2022-11-07",{"date":41,"type":20},"2037-06",{"name":43,"class":44},"St. Jude Children's Research Hospital","OTHER",2,{"id":47,"slug":48,"hasResults":11,"nctId":49,"briefTitle":50,"officialTitle":51,"acronym":52,"eligibilityCriteria":53,"healthyVolunteers":11,"sex":16,"minAge":54,"maxAge":4,"enrollmentInfo":55,"targetDuration":4,"studyType":21,"phases":57,"briefSummary":58,"conditions":59,"keywords":100,"overallStatus":33,"whyStopped":4,"lastUpdateSubmitDate":121,"lastUpdatePostDateStruct":122,"startDateStruct":124,"completionDateStruct":126,"leadSponsor":128,"locationsCount":130},"100565188","phase-2-agnostic-therapy-in-rare-solid-tumors-100565188","NCT06638931","Agnostic Therapy in Rare Solid Tumors","Phase II Basket Study to Evaluate the Tissue-agnostic Efficacy of Anti-Programmed Cell Death Protein 1 (Anti-PD1) Monoclonal Antibody in Patients With Advanced Rare Tumors","ANTARES","Inclusion Criteria\n\n1. Age 18 years or older.\n2. Patients with immunohistochemistry for PD-L1 with a combined positive score (CPS) of 10 or higher.\n3. Patients with progression or intolerance to already approved and accessible treatments for the specific neoplasm and population.\n4. Documented disease progression radiologically after the last routine treatment.\n5. Eastern Cooperative Oncology Group (ECOG) performance status of 0 or 1.\n6. Measurable lesion per RECIST v1.1. Lesions previously treated with radiotherapy can only be used as target lesions if they are confirmed to be progressing by imaging before enrollment.\n7. Male participants must meet at least one of the following conditions:\n\n   1. Considered infertile;\n   2. No fertile partner;\n   3. Has a fertile partner who agrees to follow contraceptive guidance throughout the study period and for at least 6 months after the last dose of Nivolumab;\n\n      and\n   4. Agrees to abstain from sperm donation throughout the study period and for at least 6 months after the last dose of Nivolumab.\n8. Female participants must meet at least one of the following conditions:\n\n   1. Considered infertile;\n   2. Agrees to follow contraceptive guidance throughout the study period and for at least 6 months after the last dose of Nivolumab;\n9. Estimated life expectancy greater than 12 weeks, as determined by the investigator or delegated sub-investigator.\n10. Preserved organ functions defined by:\n\n    * Absolute neutrophil count ≥ 1,000;\n    * Hemoglobin ≥ 8.0 g\u002FdL (patients may receive transfusions to reach this level);\n    * Platelet count ≥ 100,000;\n    * Total bilirubin ≤ 1.5 × Upper Limit of Normal (ULN), or ≤ 3.0 × ULN for patients with Gilbert's syndrome;\n    * Aspartate Aminotransferase (AST) and Alanine Aminotransferase (ALT) ≤ 2.5 × ULN (≤ 5 × ULN in the presence of liver metastases);\n    * Creatinine clearance \\> 30 mL\u002Fmin (estimated by Cockcroft-Gault).\n11. Diagnosis of rare cancer (List I) confirmed by histopathological examination, with the possibility of including other types of rare tumors (incidence of less than 6 in every 100,000) after careful evaluation and approval by the study board.\n\n    * List I:\n\n      * Urachal adenocarcinoma\n      * Parathyroid carcinoma\n      * Nasopharyngeal epithelial tumors\n      * Fibrolamellar carcinoma of any primary site\n      * Angiosarcoma of any primary site\n      * Secretory breast carcinoma\n      * Anal cancer\n      * Metaplastic breast carcinoma\n      * Chromophobe renal carcinoma, Microphthalmia-associated Transcription Factor (MiT) family translocation renal carcinoma; renal carcinoma with Fumarate Hydratase (FH) or Succinate Dehydrogenase (SDH) deficiency\n      * Carcinosarcoma of any primary site\n      * Small intestine cancer\n      * Cholangiocarcinoma\n      * Sertoli-Leydig cell tumors\n      * Cervical cancer of non-epidermoid histology\n      * Tracheal epithelial tumors\n      * Non-cystadenoma salivary gland tumors\n      * Mesothelioma of any site\n      * Neuroblastoma\n      * Adrenal cancer\n      * Penile cancer\n      * Apocrine carcinoma\n      * Fibrosarcoma of any primary site\n      * Cancer of unknown primary site\n      * Hemangioblastoma of any primary site\n      * Thyroid cancer\n      * Hepatoblastoma\n      * Fallopian tube cancer\n      * Leiomyosarcoma of any primary site\n      * Vaginal cancer\n      * Neurofibrosarcoma of any primary site\n      * Gallbladder cancer\n      * Osteosarcoma of any primary site\n      * Bile duct cancer\n      * Clear cell endometrial carcinoma\n      * Yolk sac tumor of any primary site\n      * Non-epidermoid bladder cancer\n      * Vulvar cancer\n      * Kaposi's sarcoma\n      * Epithelial ovarian cancer\n      * Soft tissue sarcoma\n      * Urethral cancer\n      * Granulosa cell tumor of any primary site\n      * Cystadenoma carcinoma\n      * Primitive neuroectodermal tumor of any primary site\n      * Pure or mixed neuroendocrine tumors with neuroendocrine component\n      * Trophoblastic tumor\n\nExclusion Criteria\n\n1. Previous treatment lines with immunotherapy (immune checkpoint inhibitors).\n2. Pregnant or breastfeeding individuals.\n3. Limiting comorbidity, in the opinion of the investigator.\n4. Active infection.\n5. Major surgery within the last 4 weeks.\n6. Functional class II or greater heart failure.\n7. Myocardial infarction or stroke within the last 6 months.\n8. History of pulmonary fibrosis or pneumonitis.\n9. Autoimmune diseases, except for patients with vitiligo and\u002For controlled thyroid\u002Fhypothyroidism without the use of immunosuppressors.\n10. Second invasive primary tumor diagnosed in the last 3 years and\u002For with active disease, except for localized skin tumors (non-melanoma) that have been treated with curative intent.\n11. Patients with prolonged QT interval.\n12. Uncontrolled Central Nervous System (CNS) metastases. Patients who have previously received local treatment, such as radiotherapy, will be eligible if clinical and radiological stability is demonstrated in the 2 weeks prior to the start of treatment. Patients must not be using corticosteroids for managing CNS disease.\n13. Presence of meningeal carcinomatosis.\n14. Worsening renal and liver function in the 14 days prior to enrollment.\n15. History of solid organ transplantation with or without immunosuppression.\n16. Patients with untreated acquired immunodeficiency. Immunocompromised patients may be included as long as they do not have active opportunistic disease and\u002For active infection, after thorough clinical evaluation by the investigator or sub-investigator. HIV-positive patients must have documented undetectable viral load prior to inclusion.\n17. Chronic use of corticosteroids at doses greater than 10 mg\u002Fday of prednisone or equivalent. Patients with adrenal insufficiency of non-autoimmune etiology (e.g., previous bilateral adrenalectomy) may be included if they are clinically compensated with 10 mg\u002Fday of prednisone or equivalent or less.","18 Years",{"count":56,"type":20},28,[24],"The ANTARES study is a phase II basket trial designed to evaluate the tissue-agnostic efficacy of the monoclonal anti-PD1 antibody, nivolumab, in patients with advanced or metastatic rare tumors.\n\nThe study aims to treat rare malignancies with PD-L1 expression (CPS ≥ 10), regardless of the tumor's tissue type or location. Patients who have not responded to standard treatments will be included, and treatment will last for up to 12 months. The study will assess objective response, progression-free survival, and biomarkers such as PD-L1, ctDNA, and microvesicles, in a multicenter collaborative effort to provide innovative therapeutic options for this underrepresented population",[60,61,32,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99],"Urachal Cancer","Parathyroid Carcinoma","Angiosarcoma","Secretory Carcinoma of Breast","Anal Neoplasms","Metaplastic Breast Carcinoma","Translocation Renal Cell Carcinoma","Carcinosarcoma","Small Intestine Neoplasms","Cholangiocarcinoma","Sertoli-Leydig Cell Tumor","Adenoid Cystic Carcinoma","Mesothelioma","Neuroblastoma","Adrenal Gland Neoplasms","Penile Neoplasms","Apocrine Carcinoma","Fibrosarcoma","Cancer of Unknown Primary","Hemangioblastoma","Thyroid Neoplasms","Hepatoblastoma","Fallopian Tube Neoplasms","Leiomyosarcoma","Vaginal Neoplasms","Neurofibrosarcoma","Gallbladder Neoplasms","Osteosarcoma","Biliary Tract Neoplasms","Clear Cell Endometrial Cancer","Yolk Sac Tumor","Vulvar Neoplasms","Kaposi Sarcoma","Ovarian Epithelial Cancer","Soft Tissue Sarcoma","Urethral Neoplasms","Granulosa Cell Tumor","Primitive Neuroectodermal Tumor","Neuroendocrine Tumors","Trophoblastic Tumor",[101,61,102,32,62,103,104,65,105,67,106,69,107,108,109,110,72,73,111,112,76,77,78,79,113,81,114,83,115,85,116,87,117,89,90,118,119,92,93,94,120,96,71,97,98,99],"Urachal Adenocarcinoma","Nasopharyngeal Epithelial Tumors","Secretory Breast Carcinoma","Anal Cancer","Chromophobe Renal Carcinoma","Small Intestine Cancer","Sertoli-Leydig Cell Tumors","Non-Squamous Cervical Neoplasm","Tracheal Epithelial Tumors","Non-Adenoid Cystic Salivary Tumors","Adrenal Neoplasm","Penile Cancer","Thyroid Cancer","Fallopian Tube Cancer","Vaginal Cancer","Gallbladder Cancer","Biliary Tract Cancer","Non-Squamous Bladder Cancer","Vulvar Cancer","Urethral Cancer","2026-04-10",{"date":123,"type":37},"2026-04-15",{"date":125,"type":37},"2024-07-16",{"date":127,"type":20},"2028-05",{"name":129,"class":44},"Instituto do Cancer do Estado de São Paulo",8,{"id":132,"slug":133,"hasResults":11,"nctId":134,"briefTitle":135,"officialTitle":136,"acronym":4,"eligibilityCriteria":137,"healthyVolunteers":11,"sex":16,"minAge":138,"maxAge":17,"enrollmentInfo":139,"targetDuration":4,"studyType":21,"phases":141,"briefSummary":142,"conditions":143,"keywords":147,"overallStatus":33,"whyStopped":4,"lastUpdateSubmitDate":150,"lastUpdatePostDateStruct":151,"startDateStruct":153,"completionDateStruct":155,"leadSponsor":157,"locationsCount":159},"100372833","phase-2-checkpoint-inhibition-in-pediatric-hepatocellular-carcinoma-100372833","NCT04134559","Checkpoint Inhibition In Pediatric Hepatocellular Carcinoma","The Role of Checkpoint Inhibition in Relapsed\u002FRefractory Pediatric Hepatocellular Carcinoma: Clinical Efficacy and Biologic Correlates - A Phase II Study","Inclusion Criteria:\n\n* Age: Patients must be \\\u003C30 years of age at the time of study enrollment.\n* Diagnosis: Patients must have relapsed\u002Frefractory, histologically confirmed HCC to be eligible for enrollment. Patients with hepatocellular neoplasm not otherwise specified (HCN NOS) will also be eligible.\n* Disease Status: Participants must have measurable disease by RECIST criteria, defined as at least one lesion that can be accurately measured in at least one dimension (longest diameter to be recorded for non-nodal lesions and short axis for nodal lesions) measured at ≥20 mm with conventional technique or ≥10 mm with spiral CT scan, MRI, or calipers by clinical exam. See Section 10 for the evaluation of measurable disease.\n* Performance Level: Karnofsky performance status ≥ 60% for patients ≥ 16 years of age or Lansky ≥ 60% for patients \\\u003C 16 years of age.\n* Prior Therapy: Patients must have fully recovered from the acute toxic effects of all prior anti-cancer therapy.\n* Patients must not have received standard or targeted treatment regimens within 14 days of initiation of treatment with pembrolizumab.\n* Patients must not have received prior radiotherapy within 7 days of initiation of treatment with pembrolizumab. Patients who have experienced radiation-induced adverse events must recover to a grade 1 prior to enrollment.\n* Organ Function Requirements: Participants must have normal organ and marrow function as defined below:\n\n  * Adequate Bone Marrow Function defined as:\n\n    * Peripheral absolute neutrophil count (ANC) ≥ 750\u002FμL\n    * Platelet count ≥ 75,000\u002FμL (can be transfused)\n  * Adequate Liver Function defined as:\n\n    * Total bilirubin \\\u003C 1.5 x institutional upper limit of normal (ULN)\n    * AST(SGOT) ≤ 2.5 x ULN\n    * ALT(SGPT) ≤ 2.5 x ULN\n    * If liver function studies are more elevated than the thresholds above, and if if this elevation is felt secondary to tumor, patients may still be eligible for enrollment after discussion with the study PI.\n  * Adequate Renal and Metabolic Function defined as:\n\n    * A serum creatinine based on age\u002Fgender as follows:\n    * Age Maximum Serum Creatinine (mg\u002FdL) Male Female\n    * 1 to \\\u003C2 years 0.6 0.6\n    * 2 to \\\u003C6 years 0.8 0.8\n    * 6 to \\\u003C10 years 1.0 1.0\n    * 10 to \\\u003C13 years 1.2 1.2\n    * 13 to \\\u003C16 years 1.5 1.4\n    * \\>16 years 1.7 1.4\n  * OR\n\n    * Creatinine clearance ≥60 mL\u002Fmin\u002F1.73 m2 for participants with creatinine levels above institutional normal.\n    * Amylase ≤ 1.5 x ULN\n    * Lipase ≤ 1.5 x ULN\n* If liver function studies are more elevated than the thresholds above, and if this elevation is felt secondary to tumor, patients may still be eligible for enrollment after discussion with the study PI.\n\n  \\-- Adequate Thyroid Function defined as:\n\n  \\--- TSH ≤1.5 ULN. Patients can be receiving thyroid supplementation.\n* Confirmation of Insurance Pre-authorization approval for Pembrolizumab.\n* Patients, their parent, and\u002For legally authorized representative must be able to understand and be willing to sign a written informed consent document. Assent for participants \\\u003C 18 years will follow institutional guidelines. The protocol will require approval by each institution's Institutional Review Board.\n* The effects of pembrolizumab on the developing human fetus are unknown. For this reason, patients of child-bearing and child-fathering potential must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to study entry, for the duration of study participation, and for 4 months after completion of pembrolizumab administration. Should a female become pregnant or suspect she is pregnant while she or her partner is participating in this study, she should inform her treating physician immediately. Males treated or enrolled on this protocol must also agree to use adequate contraception prior to the study, for the duration of study participation, and 4 months after completion of pembrolizumab administration.\n* Women of child bearing potential (WOCBP) must have a negative serum or urine pregnancy test within 24 hours of each treatment.\n* A tumor sample must be available for submission to the central laboratory (Dana-Farber Cancer Institute, see Section 9). If surgery was performed at the time of recurrence, this sample, in addition to a diagnostic sample should be submitted. If no re-operation was performed, archived tissue from diagnosis or the most recent procedure should be submitted (see section 9 for further details regarding tissue specifications).\n\nExclusion Criteria:\n\n* Participants who are receiving any other investigational agents are not eligible.\n* Participants who have received checkpoint inhibitors (PD-1, PD-L1, and CTLA-4 inhibitors) are not eligible.\n* Participants who have received antibody-based therapies are not eligible if they are within 3 half-lives of receipt of the last antibody dose.\n* Participants who are receiving chronic steroids are not eligible.Chronic steroids are defined as either \\> or = 2mg\u002Fkg\u002Fday of body weight or \\> or = 20mg\u002Fday of prednisone or equivalent for persons who weigh \\> or = 10kg administered for \\> or = 14 consecutive days.\n* Participants who are receiving anti-inflammatory or immunosuppressive medications are not eligible.\n* Participants with known autoimmune disease, with the exceptions of childhood asthma or atopic dermatitis, are not eligible.\n* Patients with a history of a positive test for human immunodeficiency virus or acquired immunodeficiency syndrome are not eligible.\n* Patients with a history of allergic reactions attributed to compounds of similar chemical or biologic composition to pembrolizumab are not eligible. History of severe allergy to monoclonal antibody therapies (i.e. anaphylaxis) are likewise an exclusion.\n* Patients with uncontrolled intercurrent illness including, but not limited to, ongoing or active infection, symptomatic congestive heart failure, or psychiatric illness\u002Fsocial situations that would limit compliance with study requirements are not eligible.\n* Patients with prior solid organ transplantation are not eligible.","0 Years",{"count":140,"type":20},18,[24],"This research study is studying an immunotherapy drug (pembrolizumab or KEYTRUDA) as a possible treatment for pediatric hepatocellular carcinoma or hepatocellular neoplasm not otherwise specified (HCN NOS).",[144,32,145,146],"Hepatocellular Carcinoma, Childhood","Liver Cancer","Liver Cancer, Pediatric",[148,32,145,149],"Hepatocellular Carcinoma Childhood","Liver Cancer Pediatric","2026-02-23",{"date":152,"type":37},"2026-02-24",{"date":154,"type":37},"2020-11-01",{"date":156,"type":20},"2028-01-01",{"name":158,"class":44},"Allison O'Neill, MD",5,{"id":161,"slug":162,"hasResults":11,"nctId":163,"briefTitle":164,"officialTitle":164,"acronym":165,"eligibilityCriteria":166,"healthyVolunteers":11,"sex":16,"minAge":54,"maxAge":4,"enrollmentInfo":167,"targetDuration":4,"studyType":169,"phases":4,"briefSummary":170,"conditions":171,"keywords":179,"overallStatus":33,"whyStopped":4,"lastUpdateSubmitDate":185,"lastUpdatePostDateStruct":186,"startDateStruct":188,"completionDateStruct":190,"leadSponsor":192,"locationsCount":194},"100557710","a-french-multicenter-observational-retrospective-study-of-rare-primary-liver-cancers-100557710","NCT06541652","A French Multicenter Observational Retrospective Study of Rare Primary Liver Cancers","FFCD-2205","Inclusion Criteria:\n\n* 18 years of age or older,\n* with histologically proven hepato-cholangiocarcinoma, fibrolamellar hepatocellular carcinoma, epithelioid hemangioendothelioma or hepatic angiosarcoma\n* diagnosed after January 01, 2018 living or deceased at the time of registration in the cohort\n* for living patients who have not objected to the research: (note of non-objection to be attached° agreeing to participate in ancillary studies must sign the biological consent form for participation in biological studies.\n\nExclusion Criteria:\n\n* without social security\n* No access to tumor block",{"count":168,"type":20},150,"OBSERVATIONAL","The aim of this French multicenter retrospective study is to describ rare primary hepatic cancers clinical, histological and radiological features, to obtain a biological tumor and blood collection, and to evaluate the efficacy of treatments received in clinical practice in order to determine optimal therapeutic sequences. This retrospective cohort will be the backbone of future translational studies aimed at identifying new molecular, histological, circulating and radiological tumor biomarkers, potentially useful at every stage of diagnosis and prognostic or theranostic evaluation.",[172,32,173,81,174,175,176,177,178,69],"Hepatocholangiocarcinoma","Hepatic Epithelioid Hemangioendothelioma","Hepatic Neuroendocrine Carcinoma","Hepatic Carcinosarcomas","Hepatic Cystadenoma","Hepatic Leiomyosarcomas","Hepatic Angiosarcomas",[180,181,182,183,184],"rare primary liver cancers","LIVER","HEPATIC","COHORT","RISK FACTORS","2025-08-04",{"date":187,"type":37},"2025-08-05",{"date":189,"type":37},"2024-03-26",{"date":191,"type":20},"2031-02",{"name":193,"class":44},"Federation Francophone de Cancerologie Digestive",40]