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Paraganglioma clinical trials

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NCT ID: NCT05233878 Recruiting - Paraganglioma Clinical Trials

Prognostic's Factors of Head and Neck Paragangliomas Evolution

PRONO-PARAG
Start date: July 19, 2022
Phase:
Study type: Observational

Cervical paragangliomas (HNPG) are rare tumors (0.6% of head and neck tumors) arising from chromaffin tissue, of cervical paraganglia (PGL). The most common locations are carotid body (60% of cases), jugulo-tympanic region and vagal body. More than 30% are proved to occur in a context of genetic predisposition, more often in young people, and genetic screening is recommended in all patients. Multifocal tumors represent 12% of all HNPG and until 50% of familial forms. Most of HNPG are non-secreting, benign and slow growing tumors, but up to 30% present complications of local growth, and up to 10% can develop distant metastasis that define malignancy since there is no pathological marker. Historically, surgical treatment was the standard of care but represents nowadays around 50% of the treatment, mostly due to the identification of high morbidity rates. The rate of recurrence is probably around 10% at 5 years. Radiotherapy and active follow-up represent the main therapeutic alternatives. The standard of care is classically surgical but may expose to important sequelae leading to a review of its primary indication. Indeed, cranial nerve palsies (VII, IX, X, XI and XII) may complicate up to 20% of carotid PGL surgeries and up to 95% of vagal PGL surgeries. They are leading to significant functional sequelae, sometimes requiring recourse to a gastrostomy (4/79 patients operated on in a retrospective cohort). First bite syndrome, Claude Bernard Horner syndrome, baroreceptor failure, xerostomia, and ischemic events complicate 5.8%, 4.9%, 1.9%, 1%, and 1% of surgeries respectively. In a local retrospective study conducted by the Hospices Civils de Lyon on 34 operated cervical PGL, the overall complication rate reached 62%. These complications depend mainly on the location tumor and its size. Control rate of irradiated HNPG at 5 years from retrospective series seems to be around 90%. They seems also to have a possible better progression-free survival at 15 years than surgery. The tolerance is correct, the risk of induced malignancy is estimed at 1/1000 to 1/2000. Without treatment, 44% of cervical PGL show a significant progression (median follow-up 51 months). Progression is estimated at 0.41 mm/year for jugulo-tympanic PGL and 1.6 mm/year for vagal and carotid PGL. Currently, there is no clear and robust consensus regarding the follow-up of cervical PGL and the indications for different therapeutic strategies. Data available are represented by retrospective studies only, mostly small in size, with heterogeneous and often inadequate follow-up compared to slow tumor growth. Thus, this prospective cohort study with a standardized long-term follow-up will allow to characterize the management modalities and the evolution of this population.

NCT ID: NCT05142241 Recruiting - Clinical trials for Malignant Solid Neoplasm

Testing the Combination of Anti-Cancer Drugs Talazoparib and Temozolomide in Patients With Advanced Stage Rare Cancers, RARE 2 Trial

Start date: July 11, 2022
Phase: Phase 2
Study type: Interventional

This phase II trial tests whether combination of talazoparib and temozolomide works to shrink tumors in patients with rare cancer that may have spread from where it first started to nearby tissue, lymph nodes, or distant parts of the body (advanced). Talazoparib is an inhibitor of poly adenosine diphosphate-ribose polymerase (PARP), an enzyme that helps repair deoxyribonucleic acid (DNA) when it becomes damaged. Blocking PARP may help keep cancer cells from repairing their damaged DNA, causing them to die. PARP inhibitors are a type of targeted therapy. Temozolomide is in a class of medications called alkylating agents. It damages the cell's DNA and may kill cancer cells. Giving talazoparib in combination with temozolomide may help shrink advanced rare cancers or stop them from growing.

NCT ID: NCT05133349 Recruiting - Clinical trials for Advanced or Metastatic Paraganglioma/ Pheochromocytoma

A Prospective Phase II Efficacy and Safety Study of Anlotinib in Metastatic or Locally Advanced Pheochromocytoma/ Paraganglioma : Open-label Single-arm, Exploratory Trial

EASOAIPPGL
Start date: September 14, 2021
Phase: Phase 2
Study type: Interventional

This is an open-label phase II study of an investigational drug, anlotinib in participants with advanced malignant paraganglioma or pheochromocytoma. Pheochromocytoma and paraganglioma (PPGL) are tumors originating from the adrenal medulla or adrenal diplomatic sensory chain, respectively, which can synthesize and secrete large amounts of catecholamines. In this study, participants whose disease has advanced or spread despite prior standard therapy, will receive anlotinib for 2-weeks followed by a 1-week rest period, until disease progression (PD) or drug toxicity intolerance. Anlotinib is an investigational drug, which has been shown to shrink tumours in several tumour models. The study will evaluate the efficacy as well as the toxicity profile of anlotinib when used as an alternative treatment for participants with PPGL tumours.

NCT ID: NCT05069220 Recruiting - Neuroblastoma Clinical Trials

18F-MFBG PET/CT in the Evaluation of Neural Crest Tumor

Start date: September 1, 2021
Phase: Early Phase 1
Study type: Interventional

The aim of this study is to evaluate the diagnostic performance and tumor burden of 18F-metafluorobenzylguanidine (18F-MFBG) positron emission tomography (PET) in patients with neuroendocrine tumors mainly in pheochromocytoma and paraganglioma (PPGL) and neuroblastoma (NB).

NCT ID: NCT04924075 Recruiting - Clinical trials for Pancreatic Neuroendocrine Tumor

Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Solid Tumors With HIF-2α Related Genetic Alterations (MK-6482-015)

Start date: August 12, 2021
Phase: Phase 2
Study type: Interventional

This is a study to evaluate the efficacy and safety of belzutifan monotherapy in participants with advanced pheochromocytoma/paraganglioma (PPGL), pancreatic neuroendocrine tumor (pNET), von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Advanced Solid Tumors With hypoxia inducible factor-2 alpha (HIF-2α) related genetic alterations. The primary objective of the study is to evaluate the objective response rate (ORR) of belzutifan per Response Evaluation Criteria in Solid Tumors Version 1.1 (RECIST 1.1) by blinded independent central review (BICR).

NCT ID: NCT04891081 Recruiting - Pheochromocytoma Clinical Trials

Plasma Metanephrines in Patients With Cyanotic and Acyanotic Congenital Heart Disease

Start date: November 1, 2019
Phase:
Study type: Observational [Patient Registry]

The aim of our study is to compare plasma metanephrines in patients with cyanotic and acyanotic congenital heart disease and possible association with chronic hypoxic stress.

NCT ID: NCT04860700 Recruiting - Clinical trials for Malignant Paraganglioma

The Efficacy and Safety of Anlotinib in Patients With Metastatic Pheochromocytoma or Paraganglioma

Start date: April 30, 2021
Phase: Phase 2
Study type: Interventional

This phase II trial studies how well anlotinib hydrochloride works in treating patients with metastatic pheochromocytoma or paraganglioma. Anlotinib hydrochloride may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth and by blocking blood flow to the tumor.

NCT ID: NCT04583384 Recruiting - Clinical trials for Paraganglioma of Head and Neck

In Vivo Succinate Detection After External Beam Radiation Therapy in SDHx- Paragangliomas

RADIOSUCCES
Start date: October 27, 2020
Phase: N/A
Study type: Interventional

Qualitative and quantitative biomarker of response to radiotherapy is needed in paragangliomas. We aim at assessing the added value of 1H-SRM for the evaluation of early response to EBR therapy in patients with cervical SDHx PGL.

NCT ID: NCT04573816 Recruiting - Pheochromocytoma Clinical Trials

Development of a Tele-monitoring Program for Patients Undergoing Surgery for Pheochromocytoma and / or Paraganglioma

e-PPGL
Start date: October 8, 2020
Phase:
Study type: Observational

Pheochromocytomas and paragangliomas (PPGL) are rare tumors treated by surgical excision. During follow-up, more than 15% of patients will have recurrences in the form of new tumors, locoregional recurrence or metastases. This subgroup is initially not identifiable. It is therefore usual to perform annual monitoring of all patients throughout their lives by questioning and measuring blood pressure during a medical consultation and by measuring urinary or plasma metanephrines and normetanephrines. The main objective of this prospective monocentric study is to evaluate the reliability of an optimized remote monitoring program in comparison to a usual in-clinic monitoring of patients surgically-cured and tumor-free at the time of inclusion.

NCT ID: NCT04394858 Recruiting - Clinical trials for Metastatic Adrenal Gland Pheochromocytoma

Testing the Addition of an Anticancer Drug, Olaparib, to the Usual Chemotherapy (Temozolomide) for Advanced Neuroendocrine Cancer

Start date: March 17, 2021
Phase: Phase 2
Study type: Interventional

This phase II trial studies how well the addition of olaparib to the usual treatment, temozolomide, works in treating patients with neuroendocrine cancer (pheochromocytoma or paraganglioma) that has spread from where it first started (primary site) to other places in the body (metastatic) or cannot be removed by surgery (unresectable). Poly (adenosine diphosphate [ADP]-ribose) polymerases (PARPs) are proteins that help repair deoxyribonucleic acid (DNA) mutations. PARP inhibitors, such as olaparib, can keep PARP from working, so tumor cells can't repair themselves, and they may stop growing. Chemotherapy drugs, such as temozolomide, work in different ways to stop the growth of tumor cells, either by killing the cells, by stopping them from dividing, or by stopping them from spreading. Giving olaparib with temozolomide may shrink or stabilize the cancer in patients with pheochromocytoma or paraganglioma better than temozolomide alone.